bluuebunny/crossref_metadata_2025 huggingface dataset:
Sonographic Findings in Celiac Disease
Authors: Riccabona M. , Rossipal E.
Published: January 2024

In 43 children who had been admitted to our department due to symptoms of malabsorption, sonography of the small bowel was performed before biopsy. Seventeen of the patients were shown to have celiac disease. Twenty‐six patients had gastroenteritis, postenteritis syndrome, and enteropathic cow's milk allergy, and one patient had sarcoidosis. At the time of diagnosis, 16 children with celiac disease showed a sonographically abnormal appearance of the small‐bowel wall structure. In addition to hyperperistalsis, slight ascites, pericardial fluid, or liver tissue texture changes could be found in some of the patients. Relating the findings of abnormal small‐bowel wall structures to the data of the small‐bowel biopsy, we found a sensitivity of 94% and a specificity of 88% for sonographic detection of changes related to celiac disease. Our findings indicate that sonographic data can be of great help in exploring children with signs of malabsorption. These data provide a further argument for the decision to perform a biopsy of the small bowel.
Presence of Multiple Forms of Peptidase Inhibitors in Rat Milk
Authors: Rao R. K. , Lam K. , Philipps A. F. , Williams C. , Lake M. , Koldovský O.
Published: January 2024

Peptides such as somatostatin (SS14), epidermal growth factor (EGF), transforming growth factor‐α (TGFα), and insulin‐like growth factors (IGF‐I and IGF‐II) are present in breast milk from various species, and their significance in the developing gastrointestinal tract has been suggested. Our recent studies have indicated that rat milk soluble fraction (RMSF) protects SS14 in the gastrointestinal lumen by inhibiting in vitro the luminal peptidolysis. In the present studies, we have shown that RMSF inhibited in vitro degradation by midjejunal luminal flushings of suckling rats of 125I‐labeled somatostatin 14[Tyr11], EGF, TGFα, IGF‐I and IGF‐II, as well as trypsin activity in vitro against benzoyl‐L‐arginyl‐p‐nitroanilide. The inhibitory factors present in the RMSF were further fractionated by gel filtration on Sephadex G100, ion‐exchange chromatography on DEAE‐Sephadex, and fast protein liquid chromatography (FPLC). Gel filtration of Sephadex G100 separated RMSF into three peaks of proteins: G1, G2, and G3; peptidase inhibitor activities were present exclusively in G1. Ion‐exchange chromatography on DEAE‐Sephadex column resolved peptidase inhibitory activity (G1) into three different peaks, D1, D2, and D3, eluted at sodium chloride concentrations of 0.05 M, 0.1 M, and 0.2 M, respectively. Further purification of D2 by FPLC resulted in a fraction rich in peptidase inhibitory activity, which was essentially free of trypsin inhibitory activity. Results indicate the presence of at least three peptidase inhibitors in rat milk, which may play a role in the protection of milk‐borne peptides in the gastrointestinal lumen.
Clinical and Biochemical Findings in Progressive Familial Intrahepatic Cholestasis
Authors: Whitington Peter F. , Freese Deborah K. , Alonso Estella M. , Schwarzenberg S. Jane , Sharp Harvey L.
Published: January 2024

SummaryThe clinical findings in 33 patients with progressive familial intrahepatic cholestasis (PFIC) are presented. Symptoms developed almost invariably before 6 months of age with severe pruritus and moderate jaundice. Other clinical findings included wheezing and nosebleeds, fat‐soluble vitamin deficiency states, and cholelithiasis. Lower values for γ‐glutamyl transpeptidase, averaging 15 IU/L before the administration of phenobarbital, and cholesterol, which averaged 156 mg/dl, are helpful in distinguishing PFIC from other pediatric cholestatic liver diseases. Autosomal recessive inheritance is probable. Twenty‐six patients are alive at 12.9 ± 6.7 years of age, all having had successful surgical treatment, either partial biliary diversion (n = 17) or orthotopic liver transplantation (n = 10). Seven patients died at a mean age of 3.9 ± 2.4 years, as a result of liver failure in two, hepatocellular carcinoma in two, and complications of liver transplantation in three.
Longitudinal Growth in Children and Adolescents with Inflammatory Bowel Disease
Authors: Hildebrand Hans , Karlberg Johan , Kristiansson Bengt
Published: January 2024

SummaryWeight and height were followed longitudinally from birth to adulthood in children with inflammatory bowel disease living in a defined area of Sweden, 1983 through 1987; 124 children out of a possible 128 were studied. During the year preceding diagnosis, height growth velocity was significantly reduced in both ulcerative colitis and Crohn's disease. At the time of diagnosis, weight‐for‐height was subnormal in both children with ulcerative colitis (p < 0.05) and those with Crohn's disease (p < 0.001), while height was reduced only in children with Crohn's disease (p < 0.05). Weight for height was normalized within one year in ulcerative colitis, after the initiation of medical therapy. In Crohn's disease, weight‐for‐height improved during the years following diagnosis but height remained subnormal. Children with ulcerative colitis reached puberty at the normal time and their final heights became normal. In children with Crohn's disease, puberty was delayed (p < 0.001) and final height was reduced (p < 0.01). The impact of inflammatory bowel disease on growth was substantial, but it was smaller in this study than in many other published studies. The possible reasons for this difference include use of population‐based material and a relatively short interval between the first symptoms and the start of treatment. Our findings indicate that, although final height was significantly reduced in children with Crohn's disease, delayed puberty reduced the negative effects on permanent adult height, to a certain extent compensating for the period of poor growth earlier in life.
Peripheral γδ T Cell Receptor‐Bearing Lymphocytes Are Increased in Children with Celiac Disease
Authors: Klemola T. , Tarkkanen J. , ürmälä T. , Saxen H. , Savilahti E.
Published: January 2024

