bluuebunny/crossref_metadata_2025 huggingface dataset:
Duodenal Ulcer in Sickle Cell Anemia
Authors: Rao Sudha , Royal Joyce E. , Conrad Harold A. , Harris Vivian , Ahuja Jaya
Published: January 2024

A 14‐year‐old black male with sickle cell anemia developed a duodenal ulcer that masqueraded as sickle cell‐related abdominal pain crisis on multiple occasions. Malingering and poor therapeutic compliance aggravated the ulcer in this patient, who ultimately succumbed to a catastrophic bleed. Duodenal ulcer appears to be an infrequent but difficult to treat lesion in sickle cell disease. An in‐depth review on the occurrence of duodenal ulcer in sickle cell anemia is presented. The etiological mechanisms of peptic ulcer disease in this population and the potential benefits of transfusion therapy are discussed.
Gallbladder Sludge and Lithiasis in an Infant Born to a Morphine User Mother
Authors: Figueroa‐Colon Reinaldo , Tolaymat Naser , Kao Simon C. S.
Published: January 2024

We report a 3‐month‐old boy with gallbladder sludge formation and lithiasis. The known associated factors causing cholelithiasis in infancy were not present. We postulate that maternal chronic use of morphine during pregnancy may result in biliary sludge formation in utero in the fetus with progression and manifestation of cholelithiasis at 3‐months of age.
Hepatotoxicity Due to Pemoline (Cylert)
Authors: Pratt David S. , Dubois Reuben S.
Published: January 2024

Pemoline (Cylert) is an agent used to treat attention deficit disorders and other behavioral syndromes. There have, however, been three published reports of mild hepatic dysfunction in five patients coincident with pemoline therapy. We report two further cases of probable pemoline hepatotoxicity. One case involved a mild aminotransferase elevation in a 6‐year‐old who was on pemoline for 5 months. The second case, in an 11‐year‐old, developed hepatic failure with marked prolongation in prothrombin time nonresponsive to parenteral vitamin K, deep jaundice, and submassive hepatic necrosis. This patient had a long history of pemoline usage. Pharmacokinetics are briefly discussed. A spectrum of hepatic disease due to pemoline is considered and the importance of obtaining aminotransferase values before, during initiation, and throughout treatment is stressed.
Essential Fatty Acid Deficiency Mimicking Porphyria Cutanea Tarda in a Patient with Chronic Cholestasis
Authors: Levy Joseph , DeFelice Amy , Lepage Guy
Published: January 2024

Essential fatty acid deficiency was documented in a 3‐year‐old boy with chronic cholestasis secondary to paucity of intrahepatic bile ducts (Alagille's syndrome). Dietary management had consisted almost exclusively of a proprietary formula with over 80% of the fat as medium‐chain triglycerides. The bullous lesions involved mostly sun‐exposed areas and were diagnosed initially as being compatible with acquired porphyria cutanea tarda. Improvement followed correction of the fatty acid abnormalities with a polyunsaturated fat supplement administered orally. We postulate that the association of fatty acid deficiency and abnormal vitamin E status contributed to skin damage, possibly involving photosensitizing compounds poorly cleared by the markedly cholestatic liver.
Hypersensitivity Reaction in an Infant Fed Hydrolyzed Lactalbumin Contained in a Semielemental Formula
Authors: Heyman Melvin B. , Stoker Terry W. , Rudolph Colin D. , Frick Oscar L.
Published: January 2024

Following introduction of milk protein formula feedings, a 6‐month‐old male developed profuse, watery diarrhea progressing to shock, requiring cardiopulmonary resuscitation. Reinstitution of enteral feedings with a formula containing hydrolyzed lactalbumin (Travasorb STD) resulted in recurrence of diarrhea with fever. Intestinal and rectal biopsies showed only nonspecific inflammatory changes. He was discharged on an elemental formula (Vivonex). Twenty‐three months later, while admitted for evaluation of hypophosphatemic rickets, immunologic testing using the lymphocyte migration inhibition factor (LIF) test demonstrated positive reactions, especially to α‐lactalbumin (56% inhibition) and whole cow's milk (22%, normal of <20% inhibition). Skin tests revealed sensitivity to cow's milk and eggs. Soy formula also produced diarrhea and bloody stools. Protein hydrolysate formulas, touted as hypoallergenic diets, are useful in infants with intolerance to milk protein. This is the first documented case of an immunological reaction to the hydrolyzed whey protein, lactalbumin. Although protein hydrolysate formulas are effective treatment in most infants with milk protein intolerance, allergic reactions are possible. Caution and close observation should be exercised in immunologically sensitized infants rechallenged with any formula.
Vitamin A in Cystic Fibrosis
Authors: Eid Nemr S. , Shoemaker Lawrence R. , Samiec Tamisha D.
Published: January 2024

Much has been learned about vitamin A physiology in the last 50 years, yet few changes have been made in therapy. Unfamiliarity with vitamin A bioavailability and distribution may inadvertently result in toxicity. A literature search demonstrates that hypovitaminosis A has rarely been reported in patients with cystic fibrosis, and may manifest very differently in children of different ages. Furthermore, hypervitaminosis A may present with similar features, and can result from correction of deficiency. We report such a case in a 4.5‐month‐old infant, newly diagnosed with cystic fibrosis, who suffered first from vitamin A deficiency and then vitamin A toxicity. A brief review of vitamin A physiology, deficiency, and toxicity is presented.
Routine L‐Ascorbic Acid Supplementation Does Not Alter Iron, Copper, and Zinc Balance in Low‐Birth‐Weight Infants Fed a Cows'‐Milk Formula
Authors: Stack T. , Aggett P. J. , Aitken E. , Lloyd D. J.
Published: January 2024

