bluuebunny/crossref_metadata_2025_split huggingface dataset:
Ultrasound Studies of the Intestinal Wall in Patients with Cystic Fibrosis
Authors: Pohl M. , Krackhardt B. , Posselt H. G. , Lembcke B. 
Published: February 2024

Background:In 1994, first published reports described cystic fibrosis patients who experienced a then unknown complication‐ileocecal and colonic stenoses with submucosal proliferation requiring surgical intervention. To investigate a suspected correlation between increased intestinal wall diameter and high doses of pancreatic enzymes, we carried out a prospective study in our CF‐outpatient clinic.Methods:By ultrasound analysis we measured the intestinal wall diameter in 201 patients. One hundred ninety‐three patients treated with pancreatic enzymes had pancreatic insufficiency. Eight patients showed normal pancreatic function, seven of them had never been treated with pancreatic enzymes. The control group included 12 healthy children. Measuring points were the distal ileum, cecum, ascending, and descending colon. Measurements were made by the longitudinal and cross sectional cut. The following aspects of the patients' history were recorded (a) current type of pancreatic enzyme medication; (b) total dosage per day (with reference to lipase units); (c) duration of therapy with standard‐strength pancreatic enzyme (SSPE) preparations (≤10,000 lipase units per capsule) and HSPE preparations (≥20,000 lipase units per capsule); (d) gastrointestinal complication (distal intestinal obstruction syndrome, meconium ileus, abdominal surgery, intussusception), diabetes mellitus, and hepatobiliary complications.Results:The intestinal wall diamater in patients receiving HSPE therapy was greater (with prominent submucosal layer) than that in patients receiving SSPE therapy or in patients with pancreatic sufficiency. Healthy subjects had the smallest intestinal wall diameter. There was no correlation between patient history and increased intestinal wall thickness.Conclusions:Ultrasound detects characteristic ileocoecal wall lesions in the majority of cystic fibrosis patients on pancreatic enzymes. These lesions may lead to significantly increased ileocoecal wall thickness, which is correlated but not restricted to HSPE.
Abnormal Bile Acid Metabolism and Neonatal Hemochromatosis: A Subset with Poor Prognosis
Authors: Siafakas Constantinos G. , Jonas Maureen M. , Perez‐Atayde Antonio R. 
Published: February 2024

Background:Inborn errors of bile acid synthesis are newly recognized disorders that may cause the phenotypic appearance of neonatal hepatitis or neonatal cholestasis.Methods:This is a clinicopathologic study of two sets of siblings with cholestatic neonatal liver failure.Results:In 3 of the infants, diagnostic evaluation, including analysis of urinary bile salts, revealed a predominance of 7α‐hydroxy‐3‐oxo‐4‐cholenoic and 7α, 12α‐dihydroxy‐3‐oxo‐4‐cholenoic acids, a pattern consistent with Δ4‐3‐oxosteroid 5β‐reductase deficiency, which could be primary or secondary. The fourth infant died before such testing could be carried out. In addition, all 4 infants had histologically disseminated hemochromatosis and met diagnostic criteria for neonatal hemochromatosis. In the 3 infants studied, histologic examination of the liver disclosed giant cell hepatitis with extensive loss of hepatic parenchyma and rapid progression to cirrhosis. Early treatment with ursodeoxycholic acid and cholic acid, previously reported as effective therapy, was given to 2 siblings; it failed to reverse or halt the liver damage, and both infants died. One infant, with the original diagnosis of neonatal hemochromatosis, was treated with a variety of antioxidants and chelation therapy, as recently reported. No improvement was demonstrated, and he went on to liver transplantation.Conclusions:The presentation of Δ4‐3‐oxosteroid 5β‐reductase deficiency as neonatal hemochromatosis may represent a distinct subset of this disorder with an accelerated course, no response to therapy and poor prognosis.
Spermine Induces Maturation of the Immature Intestinal Immune System in Neonatal Mice
Authors: ter Steege Jessica C. A. , Buurman Wim A. , Forget Pierre‐Philippe 
Published: February 2024