The percentages of γδ T cell receptor‐bearing (TCR+) cells in the peripheral blood and jejunal mucosa of patients with celiac disease were measured before treatment, during a gluten free‐diet, and after a gluten challenge. The percentages of the different cell types were evaluated by flow cytometry, and immunohistochemical staining of the jejunal specimen was used to identify lymphocyte surface markers. Nineteen blood samples and intestinal specimens from 13 children with celiac disease (eight samples taken before treatment, six during gluten‐free diet, and five after gluten challenge) and samples from nine controls were studied. The proportion of γδ TCR+ lymphocytes was significantly higher both in the peripheral blood and the jejunal mucosa of the patients with celiac disease. A significant correlation was found between the percentage of peripheral γδ TCR+ cells and the density of γδ TCR+ cells in the lamina propria.
Is a Low‐Osmolarity ORS Solution More Efficacious Than Standard WHO ORS Solution?
Authors: El‐Mougi M. , El‐Akkad N. , Hendawi A. , Hassan M. , Amer A. , Fontaine O. , Pierce N. F.
Published: January 2024

The clinical efficacy of a diluted oral rehydration salts (ORS) solution was compared in a pilot study with that of intravenous (i.v.) therapy and of standard World Health Organization (WHO)/United Nations Childrens Fund (UNICEF) ORS solution in children with acute diarrhea. Sixty‐one boys aged 3 to 24 months, admitted to hospital with acute diarrhea and signs of dehydration, were randomly assigned to groups receiving standard ORS solution, diluted ORS solution, or i.v. therapy. In children treated with standard ORS solution and small amounts of plain water, the total fluid intake was 25–39% greater, the stool output was 58–77% greater (p < 0.01), and the duration of diarrhea was 30–55% greater than in the other treatment groups. Intake of plain water, taken separately or added to the ORS solution, was greater in children given diluted ORS solution (73 ± 23 ml/kg) than in those given standard ORS solution (21 ± 32 ml/kg) (p < 0.001). The mean serum sodium concentration increased by 2.2 mEq/L in children given standard ORS solution, whereas it decreased by 2.9 mEq/L in those given diluted ORS solution. This study shows that some children develop worsening diarrhea and increasing serum sodium concentrations when treated with standard ORS solution and given only small amounts of plain water. This is probably caused by the slight hypertonicity of standard ORS solution combined with transient partial glucose malabsorption. This can be avoided if water, breast milk, or another low‐solute drink is given liberally during maintenance therapy with ORS solution, as recommended by the WHO.
The Acid Steatocrit
Authors: Tran M. , Forget P. , Van den Neucker A. , Strik J. , van Kreel B. , Kuijten R.
Published: January 2024

Summary: The steatocrit method has recently been introduced as a simple screening test for steatorrhea. As it seemed likely that separation of fecal homogenate by centrifugation into a lipid phase, a watery phase, and a solid phase would be pH‐dependent, we evaluated the effect of fecal acidification on steatocrit results. We also compared classical and acid steatocrit results in healthy children and in patients with cystic fibrosis and studied the relationship between two steatocrit methods and fecal fat content as measured by a reference chemical method. Steatocrit results increased with the degree of fecal acidification, and maximal results were obtained at the lowest fecal pH values. Means and SEM for classical and acid steatocrit values were 1.1 ± 0.4% (classical) versus 3.8 ± 1% (acid) in controls (n = 6) and 5.4 ± 1.9% (classical) versus 26.9 ± 4.3% (acid) in cystic fibrosis patients (n = 9). The correlations between fecal fat content measured chemically and steatocrit results were 0.18 (p = 0.35) and 0.81 (p < 0.0001) for classical and acid steatocrit, respectively. We conclude that acidification of fecal homogenates leads to a marked improvement in the steatocrit method.
The Oligosaccharide Composition of Human Milk
Authors: Miller Janette Brand , Bull Stefan , Miller John , McVeagh Patricia
Published: January 2024

Oligosaccharides are an important component of human milk, but little is known about variations in their composition. The aim of this study was to determine the temporal and inter‐individual variations in carbohydrate composition of human milk during the first 3 months of lactation. Serial milk samples of 10 mothers (eight full‐term and two preterm births) were analyzed to determine the concentration of lactose and three monosaccharide components derived from the non‐lactose carbohydrate: sialic acid, N‐acetylglucosamine, and fucose. In full‐term milk, sialic acid and N‐acetylglucosamine were found to decrease significantly (p < 0.05) from weeks 1 to 13 postnatally. On average (mean ± SD), sialic acid decreased by 71% (from 879 ± 157 to 256 ± 82 mg/L; p < 0.05) and N‐acetylglucosamine by 56% (from 1,459 ± 282 to 646 ± 214 mg/L; p < 0.05), while fucose decreased by only 35% (from 660 ± 192 to 432 ± 180 mg/L; p > 0.05). On average, lactose concentration increased by 17% over the same period, from 55.4 ± 4.2 g/L in week 1 to 64.9 ± 2.3 g/L at 3 months. Preterm milk contained higher concentrations of each component, but temporal changes were similar to those seen in full‐term milk. Apart from temporal changes, there were large inter‐individual differences in oligosaccharide composition: fucose varied fourfold, sialic acid threefold, and N‐acetylglucosamine twofold among women at the same stage of lactation. The changes observed may simply reflect the aging of the cells responsible for milk secretion, but they are also consistent with a programmed adaptation of the milk composition to the needs of the infant.
Sex Differences in Iron Stores of Adolescents
Authors: Bergström Erik , Kernell Olle , Lönnerdal Bo , Åke Persson Lars
Published: January 2024