The effect of ascorbic acid (AA) [284 μmol (50 mg) twice daily] on the net intestinal absorption and maximum apparent retention of Fe, Cu, and Zn was investigated by metabolic balance studies in a randomised crossover study of six low‐birth‐weight (LBW) neonates fed a cows'‐milk‐based formula containing (μmol/L) Fe, 126; Cu, 11; Zn, 87; and AA, 400. Absorption ± SD (Fe, −5.0 ± 7.5; Cu, 0 ± 0.4; Zn, −0.8 ± 3.4) (μmol kg‐1 day‐1) was not altered by AA (Fe, −4.1 ± 4.6; Cu, 0.3 ± 0.6; Zn, −1.1 ± 2.7) neither was retention (without AA: Fe, −6.0 ± 8.4; Cu, −0.1 ± 0.3; Zn, −2.4 ± 4.2; with AA: Fe, −4.9 ± 4.7; Cu, 0.1 ± 0.6; and Zn, −2.7 ± 3.1). Supplements of AA administered as in the circumstances of routine care of LBW neonates do not enhance the absorption and retention of Fe, nor do they impair these aspects of the metabolism of Cu and Zn.
Rapid Endoscopy Room Diagnosis of Campylobacter pylori‐Associated Gastritis in Children
Authors: Yeung C. K. , Yuen K. Y. , Fu K. H. , Tsang T. M. , Seto W. H. , Saing Htut
Published: January 2024

A modified urease broth test was used in 109 gastric biopsy specimens of children that yielded a 96% sensitivity and a 100% specificity rate for Campylobacter pylori infection. The longest reaction time was ±4 min. The correlation between the degree of C. pylori infection and the reaction time was found to be highly significant (γ = 0.78, p<0.001). Patients were examined as outpatients, and immediate medications were prescribed for eradication of the bacteria on the basis of the urease broth test results. This is the first report on the use of this modified rapid urease test for the diagnosis of C. pylori infection in children.
Plasma Amino Acids in Long‐Term Models for Obstructive Versus Toxic Liver Injury in Developing Rats
Authors: Weisdorf Sally A. , Freese Deborah K. , Radmer William J. , Dehner Louis P. , Cerra Frank B.
Published: January 2024

We evaluated plasma amino acid (AA) concentrations associated with a histologically defined lesion caused by bile duct ligation (BDL) in developing rats. Nineteen rats that underwent BDL at 14 days of age had marked bile duct proliferation with bridging fibrosis, multifocal lobular necrosis, and minimal polymorphonuclear periportal infiltrate in their livers at sacrifice (11–31 days after ligation). These were compared to two age‐matched control groups: 21 nonoperated rats and 22 shamoperated rats; and eight rats with cirrhosis caused by carbon tetrachloride. Signs of liver damage including jaundice, growth failure, bleeding, and ascites were accompanied by elevated bilirubin, ammonia, aspartate aminotransferase (AST), and alkaline phosphatase levels in BDL rats compared to controls. They had higher concentrations of total AAs, phenylalanine, tyrosine, and cyst(e)ine when compared to controls and to CCl4‐treated rats. Micronodular cirrhosis was present in CCl4‐treated rats with elevated AST and alkaline phosphatase levels. Glutamine and glutamate levels were higher in them than in BDL rats or controls, and branched chain AA levels were lower. These two chronic lesions, one obstructive and one hepatotoxic, both result in fibrotic change, but their metabolic abnormalities as reflected in plasma AA levels are distinct. We found that BDL is an appropriate model with which to study metabolic changes and growth failure due to chronic biliary stasis during its progression to frank cirrhosis.
Necrotizing Perianal Infection in Children
Authors: Chuang Jiin‐Haur , Wong Kin‐Sun
Published: January 2024

Two girls aged 1 9/12 years and 5 months were treated for necrotizing perianal infection. In both patients, the necrotizing ulcers around the anus developed following an episode of diarrhea. A poor nutritional status with low body weight (25th percentile) and hypoalbuminemia (2.5 g/L) in the elder patient, as well as a hypogammaglobulinemia in the younger one, may predispose them to bacterial infection and subsequent perianal lesions. Anorectal trauma was denied. The necrotizing infection was confined to the skin and the subcutaneous tissue layer, and both patients had a benign clinical course. We report it and briefly discuss the etiology and treatment of this rare disorder.
Casein and Casein Subunits in Preterm Milk, Colostrum, and Mature Human Milk
Authors: Kunz Clemens , Lönnerdal Bo
Published: January 2024

Human milk proteins have both nutritional and physiological roles for the breast‐fed infant. While the biochemistry and developmental patterns for many whey proteins are well known, our knowledge of human casein and its subunits is still limited. We have recently developed a method to isolate casein from whey proteins in human milk and to separate the casein subunits by fast protein liquid chromatography. In this study we have applied this methodology to study the casein subunit pattern in pretern milk, colostrum, and mature milk. Casein concentration increased with lactation time, largely due to an increase in glycosylated forms of casein (k‐caseins). Thus, the relative proportion of β‐casein to k‐casein decreased during the lactation period. The patterns of phos‐phorylated and glycosylated casein subunits were found to vary during lactation, showing that both synthesis and posttranslational modification of β‐ and k‐casein are regulated by different mechanisms.
Effect of Dietary Nucleosides on Growth and Maturation of the Developing Gut in the Rat
Authors: Uauy Ricardo , Stringel Gustavo , Thomas Rita , Quan Richard
Published: January 2024