Background:In mice, immunological adaptation of the gut to microbial and nutritional antigens occurs at weaning in parallel with biochemical and morphological maturation. Because oral administration of spermine to neonatal rats has been shown to induce biochemical and morphological maturation, we investigated whether spermine also affects maturation of the mucosal immune system.Methods:Swiss mice 7, 12, and 27 days old were given spermine orally (0.5 μmol/g body weight) during 3 days. Intestinal length was measured, and lactase and sucrase activities were determined. The phenotype of intraepithelial and lamina propria lymphocytes was assessed by FACS analysis using markers for CD3, TCRαβ, TCRγδ, CD4, CD8α, CD8β, CD5, CD18, CD54, and CD49d.Results:Similar to what occurs during natural development, spermine treatment of neonatal mice increased intestinal length, decreased lactase activity, and increased sucrase activity. The percentage of intraepithelial lymphocytes expressing TCRαβ, CD4, CD5, and CD54, as well as the levels of expression of these antigens, increased after spermine treatment on day 12, similarly to natural maturation. The increase in expression of CD3, TCRγδ, CD18, and CD49d did not reach statistical significance. No effect was observed on CD8 expression. The phenotype of lamina propria lymphocytes was not affected. Spermine administration to 7‐ and 27‐day‐old mice had no effect on the phenotype of either intraepithelial or lamina propria lymphocytes.Conclusions:Oral spermine administration to neonatal mice induced, in parallel with biochemical maturation, precocious maturation of the murine intestinal immune system and particularly affected differentiation of the intraepithelial lymphocyte population.
Is a Gastric Drainage Procedure Necessary at the Time ofAntireflux Surgery?
Authors: Brown Robin A. , Wynchank Sinclair , Rode Heinz , Millar Alastair J. W. , Mann Michael D. 
Published: February 2024

BackgroundGastroesophageal reflux is part of a generalized foregut motility disorder, which may also include delayed gastric emptying. With persistence of gastroesophageal reflux, or the presence of complications, including recurrent aspiration syndrome and esophageal stricture formation, surgical correction may be indicated. It is uncertain whether a procedure to resolve delayed gastric emptying is indicated at this time as well.MethodsSixty‐seven children with proven gastroesophageal reflux had preoperative gastric emptying assessed using 99Technetium‐Sn‐colloid labelled milk. Delayed gastric emptying was defined as a gastric residual activity of more than 40% at 2 hours after feeding. The antireflux operation was a partial anterior fundoplication. Postoperative milk scans assessed the effect of surgery on gastric emptying.ResultsGastric emptying at 2 hours improved overall from a median of 22% before surgery to 17% after surgery. In 17 patients delayed gastric emptying was identified before surgery; in 15 of those it returned to within normal limits after surgery. In 50 children with normal gastric emptying before surgery (gastric residual activity at 2 hours 16%), 14 (28%) showed delayed gastric emptying in the postoperative scan.ConclusionsDelayed gastric emptying is common in children who undergo surgery for gastroesophageal reflux disease. A partial anterior fundoplication antireflux operation improves gastric emptying to within normal limits in the majority (88%) in this group, rendering a synchronous gastric drainage procedure unnecessary.
Simultaneous Determination of Fecal Fat, Nitrogen, and Water by Near‐Infrared Reflectance Spectroscopy
Authors: Neumeister Volker , Henker Jobst , Kaltenborn Georg , Sprössig Christa , Jaross Werner 
Published: February 2024

BackgroundDeterminations of fecal fat and nitrogen reveal evidence of malabsorption and assist in estimating the efficacy of pancreatic enzyme treatment. Seventy‐two‐hour stool collection, with chemical analysis of fecal fat, and Kjeldahl's method for measurement of fecal nitrogen are generally accepted as standard methods for making these determinations. However, these traditional methods are expensive, timeconsuming, and cumbersome. This study evaluated the efficiency and validity of an alternative method, using nearinfrared reflectance spectroscopy (NIRS) and compared results with those of the standard methods.MethodsNear‐infrared reflectance spectroscopy is a secondary method: The instrument first has to be calibrated with samples analyzed by the standard method. Sixty‐three stool samples with known fat content (range 4.79‐292.5 mg/g), 24 samples with known nitrogen content (range 5.36‐19.38 mg/g), and 24 samples with known water concentration (range 60.1‐82.22%) served for calibration. A further 69 samples were analyzed to validate the procedure.ResultsThere was a satisfactory correlation between the measurements produced by near‐infrared reflectance spectroscopy and those produced by standard methods: fat r = 0.97; nitrogen r = 0.94; water r = 0.96.ConclusionsNear‐infrared reflectance spectroscopy appears to be a reliable, simple, and rapid method of measuring different fecal components‐as precise and accurate as the standard methods. Stool samples should be analyzed immediately after collecting or stored only a few days before analyzing.
Outcome After Ileoanal Anastomosis in Pediatric Patients with Ulcerative Colitis
Authors: Durno Carol , Sherman Philip , Harris Kim , Smith Claire , Dupuis Annie , Shandling Barry , Wesson David , Filler Robert , Superina Riccardo , Griffiths Anne 
Published: February 2024