We evaluated iron status and its determinants in healthy adolescents. Fasting morning blood samples from a school‐based cross‐sectional study were analyzed for serum ferritin (SF), serum iron, total iron‐binding capacity, and circulating transferrin receptors. Physical development, chronic disease, medication, dietary intake, and physical activity were assessed using clinical examination, questionnaires, and 7‐day records. The risk of having low serum ferritin values was estimated using bivariate and multivariate regression. Subjects were 867 healthy Swedish adolescents, 14− and 17‐year‐olds (472 boys and 395 girls). SF values increased with pubertal stage in boys but not in girls. Five percent of the boys and 15% of the girls had SF values <12 μg/L. Of the 17‐year‐old boys, 7% compared to 1% of the 17‐year‐old girls had SF values > 100 μg/L. Forty‐one percent of cases with SF values >12 μg/L had serum iron values < 15 μM, and 22% had transferrin saturation values <16%. Mean total iron intakes of the boys were high [1.6 times recommended daily allowance (RDA)] and mean intakes of the girls were adequate (0.9 times RDA). Low heme iron intakes increased the risk of low iron stores (<12 μg/L) in girls but not in boys. Total iron intake or other dietary factors, physical development, or level of physical activity did not influence the risk of low SF. The findings of this study suggest that the differences in iron status between boys and girls in adolescence results primarily from biological differences other than menstrual bleeding or insufficient iron intake. Furthermore, the results question the role of SF as an indicator of iron deficiency in adolescence, in particular if age and sex are not taken into consideration. We suggest that different reference values for SF, including the cut‐off limit for low SF, adjusted for age and sex, should be considered. The high iron intakes and corresponding high SF values found in the older boys are noticeable in light of the possible negative health consequences of iron overload.
Improvement of Growth After Growth Hormone Treatment in Children Who Undergo Liver Transplantation
Authors: Rodeck Burkhard , Kardorff Ruedigor , Melter Michael , Ehrich Jochen H. H.
Published: January 2024

Background

Chronic liver insufficiency in children is frequently associated with growth retardation. Growth resumes after successful orthotopic liver transplantation in the majority of children with previous chronic liver failure. However, a subgroup of children demonstrates stunted growth even after orthotopic liver transplantation. The current study was conducted to determine whether administration of recombinant human growth hormone might benefit these patients.

Methods

Ten children were identified who met the criteria of growth failure despite normal transplant function in a cohort of 60 transplantation patients: height standard deviation score (hSDS) for chronological age less than −2, and growth velocity SDS (gvSDS) for chronological age equaling 0. Seven of these patients were treated with subcutaneous injections of recombinant human growth hormone at 4.0 U/m2 body surface area per day for at least 1 year. Two patients in this group showed insufficient growth hormone response to stimulation (arginine, clonidine) before therapy. Treatment was begun after a median time of 4.6 years after liver transplantation (2.55–8.4 years). Five children were treated with cyclosporin A and prednisolone and two with tacrolimus and prednisolone for maintenance immunosuppression.

Results

Within 3 months of treatment, median serum levels of insulin‐like growth factor (IGF)‐I increased from 0.05 to 0.71 (P < 0.02). Within 1 year, median hSDS improved from −2.7 (range, −5.6 to −2.3) to −2.1 (−4.5 to −1.4;P < 0.03). Median annual growth rate increased from 3.9 cm/year (range, 3–6) in the year before treatment to 8.2 cm/year (range, 6.1–10.4;P < 0.02) after the beginning of recombinant human growth hormone therapy. All patients tolerated treatment without side effects. During the cumulative treatment time of 14 years no rejection episode was observed.

Conclusions

Short‐statured prepubertal liver transplant recipients who do not show sufficient compensatory growth after transplantation benefit from treatment with recombinant human growth hormone. Treatment with the hormone was safe without any side effects.
Partially Hydrolyzed Guar Gum–Supplemented Oral Rehydration Solution in the Treatment of Acute Diarrhea in Children
Authors: Alam Nur H. , Meier Remy , Schneider Heinz , Sarker Shafiqul A. , Bardhan Pradip K. , Mahalanabis Dilip , Fuchs George J. , Gyr Niklaus
Published: January 2024

Background

Partially hydrolyzed guar gum (Benefiber; Novartis Nutrition, Minneapolis, MN, U.S.A.) is fermented by colonic bacteria liberating short‐chain fatty acids (SCFAs), which accelerate colonic absorption of salt and water. The purpose of this study was to evaluate the effect of Benefiber (BF)‐supplemented World Health Organization Oral Rehydration Solution (WHO ORS) in the treatment of acute noncholera diarrhea in children.

Methods

A double‐blind, randomized, controlled clinical trial was performed at ICDDR,B in 150 male children aged 4 to 18 months who had watery diarrhea of less than 48 hours' duration. After admission, children were assigned to receive either WHO ORS or BF‐supplemented WHO ORS until recovery. Major outcome measures, such as duration of diarrhea and amount of stool output, were compared between the treatment groups.

Results

Patients receiving BF‐supplemented WHO ORS had significantly reduced duration of diarrhea compared with the control group (mean ± SD, 74 ± 37 vs. 90 ± 50 hours, P = 0.03). Survival analysis for duration of diarrhea also showed a reduction the BF‐supplemented WHO ORS–treated group (P = 0.025, log rank test). There was also less stool output daily from days 2 through 7 in the patients treated with BF‐supplemented WHO ORS compared with that in the children treated with WHO ORS; the reduction was significant on day 7 only.