Dietary nucleoside (DN) as a precursor for nucleic acid synthesis may be important for rapidly dividing cells, since gut epithelial cells have limited capacity for de novo purine and pyrimidine synthesis. We evaluated in a controlled blinded study the effect of added nucleosides, 0.8% by weight, given for 2 weeks, on gut growth and maturation in 20 weanling rats. Mucosal protein and DNA in the proximal intestinal segment were 50% and 77% higher, respectively, in the DN‐supplemented group (n = 10; p < 0.05). Villus height based on cell count was 25% greater in the DN group (p < 0.05). Maltase activity was significantly greater in proximal, middle, and distal intestinal segments, and the largest increase, 87%, was seen in the proximal gut mucosa. The maltase/lactase ratio was also higher in this segment. Increases in sucrase were less prominent. Lactase was minimally affected. The pattern of change in disaccharidase activity suggests that DN may enhance gut growth and maturation of the intestine in the weanling rat, the effects being more pronounced in the proximal segment. Diets free of nucleosides and nitrogenous bases may have adverse effects on the gut.
An Acute Form of Tyrosinemia Type I With Multiple Intrahepatic Mass Lesions
Authors: Tazawa Yusaku , Kikuchi Masahiro , Kurobane Ikuo , Watanabe Ayako , Nakai Hiroshi , Narisawa Kuniaki , Tada Keiya
Published: January 2024

The acute form of tyrosinemia type I usually causes severe hepatocellular dysfunction. We report a 4‐month‐old infant with hepatosplenomegaly, ascites, and multiple intrahepatic mass lesions mimicking hepatoma. A marked increase of serum α‐fetoprotein (97.6 μg/ml) and multiple small low‐density lesions in the liver demonstrated by computed tomography suggested the presence of hepatoma. The liver specimens obtained at laparatomy showed mixed nodular cirrhosis with fatty metamorphosis. Serum levels of tyrosine (6.6 mg/100ml) and methionine (5.9 mg/100 ml) were increased. Urinary organic acid analyses disclosed increased excretions of succinylacetone (1,330 mg/g creatinine) and δ‐amino‐levulinic acid (113.6 mg/g creatinine). Histological abnormalities and biochemical evidences led to the correct diagnosis. This case emphasizes the need for complete investigations of puzzling cases and warns against undue reliance on noninvasive imaging techniques.
Congenital Hepatic Fibrosis, Cystic Kidneys, Mental Retardation, and Facial Dysmorphy
Authors: Labrune P. , Lange J. C. , Bedossa P. , Chaussain J. L. , Odievre M.
Published: January 2024

This report concerns a boy with congenital hepatic fibrosis, cystic kidneys, mental retardation, and minor dysmorphic features. These symptoms have rarely been reported before and indicate that congenital hepatic fibrosis may not be a single clinical entity.
Prognostic Implications of Serum α‐Fetoprotein Levels in Neonatal Hepatitis
Authors: Lee Ping‐Ing , Chang Mei‐Hwei , Chen Ding‐Shinn , Hsu Hey‐Chi , Lee Chin‐Yun
Published: January 2024

Serum β‐fetoprotein (AFP) values in 61 patients with neonatal hepatitis were compared with the normal ranges of AFP in infants and were expressed as standard deviation (SD) scores. The size of SD score indicates how many standard deviations the measured AFP value is away from the mean value of the normal population. An extremely elevated AFP value is arbitrarily defined as a SD score higher than 4.0, i.e., above mean + 4 SD. The result showed that rapid recovery from jaundice before 6 months of age occurred in 16 of 17 (94%) with SD scores of AFP lower than 4.0. By contrast, in 26 with SD scores of AFP higher than 4.0, 17 (65%) has less favorable clinical courses, either slow in recovery or progressing to chronic liver disease and even death. This correlation between AFP level and prognosis was related to the fact that higher AFP values were associated with more severe inflammatory changes in the liver, as assessed from either biochemical parameters (peak total and direct bilirubin levels) or histotogical findings (portal inflammation and giant cell transformation).
Oral Supplementation of Vitamin K for Pregnant Women and Effects on Levels of Plasma Vitamin K and PIVKA‐II in the Neonate
Authors: Motohara Kunihiko , Takagi Satoshi , Endo Fumio , Kiyota Yuji , Matsuda Ichiro
Published: January 2024

Levels of plasma vitamin K1 (VK1) and vitamin K2 (VK2) and protein‐induced vitamin K absence‐II (PIVKA‐II) were measured in Japanese mothers and their newborn (N = 33). Twenty milligrams of VK1 (N = 11) or VK2 (N = 12) were given orally to randomly selected mothers 7 to 10 days prior to delivery. Means of plasma VK1 and VK2 concentrations were significantly higher in VK1 (p < 0.01) and VK2 (p < 0.01) treated mothers than in the controls at delivery, respectively. Similarly, these levels were significantly elevated in cord plasma in VK1 (p < 0.05) and VK2 (p < 0.05) treated groups, compared with findings in the control group, although there was a large concentration gradient between maternal and cord plasma (mostly less than one‐tenth). A significant positive correlation was found in VK1 concentration between maternal and cord plasma (N = 33, p < 0.01), and the proportion of PIVKA‐II‐positive infants was significantly lower in the VK treated groups than in the control group at birth (p < 0.05). On the fifth postnatal day, mean levels of VK1 (p < 0.01) and VK2 (p < 0.01) in breast milk were significantly higher in the VK1 and VK2 treated mothers than in the control mothers, respectively. In the control group, 9 of 10 infants had a positive PIVKA‐II, but no one in the treated groups was positive, thereby indicating significant differences between control and treated groups (p < 0.01 and p < 0.01, respectively). The administration of both VK,1 and VK2 to mothers prior to delivery may prevent hypoprothrombinemia in infants, at birth, and during neonatal stages.
Placebo‐Controlled Trial of Misoprostol in Cystic Fibrosis
Authors: Robinson P. , Sly P. D.
Published: January 2024