ABSTRACTBackground:To review the outcome after restorative proctocolectomy among children and adolescents with ulcerative colitis at a pediatric inflammatory bowel disease center.Methods:The records of all patients with ulcerative colitis undergoing colectomy and ileoanal anastomosis at The Hospital for Sick Children, Toronto, Canada, were reviewed. Questionnaires concerning functional results were sent to patients with restored transanal defecation.Results:Seventy three patients (mean age, 13.2 years; range, 2.6‐18.8 years) underwent ileoanal anastomosis (19 straight ileoanal anastomosis, 41 J pouch, 13 S pouch) between January 1980 and June 1995 and were observed 5.8 ± 3.3 years. The ileoanal anastomosis is nonfunctional in 19 (26%) patients. Excision rates according to type of restorative procedure were J pouch, 7% (3 of 41); S pouch, 32% (4 of 13); and straight ileoanal anastomosis, 32% (6 of 19). Failure was usually attributable to intractable diarrhea among patients with straight ileoanal anastomosis but was caused by anastomotic leak or pelvic‐perianal sepsis among patients with pouch procedures. Failure rates did not vary with age at ileoanal anastomosis. Among patients retaining ileoanal continuity, continence problems reported in the questionnaire were frequent and tended to be more extreme among younger patients. Overall, 90% of respondents reported satisfaction with the functional outcome of the restorative operation.Conclusions:The success rate of the ileoanal anastomosis/J‐pouch procedure is comparable to that in adult series. The ileoanal anastomosis/J‐pouch procedure is the operation of choice for children and adolescents who want ileoanal continuity restored after colectomy for ulcerative colitis.
The Gluten‐Free Diet: A Nutritional Risk Factor for Adolescents with Celiac Disease?
Authors: Mariani Paolo , Viti Maria Grazia , Montouri Monica , La Vecchia Alessandra , Cipolletta Elsa , Calvani Luisa , Bonamico Margherita 
Published: February 2024

ABSTRACTBackground:The gluten‐free diet is the standard therapy for patients affected by celiac disease, although compliance with the diet is not optimal in adolescents or adults. Moreover, the gluten‐free diet may induce nutritional imbalances.Methods:Alimentary habits and diet composition were examined in 47 adolescents with celiac disease and 47 healthy aged‐matched control subjects. All subjects compiled a 3‐day alimentary record that allowed determination of their energy intakes; the macronutrient composition of their diets; and their iron, calcium, and fiber intakes. To evaluate compliance with the gluten‐free diet, immunoglobulin A antigliadin and antiendomysium antibodies were assessed in all with celiac disease.Results:The analysis of the records and the results of antibody levels showed that 25 subjects strictly followed dietetic prescriptions (group 1A), whereas 22 patients consumed gluten‐containing food (group 1B). Those with celiac disease and control subjects (group 2) consumed a normocaloric diet. Lipid and protein consumption was high, however, and the consumption of carbohydrates low. Moreover, dietary levels of calcium, fiber, and especially in girls, iron, were low. These nutritional imbalances were significantly more evident in group 1A than in group 1B, as a consequence of poor alimentary choices. Moreover, in group 1A overweight and obesity were more frequent (72%) than in group 1B (51%) and in the control subjects (47%).Conclusions:In people with celiac disease, adherence to a strict gluten‐free diet worsens the already nutritionally unbalanced diet of adolescents, increasing elevated protein and lipid consumption. In the follow‐up of patients with celiac disease, considerable effort has yet to be made to improve compliance with a gluten‐free diet, and especially to control the nutritional balance of the diet in compliant patients.
Presence of Human Papillomavirus in Extrahepatic Biliary Atresia
Authors: Drut Ricardo , Drut Rosa Mónica , Gómez María Atilia , Rúa Eduardo Cueto , Lojo María Mercedes 
Published: February 2024