Conclusion

Benefiber added to standard WHO ORS substantially reduces the duration of diarrhea and modestly reduced stool output in acute noncholera diarrhea in young children, indicating its potential as a new antidiarrheal therapy for acute diarrhea in children.
Free Glutamine and Glutamic Acid Increase in Human Milk Through a Three‐Month Lactation Period
Authors: Agostoni Carlo , Carratù Brunella , Boniglia Concetta , Lammardo Anna Maria , Riva Enrica , Sanzini Elisabetta
Published: January 2024

Background

Previous short observational studies on the free amino acid (FAA) content of human milk have shown that glutamine and glutamic acid increase in the first 4 to 6 weeks of life.

Methods

Changes in human milk content of free amino acids (FAAs) was determined at colostrum, 1 month, and 3 months of lactation in 16 healthy lactating women after delivery of full‐term infants. Milk was collected at the end of each feeding (hindmilk) during 24 hours.

Results

Glutamic acid and taurine were the most abundant FAAs at colostrum. Although taurine remained stable throughout lactation, glutamic acid (the prevalent FAA) and glutamine increased approximately 2.5 and 20 times, respectively, with progressing lactation representing more than 50% of total FAA at 3 months. The content of essential FAA was also stable, so the change in total FAA content was almost entirely due to the changes in glutamic acid and glutamine.

Conclusions

Breast‐fed infants are supplied with progressively increasing amounts of glutamine and glutamic acid throughout lactation. The increasing intake of glutamic acid and glutamine could benefit breast‐fed infants with molecules that are likely to protect the enteral mucosa and act as neurotransmitters and as a source of nitrogen.
Comparative Evaluation of Serologic Tests for Celiac Disease: A European Initiative Toward Standardization
Authors: Stern Martin ,   Working Group on Serologic Screening for Celiac Disease
Published: January 2024

Background

Serologic methods have been used widely to test for celiac disease and have gained importance in diagnostic definition and in new epidemiologic findings. However, there is no standardization, and there are no reference protocols and materials.

Methods

The European working group on Serological Screening for Celiac Disease has defined robust noncommercial test protocols for immunoglobulin (Ig)G and IgA gliadin antibodies and for IgA autoantibodies against endomysium and tissue transglutaminase. Standard curves were linear in the decisive range, and intra‐assay variation coefficients were less than 5% to 10%. Calibration was performed with a group reference serum. Joint cutoff limits were used. Seven laboratories took part in the final collaborative study on 252 randomized sera classified by histology (103 pediatric and adult patients with active celiac disease, 89 disease control subjects, and 60 blood donors).

Results

IgA autoantibodies against endomysium and tissue transglutaminase rendered superior sensitivity (90% and 93%, respectively) and specificity (99% and 95%, respectively) over IgA and IgG gliadin antibodies. Tissue transglutaminase antibody testing showed superior receiver operating characteristic performance compared with gliadin antibodies. The κ values for interlaboratory reproducibility showed superiority for IgA endomysium (0.93) in comparison with tissue transglutaminase antibodies (0.83) and gliadin antibodies (0.82 for IgG, 0.62 for IgA).

Conclusions

Basic criteria of standardization and quality assessment must be fulfilled by any given test protocol proposed for serologic investigation of celiac disease. The working group has produced robust test protocols and reference materials available for standardization to further improve reliability of serologic testing for celiac disease.
Bone Marrow Failure in Children With Acute Liver Failure
Authors: Tung John , Hadzic Nedim , Layton Mark , Baker Alastair J. , Dhawan Anil , Rela Mohamed , Heaton Nigel D. , Mieli‐Vergani Giorgina
Published: January 2024

Background

Aplastic anemia is a rare but well‐recognized complication of acute hepatitis and acute liver failure. The cause is unknown, and the condition is fatal without bone marrow recovery. Treatment includes immunosuppression regimens or bone marrow transplantation. The purpose of this study was to investigate the incidence, cause, treatment, and outcomes of this disorder in children.

Methods

Retrospective chart review of 75 patients with acute liver failure in a major pediatric liver center.

Results

Eight patients had evidence of bone marrow failure. Of those, six had aplastic anemia, and two had transient bone marrow suppression. There were five boys, median age 57 months (range, 36–132 months). Two had parvovirus B19, six had non‐A, non‐B, non‐C hepatitis. Five underwent liver transplantation: auxiliary in one, orthotopic in four. The interval between initial symptoms and development of aplastic anemia and/or bone marrow suppression was 21 to 99 days (median, 39 days). Four patients with aplastic anemia received intravenous antithymocyte globulin (ATG) or antilymphocyte globulin (ALG). Median recovery period of granulopoiesis was 62 days (range, 27–115 days). Two made a full recovery, one had myelodysplasia, and one with unresponsive disease died of septic complications. Four did not receive ATG/ALG, two had aplastic anemia, and two had bone marrow suppression. Three underwent liver transplantation, and all four resumed granulopoiesis. One child who underwent liver transplantation died of sepsis with chronic rejection. Median recovery of granulopoiesis was 99 days (range, 20–153 days).

Conclusions

Bone marrow failure occurs in 10.7% of children with acute liver failure. It sometimes occurs in association with non‐A, non‐B, non‐C hepatitis and parvovirus B19 infection. Treatment with ATG/ALG is successful and is well tolerated in most cases.
Is Esophagogastroduodenoscopy Necessary in all Caustic Ingestions?
Authors: Gupta Sandeep K. , Croffie Joseph M. , Fitzgerald Joseph F.
Published: January 2024

Background

It is unclear whether symptoms alone can identify patients with caustic ingestion who will benefit from esophagogastroduodenoscopy (EGD). The published data are contradictory. The purpose of the current study was to determine the relationship between initial symptoms and EGD findings in patients with caustic ingestion.