Seventeen children with cystic fibrosis were studied in a 6‐week double‐blind, placebo‐controlled, crossover trial to determine the efficacy of misoprostol, 100 μg, four times a day, in improving fat absorption in patients already on pancreatic enzyme therapy. In those patients who had greater than 90% absorption on enzyme therapy alone, no further significant increase in absorption was achieved with misoprostol administration. Those patients who had absorption of less than 90% on standard enzyme therapy showed a significant improvement with misoprostol administration (p < 0.01). One patient had a significant elevation in the eosinophil count during the period of misoprostol administration, but there were no significant changes in any other hematological or biochemical parameter. Misoprostol appears to be of benefit to those children with cystis fibrosis who have residual malabsorption on standard enzyme therapy.
Intravenous Erythromycin for Postpyloric Intubation
Authors: Di Lorenzo Carlo , Lachman Ralph , Hyman Paul E.
Published: January 2024

Of 100 consecutive patients referred for antro‐duodenal manometry, we successfully intubated 89 with endoscopic and/or fluoroscopic guidance. In 8 of 8 patients who had failed with our usual techniques, we infused erythromycin 3 mg/kg i.v. over 1 h, and the tube moved through the pylorus and into the duodenum. Erythromycin, which stimulates propagation of 3 high amplitude antral contractions/min, appears to be a safe, noninvasive means to facilitate the postpyloric passage of tubes.
Serum Lipoprotein Profile in Children With Celiac Disease
Authors: Rosenthal E. , Hoffman R. , Aviram M. , Benderly A. , Erde P. , Brook J. G.
Published: January 2024

Jejunal mucosa is responsible for the absorption of triglycerides and the production of lipoproteins [chylomicrons, very‐low‐density lipoprotein (VLDL), high‐density lipoprotein (HDL)] and apolipoproteins (B‐48, A‐I, A‐II, A‐IV, C‐II). Mucosal damage is known to cause fat malabsorption and probably also affects the serum lipid profile. To determine lipoprotein production in states of enterocyte dysfunction, we compared the serum lipid profiles in a group of 12 children with untreated celiac disease (flat jejunal mucosa) with the profiles in a control group of 10 children suffering from other intestinal diseases. Statistically significant differences were found in the following parameters (celiac versus control): plasma levels of triglycerides (70 versus 119 mg/dl), cholesterol content in LDL (107 versus 67.7 mg/dl), protein content in VLDL (6 versus 10 mg/dl), and level of apoprotein A‐I (112 versus 140 mg/dl). No significant differences were found between the two groups in the serum levels of total cholesterol, the cholesterol content in VLDL and HDL, the protein content in LDL and HDL, and the level of apoprotein B. Following institution of a gluten‐free diet, the lipoprotein profile reverted to normal. These data suggest that the changes in the serum lipoprotein profile in celiac disease are secondary to alterations in enterocyte function and not only a reflection of fat malabsorption.
Whey Protein Hydrolysate Formula for Infants with Gastrointestinal Intolerance to Cow Milk and Soy Protein in Infant Formulas
Authors: Merritt Russell J. , Carter Margaret , Haight Michael , Eisenberg Larry D.
Published: January 2024

Twenty‐one infants less than 6 months of age with gastrointestinal symptoms of cow milk and/or soy protein‐based infant formula intolerance (diarrhea in 14, hematochezia in 16, emesis in 8, failure to thrive in 4, and colic in 10) were treated clinically with a whey protein hydrolysate formula. Six patients improved when placed directly on the formula, and 15 remained asymptomatic or improved when given the whey hydrolysate formula following initial treatment with a casein hydrolysate or elemental formula. Eighteen had supporting evidence of an allergic basis for their symptoms, including a family history of allergies in 6, a clinical challenge with the offending formula in 1, laboratory tests consistent with atopy in 11, and/or rectal biopsy with histologic allergic features in 7. The whey hydrolysate formula may be an acceptable alternative to soy or casein hydrolysate formulas in most infants with gastrointestinal symptoms of cow milk and/or formula intolerance.
Syringomyelia Presenting as Cricopharyngeal Pseudostricture
Authors: Malik Munir I. , Schwarz Kathleen B. , Kotagal Suresh
Published: January 2024

A 14‐year‐old boy was referred for evaluation of a cervical esophageal “stricture” diagnosed by upper gastrointestinal radiograph, which had been performed for evaluation of dysphagia and dysphonia of several months' duration. Neurological examination revealed several cranial nerve abnormalities and hyperreflexia, raising the suspicion of a cervicomedullary junction lesion. However, computed tomography of the head, neck, and spine was completely normal. Findings on the swallowing videofluoroscopy were interpreted as consistent with a stricture (a “tight constriction distal to the pyriform sinus”). However, no evidence for this was found by either esophagoscopy or esophageal manometry, which revealed a low‐pressure cervical esophageal sphincter. Neurologic evaluation was pursued with magnetic resonance imaging, which revealed a large syrinx extending from C2 to T2 segments. We report this case to point out the importance of considering neurologic disease in the differential diagnosis of “stricture” of the cervical esophagus.
Eosinophilic Proctitis with Giant Cells
Authors: Raafat F. , Castro R. , Booth I. W.
Published: January 2024

Two infants, subsequently shown to have cow's milk protein intolerance, presented with rectal bleeding in the first week of life. Rectal biopsies showed eosinophilic infiltration of the mucosa with several multinucleated giant cells. IgG, IgM, and IgA containing plasma cells were present but IgE could not be detected in rectal biopsies. While cow's milk protein intolerance is a well‐recognized cause of proctocolitis in infancy, eosinophilic granulomatous‐like lesions with giant cells may be a prominent feature and do not exclude the diagnosis.
Variations in Oligosaccharides and Lactose in Human Milk During the First Week of Lactation
Authors: Viverge D. , Grimmonprez L. , Cassanas G. , Bardet L. , Solere M.
Published: January 2024