Methods

Chart review of all caustic ingestions who underwent EGD during a 4‐year period (December 1993 through November 1997).

Results

Twenty‐eight patients (15 girls; mean age, 2.7 years (range, 0.92–13.33) underwent EGD after caustic ingestion. Fourteen percent (4/28) of patients were asymptomatic, and findings on endoscopy were normal. Another 57% (16/28) had normal endoscopic findings, although all were symptomatic. Twenty‐nine percent (8/28) of patients had esophageal injury on EGD, and all were symptomatic. Esophageal injury was graded as 1 (mucosal erythema), 2 (superficial burns; noncircumferential) or 3 (deep burns; circumferential). The injury was grade 1 in three of eight patients and grade 2 in two; all had one symptom each. Grade 3 injury was found in three of eight patients: two had two symptoms (drooling and vomiting, drooling and stridor), and one had one symptom (dysphagia). All patients with grade 3 injury subsequently underwent esophageal dilations. Follow‐up information was secured for two of the three patients with grade 1 injury and both patients with grade 2 injury at 34.3 months (range, 24–50) after the ingestion, and all were asymptomatic. Of the 20 patients with absence of esophageal mucosal damage, follow‐up data were available for l5 patients at 37.2 months (range, 7–63) after the event and all were well.

Conclusions

All patients with clinically significant injury (grades 2 and 3) were symptomatic at initial assessment. No single symptom or combination of symptoms could identify all patients with esophageal injury. All asymptomatic patients had normal findings on endoscopic examinations. Esophagogastroduodenoscopy seems unnecessary in asymptomatic patients with alleged caustic ingestion. A larger, prospective study would be necessary to unequivocally answer this clinically important question.
Atypical Morphologic Presentation of Biliary Atresia and Value of Serial Liver Biopsies
Authors: Azar Georgia , Beneck Debra , Lane Bernard , Markowitz James , Daum Fredric , Kahn Ellen
Published: January 2024

Background

Liver biopsy findings are important in diagnosing extrahepatic biliary atresia. Diffuse ductular proliferation is a characteristic finding. We describe four patients with conjugated hyperbilirubinemia in whom the initial liver biopsy findings showed a lack of ductular proliferation, despite subsequent development of biliary atresia.

Results

On initial biopsy, paucity of intrahepatic bile ducts was present in three of four patients, with a bile duct to portal space ratio of 0.3 to 0.4 (normal, 0.9–1.8). A normal bile duct to portal space ratio of 1.0 was observed in the fourth patient. Ductular proliferation became apparent in three subjects between 9 and 12 weeks of age, and biliary atresia was noted at the time of a Kasai portoenterostomy. The fourth child had well‐developed biliary cirrhosis at liver transplantation.

Conclusions

Changes characteristic of biliary atresia may appear even after 9 weeks of age. Bile duct paucity and normal bile duct to portal space ratio do not preclude the subsequent development of biliary atresia. Infants with unexplained conjugated hyperbilirubinemia and acholic stools should undergo sequential liver biopsies until clinical improvement occurs or until biliary atresia can be excluded from the differential diagnosis.
The IMPACT Questionnaire: A Valid Measure of Health‐Related Quality of Life in Pediatric Inflammatory Bowel Disease
Authors: Otley Anthony , Smith Claire , Nicholas David , Munk Marla , Avolio Julie , Sherman Philip M. , Griffiths Anne M.
Published: January 2024

Background and aims

IMPACT is a disease‐specific health‐related quality‐of‐life questionnaire developed for use in pediatric inflammatory bowel disease through a process of patient interviews and analysis of patient responses to an item‐reduction questionnaire. This study sought to assess the feasibility, reliability, and validity of the instrument.

Methods

The readability statistics and number of unanswered questions were assessed among 147 patients (97 CD, 50 UC) with mean age 14.4 ± 2.2 years (range 9.2–18.0 years) using the self‐administered questionnaire. Internal consistency was assessed with Cronbach's α and test‐retest reliability using intraclass correlation coefficient (ICC). Construct validity was based on a priori hypotheses. Mean total scores were compared by ANOVA among patients grouped according to disease activity and disease severity within the past year.

Results

The readability statistic showed a Flesch‐Kincaid Grade level of 4.5. Only 0.68% of questions were left blank. Reliability was excellent with Cronbach's α = 0.96, and an ICC of 0.90 in patients with stable disease over a two‐week period (n = 32). The mean total IMPACT score for patients with quiescent disease (180 ± 32) was significantly higher (better QOL) than for those with active disease (146 ± 31 for mild, 133 ± 34 for moderate/severe) (P < 0.005).

Conclusions

The IMPACT questionnaire is a valid and reliable reflection of health‐related quality of life of older children and adolescents with both ulcerative colitis and Crohn's disease.
Hydrolyzed Versus Nonhydrolyzed Protein Diet in Short Bowel Syndrome in Children
Authors: Ksiazyk Janusz , Piena Marjolein , Kierkus Jaroslaw , Lyszkowska Malgorzata
Published: January 2024

Background

There is no consensus regarding the optimal enteral formula in patients with neonatal short bowel syndrome. The common practice in many centers is to give a semielemental diet.

Methods

To test the hypothesis that hydrolyzed protein is not superior to standard formula in promoting growth and development of children with short bowel syndrome, 10 children aged 4.08 ± 2.45 months (mean ± SD) underwent a prospective, randomized, crossover, double‐blind study lasting 60 days (with crossover on day 31). Two enteral formulas, which differed only with respect to the nitrogen form—hydrolyzed and nonhydrolyzed whey protein—were used. The endpoints of the study were nitrogen balance and intestinal permeability measured by the sugar absorption test (lactulose/mannitol excretion ratio).

Results

Energy intake from enteral formula in patients fed hydrolyzed and nonhydrolyzed formula was the same and amounted to about 31% of total intake. The ratio of total energy intake (enteral and parenteral) to resting energy expenditure was 1.7 ± 0.5 and 1.5 ± 0.3 in patients fed hydrolyzed and non hydrolyzed formula respectively. Nitrogen balance was 0.28 ± 0.05 g/kg/d and 0.29 ± 0.05 g/kg/day, respectively. Lactulose/mannitol ratio before the study was 0.85 ± 0.85 and after hydrolyzed and nonhydrolyzed formula was 0.59% ± 0.51% and 0.69% ± 0.72%, respectively.

Conclusion

Intestinal permeability, energy, and nitrogen balance in short bowel syndrome were not influenced in the short term by hydrolysis of the enteral nitrogen source.
The Role of Esophagogastroduodenoscopy in the Initial Evaluation of Childhood Inflammatory Bowel Disease: A 7‐Year Study
Authors: Abdullah Bisher A. , Gupta Sandeep K. , Croffie Joseph M. , Pfefferkorn Marian D. , Molleston Jean Pappas , Corkins Mark R. , Fitzgerald Joseph F.
Published: January 2024

Objectives

To assess the role of esophagogastroduodenoscopy in the evaluation of children with suspected inflammatory bowel disease.

Methods

All children with inflammatory bowel disease who underwent esophagogastroduodenoscopy during their initial evaluation at our institution during a 7‐year period (December 1993 to November 2000) were included in the study.

Results

The study included 115 patients: 81 with Crohn disease (mean age, 11.34 years; 42 males) and 34 with ulcerative colitis (mean age, 11.79 years; 20 males). Abnormal findings on esophagogastroduodenoscopy were noted in 64% of patients with Crohn disease and 50% of children with ulcerative colitis; histologic abnormalities were found in 81.6% and 70.6% of the patients, respectively. Granulomas were found in the upper gastrointestinal tracts of 23 of 81 patients (28.4%), with the most common site being the gastric mucosa. Nine of these 23 patients had granulomas solely in the upper gastrointestinal tract. Additional unsuspected pathology noted included: candidiasis, hiatal hernia, Helicobacter pylori infection, and giardiasis.

Conclusions

Endoscopic and histologic abnormalities were found in the upper gastrointestinal tracts of a significant number of children with inflammatory bowel disease. While the mechanism(s) underlying these abnormalities in patients with ulcerative colitis is unclear, the pathology can contribute to the patient's clinical condition. Pathology in the upper gastrointestinal tract should not exclude a diagnosis of ulcerative colitis. Granulomas, confirming the diagnosis of Crohn disease, were found in the upper gastrointestinal tracts of 28% of our patients with Crohn disease. In some cases, granulomas were found solely in the upper gastrointestinal tracts. Based on our data, esophagogastroduodenoscopy with biopsy should be performed in all pediatric patients with suspected inflammatory bowel disease.
Endoscopic Nodular Gastritis: An Endoscopic Indicator of High‐Grade Bacterial Colonization and Severe Gastritis in Children With Helicobacter pylori
Authors: Bahú Maria da Graça Soares , da Silveira Themis Reverbel , Maguilnick Ismael , Ulbrich‐Kulczynski Jane
Published: January 2024

Objective

To investigate the significance of endoscopic nodular gastritis associated with Helicobacter pylori infection.

Methods

This prospective study included 185 children (50.8% boys) aged 1 to 12 years (mean, 6.9 ± 3.0 years) who underwent upper intestinal endoscopy during evaluation of chronic abdominal pain. The authors assessed the endoscopic appearance of the stomach, noting those patients with endoscopic nodular gastritis. Urease activity of gastric mucosal biopsies was measured. With histologic examination, the presence and density of H. pylori organisms, the presence of follicular gastritis, the nature of inflammation, and the gastritis activity grade and overall gastritis score were assessed.

Results

H. pylori infection was identified in 50 children (27%). Endoscopic nodular gastritis was significantly associated with active chronic gastritis and follicular gastritis. Nodularity in the stomach showed a high specificity (98.5%) and positive predictive value (91.7%) for the diagnosis of H. pylori infection and was observed in 22 of 50 (44%) H. pylori‐positive patients and in 2 of 135 (1.5%) H. pylori‐negative patients. A significant association was observed between older age and the prevalence of this finding (P < 0.001). There was a significant increase in endoscopic nodular gastritis with increased H. pylori density and a positive correlation (Pearson coefficient = 0.97) with increased gastritis score on histologic examination. Increase in gastritis score was dependent on increased H. pylori density in patients with gastric nodularity; this finding was independent of age.

Conclusions

Endoscopic findings of antral nodularity in children suggest the presence of H. pylori infection and follicular gastritis and may identify cases of severe gastritis and marked bacterial colonization.
Aluminum Contamination of Parenteral Nutrition and Aluminum Loading in Children on Long‐Term Parenteral Nutrition
Authors: Advenier Emmanuelle , Landry Caroline , Colomb Virginie , Cognon Cécile , Pradeau Dominique , Florent Maurice , Goulet Olivier , Ricour Claude , Corriol Odile
Published: January 2024

Background

Children who are receiving parenteral nutrition are at risk of aluminum overload, which may contribute to such side effects as osteopenic bone disease. The aim of the present study is to determine the aluminum contamination of parenteral nutrition solutions and their components, and to assess the aluminum status of children on long‐term parenteral nutrition.