Variations in oligosaccharides and lactose in human milk were studied in 15 mothers during the first week of lactation. The neuraminyloligosaccharides and heavy neutral oligosaccharides increased slightly from days 2 to 5 postpartum and appeared to decrease until day 7. The lacto‐N‐difucohexaoses, lacto‐N‐fucopentaoses, and lacto‐N‐tetraose increased until day 5 and then decreased. Lactodifucotetraose and the fucosidolactoses decreased substantially until day 5 (p < 0.05) and appeared to stabilize in the following days. Lactose increased until day 5 (p < 0.05) and continued to increase thereafter. Lactose was negatively correlated with total oligosaccharides (p < 0.10). The fluctuations observed in total oligosaccharides from days 2 to 5 postpartum and their subsequent stabilization and regular decrease during lactation confirm the hypothesis of Kulski and Hartmann that mammary secretion occurs in three periods: colostrum for the first 36 h postpartum, transitional milk from days 2 to 5 postpartum, and mature milk after day 5. The oligosaccharide variations we found corresponded to those of other milk constituents observed by other authors. The significance of the oligosaccharide variations is discussed.
Low Blood and Plasma Carnitine Levels in Children Receiving Long‐Term Parenteral Nutrition
Authors: Dahlström K. A. , Ament M. E. , Moukarzel A. , Vinton N. E. , Cederblad G.
Published: January 2024

Total and free carnitine and acylcarnitine concentrations were analyzed in whole blood and plasma in 12 children with a mean age of 68.4 ± 42.9 months who had received carnitine‐free total parenteral nutrition (TPN) for an average of 4 years. The purpose of the study was to see if the children had become carnitine deficient and, if so, whether this correlated with poor lipid clearance. Compared to controls, the TPN‐dependent children had significantly decreased concentrations of total and free carnitine in blood (26.6 ± 9.4 (SD) μmol/L vs. 43.3 ± 9.1 μmol/L, p < 0.001, and 17.1 ± 7.7 μmol/L vs. 35.2 ± 8.1 μmol/L, p < 0.001, respectively). Similar results were found in plasma (total carnitine of 19.0 ± 8.0 μmol/L vs. 41.9 ± 5.2 μmol/L, p < 0.001, and free carnitine of 15.7 ± 7.3 μmol/L vs. 36.1 ± 5.2 μmol/L, p < 0.001, respectively). The acylcarnitine concentration in plasma was decreased in the TPN children (3.3 ± 1.5 μmol/L vs. 5.8 ± 3.0 μmol/L, p < 0.01) compared to controls. Despite the low carnitine concentrations, serum triglyceride levels and serum free fatty acid levels were within the normal range. There was no correlation between carnitine concentrations in plasma and serum triglyceride and free fatty acid levels. Our data show that children receiving carnitine‐free TPN for many years developed markedly decreased concentrations of carnitine in blood and plasma. However, no adverse effects of the low carnitine levels were found on triglyceride and free fatty acid metabolism under stable conditions.
Food Proteins and Maturation of Small Intestinal Microvillus Membranes (MVM). III. Food Protein Binding and MVM Proteins in Rats from Newborn to Young Adult Age
Authors: Stern Martin , Gellermann Barbara , Wieser Herbert
Published: January 2024

To investigate postnatal maturational profiles of functional and biochemical properties of rat small intestinal microvillus membranes (MVM), we did a longitudinal study in rats from birth to the age of 12 weeks. In parallel, we studied binding of cow's milk proteins and of the wheat gliadin peptide B 3142, as well as MVM proteins (SDS‐PAGE). Changes in MVM fluidity and lipid composition exhibited early (0–4 weeks) and intermediate and late (6–12 weeks) patterns, as has been published earlier. Postnatal changes of food protein and peptide binding occurred early during the observation period, not related to weaning. There was not much further change in binding after 6–8 weeks. Developmental profiles of MVM protein and some lipid changes resembled, but did not equal, changes in food protein binding. We conclude that changes in MVM biochemical composition affect MVM binding characteristics. In particular, high molecular weight MVM proteins (susceptible to trypsin treatment) appear to play a role in postnatal maturational differences in MVM food protein binding.
Pathogenesis of Small‐Intestinal Mucosal Lesions in Chronic Diarrhea of Infancy
Authors: Shiner Margot , Nichols Veda N. , Barrish James P. , Nichols Buford L.
Published: January 2024

Our electron microscopic study of biopsies taken from 10 infants with protracted diarrhea was conducted in an effort to determine the pathogenesis of the disorder. In this article, the ultrastructure of the jejunal mucosa of the infants is described in relation to overlying or adherent bacteria of unidentified type. In addition to the known changes on the enterocyte surface caused by adherent bacteria (cupping and effacement), other cytopathic changes, not previously reported, are documented. Included are widespread loss of enterocytes, including intraepithelial lymphocytes, into the bowel lumen; cyto‐pathological changes within the enterocytes: and marked thickening of the basal laminae of the enterocytes and the endothelium of lamina propria blood vessels. In addition. we noted deposition of collagen fibrils in the lamina propria below the basal laminae, active phagolysis within macrophages, and lack of cisternal material (immunoglobulin) in the plasma‐cell cytoplasm. Although these changes are nonspecific, they may be related in part to the presence of the nonadhering and adhering bacteria, and their identification may further our understanding of the “sick mucosa” that occurs in chronic diarrhea of infancy.
Acute Phase Proteins in Neonatal Rabbits
Authors: Baker Robert D. , Long Stephanie
Published: January 2024