Methods

Aluminum concentrations were determined by graphite furnace absorption spectroscopy in components and in final parenteral nutrition solutions. The urinary aluminum excretion and plasma aluminum concentration were determined in 10 children on long‐term parenteral nutrition.

Results

The mean aluminum concentration in the administered parenteral nutrition solutions was 1.6 ± 0.9 μmol × l−1(mean ± standard deviation (SD)). The resulting mean aluminum daily intake of the 10 patients was 0.08 ± 0.03 μmol × kg−1 × day−1.

Conclusions

Compared to two previous studies performed in 1990 and in 1995 in our hospital, the aluminum contamination of parenteral nutrition solutions and the daily aluminum intake of the children seemed to decrease. However, the plasma aluminum concentration and daily urinary aluminum excretion of the children still remain above normal standards. The children had no clinical symptoms of bone disease but aluminum accumulation in tissue can not be excluded. To prevent this iatrogenic toxicity, the aluminum contamination of parenteral nutrition should be assessed regularly.
The Nature of Colitis in Chronic Granulomatous Disease
Authors: Schäppi Michela G. , Klein Nigel J. , Lindley Keith J. , Rampling Dyanne , Smith Virpi V. , Goldblatt David , Milla Peter J.
Published: January 2024

Background

Patients with chronic granulomatous disease (CGD) may have gastrointestinal manifestations, commonly colitis. The etiology, prevalence, and inflammatory process of CGD colitis are unclear.

Objectives

To characterize the inflammatory process of CGD colitis and to compare it with other inflammatory bowel disorders.

Methods

Colonic mucosal biopsies from 8 CGD patients were immunostained for eosinophils, neutrophils, macrophages, and adhesion molecules (ICAM; VCAM, E‐selectin) and compared with normal and diseased controls (allergic colitis, ulcerative colitis, and melanosis coli). Cell types were counted and expressed as cell/mm2

Results

The inflammatory infiltrate in CGD colitis differed from the normal controls by an increase in eosinophils (110; 48–176 [median and range] versus 14.5; 3–30;P < 0.005) and macrophages (291.5; 203–480 versus 38.5; 27–64;P < 0.005). There was a paucity of neutrophils compared to ulcerative colitis (10; 0–101 versus 315.5; 78–688;P < 0.005). Expression of HLA‐DR was increased in the epithelium and vascular endothelium in CGD compared with normal controls. Patterns of expression of the adhesion molecules differed significantly in CGD from those in other inflammatory bowel diseases: intracellular adhesion molecule‐1 was more strongly expressed in the lamina propria, vascular adhesion molecule‐1 was more patchily expressed, and E‐selectin was present only in the small vessels.

Conclusions

The mechanism of inflammation and profile of inflammatory mediators in CGD colitis differs from that in other inflammatory bowel diseases.
Autoimmune Thyroid Disease and Celiac Disease in Children
Authors: Ansaldi Nicoletta , Palmas Tiziana , Corrias Andrea , Barbato Maria , D'Altiglia Mario Rocco , Campanozzi Angelo , Baldassarre Mariella , Rea Francesco , Pluvio Rosanna , Bonamico Margherita , Lazzari Rosanna , Corrao Giovanni
Published: January 2024

Background:

Celiac disease (CD) may be associated with other immunologic disorders in adults and children. Previous studies linking CD and autoimmune thyroid disease in children have included very few patients with limited biochemical and immunologic screening tests. The aim of this multicenter study was to establish the prevalence of autoimmune thyroid involvement in a large series of pediatric patients with CD.

Methods:

Five hundred seventy‐three consecutive pediatric patients were enrolled from clinics in Torino, Bologna, Foggia, Rome (two clinics), Naples, and Bari. Three hundred forty‐three patients with CD were studied, 230 girls and 113 boys (median age, 8.5 years). Two hundred fifty‐six of the patients with CD (median age, 9 years) had been following a gluten‐free diet for 3 months to 16 years; 87 patients were untreated (median age, 6.2 years). The diagnosis of CD was made using the European Society for Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) criteria. A control group of 230 subjects (median age, 8.3 years) was enrolled. Serum free triiodothyronine, free thyroxine, and thyroid‐stimulating hormone (TSH), antithyroperoxidase, antithyroglobulin, anti‐TSH receptor antibodies, and thyroid echographic pattern were considered.

Results:

Autoimmune thyroid disease was found in 90 of 343 (26.2%) patients with CD (62 on a gluten‐free diet) and in 20 (10%) of the control subjects (P = 0.001). Fifty‐four (15.7%) patients with CD and autoimmune markers had normal thyroid function (euthyroidism) as did 12 (6.0%) of the control subjects; hypothyroidism was observed in 28 (8.1%) patients with CD and in 7 (3.5%) of the control subjects. Hyperthyroidism was diagnosed in four patients with CD and in none of the control subjects with autoimmune markers. An abnormal echographic pattern was seen in 37 patients with CD (16.8%) and only in 1 (1.6%) of the control subjects (P = 0.002).

Conclusions:

The high frequency of autoimmune thyroid disease found among patients with CD, even those on a gluten‐free diet, may justify a thyroid status assessment at diagnosis and at follow‐up evaluation of children with CD. JPGN 37:63‐66, 2003.
Esophageal Eosinophilia In Children With Dysphagia
Authors: Cheung Ka Ming , Oliver Mark R. , Cameron Donald J. S. , Catto‐Smith Anthony G. , Chow Chung W.
Published: January 2024

Objectives

Children occasionally have dysphagia in the absence of an apparent primary cause. Esophageal eosinophilia is sometimes seen in these patients at the time of upper endoscopy but its significance is not clear. Although eosinophilia is regarded by some as a histologic hallmark of childhood reflux esophagitis, it may in fact signal a primary eosinophilic esophagitis in children with dysphagia. Our aim was to evaluate esophagitis, acid reflux determined by pH probe, and esophageal eosinophilia in children with the primary complaint of dysphagia.