Acute phase protein response accompanies tissue injury, inflammation, or infection. During the acute phase, serum levels of C‐reactive protein (CRP) can increase as much as 1,000‐fold. We found that in response to an intramuscular injection of turpentine, neonatal rabbit CRP‐specific RNA and serum CRP rose minimally. In contrast, adult serum levels of CRP increased 20‐fold and mRNA for CRP in adults increased commensurately. However, during neonatal acute phase reactions, changes in the synthesis of the third component of complement (C3) and albumin were induced, implying a dysynchronous development of the response of various acute phase proteins.
Esophageal Chest Pain in Children with Asthma
Authors: Berezin S. , Medow M. S. , Glassman M. S. , Newman L. J.
Published: January 2024

Sixteen patients with asthma and chest pain of >2 months duration underwent gastroenterological evaluation utilizing fiber‐optic esophagogastroduodenoscopy (EGD), esophageal manometry, and Bernstein testing. Eleven of 16 patients (75%) had endoscopic and histologic evidence of esophagitis. One patient with esophagitis exhibited high‐amplitude peristaltic contractions during motility testing. Four of these 11 patients (36%) had a positive Bernstein test. Extended intraesophageal pH monitoring of seven patients with esophagitis revealed significant gastroesophageal reflux (GER) in all of these patients. Chest pain was associated with an episode of GER in three patients (43%). Nine of 11 patients (82%) with esophagitis responded to medical therapy, resulting in resolution of esophageal inflammation and chest pain. One patient required Nissen fundoplication surgery after failure of medical therapy, and one patient who refused surgery progressively developed more severe esophagitis during 9 months of medical therapy. Children with asthma may have chest pain due to gastroesophageal reflux‐associated esophagitis that usually responds to medical therapy.
Histologic Findings Are Not Correlated with Disaccharidase Activities in Infants with Protracted Diarrhea
Authors: Shulman Robert J. , Langston Claire , Lifschitz Carlos H.
Published: January 2024

Histologic assessment as well as information about the disaccharidase activity of the small intestinal mucosa can be useful in the management of patients with small intestinal mucosal damage. In an effort to determine whether the degree of small intestinal mucosal damage would be reflected in a corresponding reduction in disaccharidase activity, we compared small intestinal mucosal histology with the results of disaccharidase activity measured in per oral suction small intestinal biopsies obtained from 21 infants with protracted diarrhea. The degree of small intestinal mucosal damage was graded using a subjective score (i.e., 0 to 4+ damage) by a pathologist (P) and by a computer‐assisted digitizing system (to assess villus surface area, VSA, and villus/crypt ratio, V/C) in a blinded fashion. The mean (±SD) age of the infants was 2.5 ± 1.5 months, and the duration of diarrhea was 25.2 ± 11.5 days. There was good correlation between the results obtained from the digitizing system and from the pathologist: VSA versus P, r = 0.695; V/C versus P, r = 0.791; p = 0.0004. All infants demonstrated some degree of small intestinal mucosal damage. The mean (±SD) values for P, VSA, and V/C were 2.2 ± 1.3, 2.9 ± 0.9, and 0.9 ± 0.5, respectively. The mean values for lactase, sucrase, and maltase were 17.1 ± 17.0, 71.1 ± 54.0, and 224.3 ± 233 μmol/min/g protein, respectively. No correlation was observed between the histologic scoring results and lactase, sucrase, or maltase measurements. Expressing the disaccharidase activities per unit wet weight of tissue did not improve the correlations. Log transformation of the data also failed to improve the correlations. We conclude that extrapolation of disaccharidase activity from the results of histologic measurements in infants with protracted diarrhea is not reliable.
The Effect of Three Intravenous Fat Emulsions Containing Different Concentrations of Linoleic and α‐Linolenic Acids on the Plasma Total Fatty Acid Profile of Neonates
Authors: McClead Richard E. , Lentz Mary E. , Coniglio John G. , Meng H. C. , Gozs Susan
Published: January 2024

We determined the fatty acid profile of total plasma lipids in infants who received one of three intravenous fat emulsions that differed primarily in their linoleic and α‐linolenic acid content: (I) a safflower oil emulsion, (II) a 50:50 mixture of safflower and soybean oils, or (III) a soybean oil emulsion. After 2 weeks of fat therapy, oleic acid, expressed as a percentage of total plasma lipid fatty acids, decreased in all groups, but less so in group III (p < 0.01). The linoleic acid percentage increased in all groups, but group I had the greatest increase (p < 0.05). Group II patients had higher percentages of the linoleic acid metabolites, dihomo–γ‐linolenic acid (II > I, p < 0.05; II > III, p < 0.01) and arachidonic acid (II > III, p < 0.05). Group II patients also had higher levels of α‐linolenic acid (II > I, p < 0.05) and its metabolite, eicosapentaenoic acid (II > I, p < 0.05). Another α‐linolenic acid metabolite, docosahexaenoic acid, however, increased in group III, remained stable in group II, and decreased in group I (III and II > I, p < 0.05). We conclude that the content of linoleic acid and α‐linolenic acid in intravenous fat emulsions results in statistically significant changes in the fatty acid profile of total plasma lipids in infants receiving total parenteral nutrition.
A Study of the Microcirculation in Whole Villi of Neonatal Mice Using a Peroxidase Histochemical Staining Method
Authors: Osborne Michael P. , Haddon Susan J. , Worton Kim J. , Spencer Anthony J. , Starkey William G. , Thornber Dawn , Stephen John
Published: January 2024

The anatomy of the microcirculation of intestinal villi from the upper, middle, and lower small intestine of neonatal mice from 8 to 14 days old was studied using a histochemical peroxidase technique that specifically stained erythrocytes. Over 8–14 days, there was little chronological variation between the same regions of gut; the exception was the lower intestine, which, in younger mice, was noticeably less well perfused with erythrocytes. Vascular beds in the middle and lower intestine comprised a hairpin loop with cross‐connections. In the upper intestine, the capillary beds were generally more complex, particularly in apical regions of the villi. Most villi were well perfused with erythrocytes, but a minority (<10%) contained considerably fewer red cells, some to the point of being totally ischemic. Other villi (<5%) were hyperemic, and the vascular beds packed and engorged with red cells. Usually, the packing density (hematocrit) of red cells within blood vessels increased progressively from villus base to apex. Red‐cell deformation was more prevalent at villus apices, with marked crenation in some villi, yet in the basal regions of these same villi, the red cells were of normal discoid shape. The peroxidase staining technique produces a reliable histological picture of red cells circulating through villi. It also reveals differential perfusion of erythrocytes between and within villi, and that blood vessels pass through hypertonic zones in the apical regions of villi.
Bile Composition of Adult Baboons Is Influenced by Breast Versus Formula Feeding
Authors: Mott Glen E. , Jackson Evelyn M. , McMahan C. Alex
Published: January 2024

We tested the hypothesis that infant cholesterol intake and breast‐ versus formula‐feeding influence the bile cholesterol saturation index and bile acid conjugate composition in adult baboons at 7–8 years of age. We also measured the influence of the postweaning intake of dietary cholesterol and fat (saturated and unsaturated) on the effects of the infant diets. The 80 baboons were derived from six sires and 80 dams and randomly assigned at birth to breast‐feeding or to one of three formulas containing about 2, 30, or 60 mg cholesterol/dl. After weaning at 16 weeks of age the animals were assigned to one of four adult diets, which contained 0.01 or 1.0 mg/kcal of cholesterol containing 40% of calories from saturated or unsaturated fat. The bile cholesterol saturation index was significantly higher at 7–8 years of age in baboons breastfed as infants compared with those fed formula (87.0% versus 72.8%, p < 0.004). The cholesterol saturation index was not significantly different among the three formula groups. Among baboons who were breast‐fed and subsequently fed saturated fat as adults, the glycine/ taurine (G/T) ratios of the bile acid conjugates were about three times those of baboons fed unsaturated fat (1.53 versus 0.47); whereas among formula‐fed animals the type of fat did not influence the G/T ratio (interaction, p = 0.022). Adult baboons fed the three formulas in infancy had an inverse relationship of the G/T ratio to the level of formula cholesterol (p < 0.05), We conclude that breastfeeding and formula feeding in infancy differentially affect biliary lipid composition and its response to dietary cholesterol and fat in adulthood. These deferred effects of infant diet on bile lipid composition may influence gallstone formation and intestinal cholesterol and bile acid metabolism.
Biliary Infusion Therapy in the Inspissated Bile Syndrome of Cystic Fibrosis
Authors: Evans Jonathan S. , George Donald E. , Mollit Daniel
Published: January 2024

A 3.5‐month‐old white boy was born with meconium ileus, peritonitis, and jejunal atresia from cystic fibrosis. He subsequently developed unrelenting and severe extrahepatic biliary obstruction as demonstrated by liver biopsy showing periportal inflammation, cholestasis, and fibrosis. Surgical exploration confirmed the diagnosis of extrahepatic biliary obstruction by severely inspissated bile. A cholecystostomy tube was left in place. The cholestasis remained unresponsive to conservative medical therapy. The obstruction was relieved by hydrostatic infusion of 2% N‐acetylcysteine into the biliary tree over a 6‐day period. The child also received concurrently four i.v. injections of synthetic cholecystokinin. This therapeutic modality was thought to be both safe and effective.
Hemobilia in a Child with Liver Abscess
Authors: Khalil A. , Chadha V. , Mandapati R. , Mathur N. B. , Mittal S. K. , Gulati P. , Chowdhury V. , Tyagi S. , Khalilullah M.
Published: January 2024

Hemobilia in children is rare and has so far been described only as a complication of blunt abdominal trauma. A case of hemobilia secondary to liver abscess treated successfully by selective arterial embolization is described. Hemobilia should be considered in any case of gastrointestinal hemorrhage before any operative intervention is undertaken.
Chronic Diarrhea and Malnutrition—Histology of the Small Intestinal Lesion
Authors: Sullivan Peter B. , Marsh Michael N. , Mirakian Rita , Hill Susan M. , Milla Peter J. , Neale Graham
Published: January 2024

The purpose of this study was to quantitate the jejunal lesion in Gambian children with chronic diarrhea‐malnutrition syndrome. There were 40 subjects (20 male, 20 female) with a mean age of 19.7 months. All were severely malnourished, with marasmus in 30, marasmic kwashiorkor in 9, and kwashiorkor in 1. Of subjects tested, 70% were anergic to intradermal challenge with either purified protein derivative or candidin. Jejunal biopsies, performed on every subject after admission to hospital, were studied by computerised image analysis and immunocytochemistry. A spectrum of mucosal changes that varied from “normal” to “flat” was seen. Mucosae with “normal” architecture revealed infiltration of villous epithelium by small lymphocytes, while crypt hypertrophy was invariably present. At the other extreme, the surface epithelium of flat mucosae was less severely infiltrated, although heavy lymphoid infiltrates persisted within crypt epithelium. Immunohistochemical studies revealed that most intraepithelial lymphocytes were of the CD8 + phenotype. Mucosal morphology did not relate to clinical, biochemical, or anthropometric data for each child. These findings are consistent with an intestinal reaction to some environmental antigen (dietary, microbial, or both) of the cell‐mediated type. This interpretation is strengthened by the expression of major histocompatability class 2D locus alloantigens on crypt epithelial cells in the absence of gut‐reactive autoantibodies.
Unidirectional Water Absorption as a Measure of Intestinal Surface Area in Rats
Authors: Fiedorek Stephen C. , Klish William J. , Graham David Y.
Published: January 2024