Methods

A retrospective study was performed in 42 children, admitted for investigation of dysphagia, in whom no primary cause could be found. Twenty‐one children (mean age ± SD, 10.1 ± 4.0 years) had esophageal eosinophilia and 21 children (8.3 ± 4.7 years) did not. Clinical, endoscopic, manometric and esophageal pH parameters in these two groups were compared.

Results

Patients with esophageal eosinophilia were more often male (p<0.01) with a history of allergy (p<0.001) and food bolus obstruction (p<0.05) requiring endoscopic removal. Their esophageal mucosa appeared wrinkled and thickened at endoscopy with basal cell proliferation, and large numbers of eosinophils in esophageal mucosal biopsies. Continuous esophageal pH records and motility studies, when obtained, were similar in both groups and were within normal values.

Conclusion

Children with dysphagia who have esophageal eosinophilia are unlikely to have pathologic gastroesophageal reflux.
A Randomized, Controlled Trial of Parenteral Glutamine in Ill, Very Low Birth‐weight Neonates
Authors: Thompson S. W. , McClure B. G. , Tubman T. R. J.
Published: January 2024

Objective

The role of “novel substrates” in neonatal nutrition has generated much interest in recent years. Glutamine has been recognized as a “conditionally essential” amino acid in critically ill adults, particularly for gut and immune function; however, its potential role in the neonate remains unclear. The authors examined the safety and benefits of parenteral glutamine in ill, preterm neonates.

Design

Randomized controlled trial.

Methods

Thirty‐five ill preterm neonates of <1000 g birth‐weight were randomized to receive either glutamine‐supplemented parenteral nutrition (PN) (n = 17) or standard PN (n = 18).

Results

There were no significant differences in birth‐weight, gestational age, male‐to‐female ratio, or Clinical Risk Index for Babies (CRIB) score between the two groups. During PN there were no significant differences between the groups in white cell count, differential white cell count, blood urea nitrogen, plasma ammonia, lactate, pyruvate, plasma glutamine, or glutamate. The median time to achieving full enteral nutrition (FEN) was shorter in the study group (13 days vs. 21 days, P < 0.05). The number of episodes of culture‐positive sepsis or age at discharge did not differ between groups.

Conclusions

Parenteral glutamine appears to be well tolerated and safe in the ill, preterm neonate. It may reduce the time to achieving FEN.
Gastroesophageal Reflux Disease and Helicobacter pylori Infection in Neurologically Impaired Children: Inter‐relations and Therapeutic Implications
Authors: Pollet Séverine , Gottrand Frédéric , Vincent Pascal , Kalach Nicolas , Michaud Laurent , Guimber Dominique , Turck Dominique
Published: January 2024

Background

The aim of this retrospective study was to assess the relationship between Helicobacter pylori infection and gastroesophageal reflux disease in a high‐risk population of children.

Methods

Forty‐three neurologically impaired pediatric patients with H. pylori had upper gastrointestinal endoscopy between 1990 and 2000. Infection was confirmed by positive H. pylori culture or by identification of organisms in gastric biopsy specimens (fundus, n = 2; antrum, n = 3). Reflux esophagitis was diagnosed by ulceration of the esophageal mucosa at endoscopy. Four to 6 weeks after the completion of antibiotic treatment of H. pylori, a second endoscopy was performed and the gross appearance of the esophagus was recorded.

Results

At the first endoscopy, esophagitis was noted in 14 of 43 patients. After treatment, H. pylori infection was eradicated in all 14 patients with esophagitis but in only 19 of 29 (66%) of those with normal esophagus (P = 0.01). Esophagitis was still present in 4 of 14 (29%) patients who had esophagitis at the first endoscopy. Persistent esophagitis was only related to the presence of esophagitis before treatment (P = 0.02). In 29 patients with a normal esophagus at the first endoscopy, only one case of esophagitis was observed after H. pylori eradication.

Conclusion

The data suggest that treatment of H. pylori infection should be considered in children with concomitant GERD, and such treatment is unlikely to either induce or exacerbate peptic esophagitis.
Nutritional Status and Gastrointestinal Structure and Function in Children With Ichthyosis and Growth Failure
Authors: Fowler Ashley J. , Moskowitz David G. , Wong Anthony , Cohen Sharon P. , Williams Mary L. , Heyman Melvin B.
Published: January 2024

Growth failure occurs in several of the ichthyoses, a heterogeneous group of inherited disorders characterized by thickened or scaly skin. This suggests that there may be common pathogenic mechanisms causing failure to thrive. Previous studies have proposed that a hypermetabolic state induced by epidermal inflammation and hyperproliferation or enteropathy leading to malabsorption and nutritional deficiencies might account for the growth failure in icthyosis.

Objective

The purpose of this study was to examine the extent of enteropathy and nutritional deficiency in children with severe ichthyosis and growth failure.

Methods

Ten children with different types of ichthyoses and growth failure were studied.

Results

Evaluation of gastrointestinal structure and function revealed few abnormalities other than mild fat malabsorption in two patients. Total caloric intake exceeded established requirements for age, height, and weight in all patients. Deficiencies of fat‐soluble vitamins were identified in a few subjects. Mild elevations in total calcium and magnesium blood level, elevated hematocrit levels, and constipation were observed in many patients.

Conclusions

Nutritional deficiencies and gastrointestinal abnormalities are uncommon in children with ichthyosis and failure to thrive. Chronic hypovolemia caused by impaired epidermal permeability barrier may be common in this population.