Methods to measure expeditiously the anatomic and functional mucosal surface area of the small intestine are needed. We postulated that passive, unidirectional water flux would assess the anatomic mucosal surface area and that the increase in unidirectional flux that occurs in the presence of glucose would correlate with functional surface area. These hypotheses were tested in control and methotrexate‐treated rats. A single‐pass perfusion technique was used in vivo. Methotrexate treatment reduced anatomic and functional surface area (p < 0.05) as measured by computerized planimetry, wet mucosal weight, sucrase activity, and sucrase activity normalized to mucosal DNA content. A formula to predict anatomic surface area was derived from control animal data. The best model used passive, unidirectional water flux as the independent variable, but this model overestimated mucosal surface area in the methotrexate‐treated animals (p < 0.01). Mean unidirectional water flux increased by 45% (p < 0.01) in controls during glucose perfusion, but did not increase in methotrexate‐treated animals (p > 0.37). Thus, unidirectional water flux cannot be used as a universally applicable method to assess the anatomic mucosal surface area, but glucose‐enhanced unidirectional water flux should be evaluated further as an indirect measure of functional surface area.
Hepatic Bile Salt Sulfotransferases in the Rat
Authors: Kane Robert E. , Chen Lee J.
Published: January 2024

Bile salt sulfotransferase (BSS) activity for the fetal bile acid, 3β‐hydroxy‐5‐cholenoate, was first detected in the fetus at 18–19 days of gestation and was twofold greater than for glycolithocholate. The near‐term (20–21 days of gestation) and newborn pup BSS activity was only 5–10% of that in maternal liver. The 3β‐hy‐droxy‐5‐cholenoate sulfotransferase activity rose by the second day of life to levels observed in the mature male, and to activities greater than the mature female by the time of weaning at 3 weeks of age. Sex differences in 3β‐hydroxy‐5‐cholenoate sulfotransferase activity developed during adolescence (28–35 days of age), resulting in fivefold greater activity in the mature female compared with the male. Two isoenzyme activities (BSS I and BSS II) were identified in both sexes during development by DEAE‐Sephadex A‐50 ion‐exchange chromatography of liver cytosol. In the fetal and newborn liver, only one isoenzyme activity was distinctly identified for both monohydroxy bile acids, corresponding to BSS I in older rats. After the first week of life, both BSS I and BSS II exhibited activity like glycolithocholate, but only one peak of activity was identified for 3β‐hydroxy‐5‐cholenoate, corresponding to BSS I. The 3β‐hydroxy‐5‐cholenoate sulfotransferase activity in the mature male was only 20% of the mature female because of a decline in BSS I activity in the male during adolescence. BSS I and II were further purified by taurocholate‐Sepharose 4B chromatography. BSS I had a low affinity toward taurocholate passing through the column, whereas BSS II was absorbed and could be eluted from the column with 0.2 M NaCl. BSS I had four times the activity for 3β‐hydroxy‐5‐cholenoate vs. glycolithocholate, whereas the relative activity of BSS II for 3β‐hydroxy‐5‐cholenoate was only 6% of that for glycolithocholate. BSS I was completely inhibited by 0.1 m M 3‐ketolithocholate, whereas BSS II activity was inhibited only 13%. BSS I activity was enhanced by Co2+ and Zn2+, whereas BSS II activity was inhibited by these divalent ions. We concluded that the monohydroxy bile acids were sulfated by two distinct isoenzymes that could be separated by taurocholate‐Sepharose 4B chromatography. 3β‐Hydroxy‐5‐choleno‐ate was sulfated by BSS I, an isoenzyme regulated by gonadal hormones, which appeared to explain the marked sex differences in 3β‐hydroxy‐5‐cholenoate sulfotransferase activities in mature rats.
Steroid Therapy and Duodenal Ulcer in Infants
Authors: Wolf Yehuda G. , Reyna Troy , Schropp Kurt P. , Harmel Richard P.
Published: January 2024

Two infants with acute life‐threatening complications of duodenal ulcer following steroid administration are described. Although the possible association between steroid therapy and peptic ulcer disease is well known, the need for ulcer prophylaxis during such therapy in infants is not unanimously accepted. The case for ulcer prophylaxis in this setting is presented.
Hypereosinophilic Syndrome in Childhood Appearing as Inflammatory Bowel Disease
Authors: Falade A. G. , Darbyshire P. J. , Raafat F. , Booth I. W.
Published: January 2024

A 7‐year‐old girl with hypereosinophilic syndrome is described, the first such patient to present with inflammatory bowel disease. Hepatosplenomegaly, anaemia, and hypergammaglobulinaemia were also prominent features. Colonoscopy revealed a frank colitis of nonspecific histologic appearance. An elemental diet was unhelpful, but there was a prompt clinical and partial haematological response to systemic steroids. She has remained well receiving a small maintenance dose of prednisolone for 3 years.
Fibroinflammatory Tumor of the Liver Causing Biliary Cirrhosis in an Infant
Authors: Alonso Estella M. , Zdunek Teresita M. , Brown Marilyn R. , Yousefzadeh David K. , Whitington Peter F.
Published: January 2024

We report the case of a 21‐month‐old child with a fibroinflammatory tumor of the liver causing biliary obstruction. The patient had fever, diarrhea, and jaundice. Abdominal ultrasonography, computerized tomography, and endoscopic retrograde cholangiopancreatography demonstrated the tumor to be in the porta hepatis. Growth of the mass resulted in destruction of the common hepatic duct, and chronic obstruction led to biliary cirrhosis over 4 months' time. Histologic study of the tumor revealed a fibrous stroma infiltrated by inflammatory cells. The patient was successfully treated with an orthotopic liver transplantation and is doing well 18 months following initial diagnosis without evidence of recurrence.