ABSTRACTBackground:In a previous study the human papillomavirus DNA was detected in seven cases of so‐called idiopathic neonatal giant cell hepatitis by using nested polymerase chain reaction. The purpose of the present study was to study the prevalence and possible common causes of human papillomavirus‐associated idiopathic neonatal giant cell hepatitis and extrahepatic biliary atresia.Methods:Formalin‐fixed, paraffin‐embedded archival tissues obtained in 18 cases of extrahepatic biliary atresia were studied for human papillomavirus DNA by nested polymerase chain reaction. In addition, in situ hybridization was performed on tissue obtained in 6 cases.Results:Tissue in 16 of the 18 cases studied showed amplified human papillomavirus DNA, whereas no human papillomavirus was amplified in any of 30 control samples. Main human papillomavirus detected were types 6 and 18. Punctate intranuclear positive signals were detected in the hepatocytes after in situ hybridization for human papillomavirus DNA.Conclusions:The high prevalence of human papillomavirus DNA in liver tissue in cases of extrahepatic biliary atresia suggests a strong correlation between this disorder and idiopathic neonatal giant cell hepatitis. It further suggests that this virus may be one of the causative agents in extrahepatic biliary atresia and may represent part of the spectrum of lesions associated with neonatal human papillomavirus‐induced hepatic damage.
Helicobacter pylori Reinfection Rates in Children After Eradication Therapy
Authors: Kato Seiichi , Abukawa Daiki , Furuyama Noriko , Iinuma Kazuie 
Published: February 2024

ABSTRACTBackground:There are few studies of Helicobacter pylori reinfection in childhood. In the current study the reinfection rate of H. pylori and ulcer recurrence were investigated during a follow‐up period of 12 months or more in children who had undergone eradication therapy.Methods:Twenty‐seven patients aged 5 to 16 years (6 with gastric ulcer, 13 with duodenal ulcer, and 8 with nodular gastritis) were studied. Biopsy‐based H. pylori tests performed 1 to 2 months after eradication therapy demonstrated that eradication was successful in 23 patients (5 with gastric ulcer, 11 with duodenal ulcer, and 7 with nodular gastritis) and unsuccessful in 4 (1 with gastric ulcer, 2 with duodenal ulcer, and 1 with nodular gastritis). Twenty‐three successfully treated patients were observed for a mean of 22 months (a total of 42.2 patient years of follow‐up). To assess H. pylori status, all 23 patients underwent a 13C‐urea breath test 1 year after eradication therapy. If the test result was negative, the patients underwent the follow‐up test once every year thereafter. In successfully and unsuccessfully treated patients, endoscopy was performed if a patient reported symptoms suggesting ulcer recurrence.Results:The initial follow‐up 13C‐urea breath tests showed that all 23 patients remained free of infection at 12 to 19 months. Among 17 patients, the second test confirmed reinfection in 1 at 28 months. In two patients studied, the third test showed a negative result. The reinfection rate was 2.4% per patient year. Over the follow‐up period, ulcer recurrence was found in 2 of 3 ulcer patients with eradication failure but in none of the 16 ulcer patients with successful eradication. The recurrence rate was significantly lower in successfully treated patients than in unsuccessfully treated patients (p < 0.05).Conclusions:Reinfection with H. pylori is rare in children aged more than 5 years, and successful eradication significantly reduces ulcer recurrence. This study supports the benefit of eradication therapy in older children.
Abdominal Migraine: Prophylactic Treatment and Follow‐up
Authors: Worawattanakul Mingmuang , Rhoads J. Marc , Lichtman Steven N. , Ulshen Martin H. 
Published: February 2024

ABSTRACTBackground:Abdominal migraine is a syndrome characterized by recurrent stereotypic episodes of paroxysmal abdominal pain and nausea and/or vomiting with wellness between episodes. It is often associated with a positive family history of migraine and no other apparent underlying disease. The purpose of this study was to report in patients diagnosed with abdominal migraine the outcome, the effect of prophylactic treatment, and the duration of treatment.Methods:The records of 53 patients who underwent treatment after a diagnosis of abdominal migraine were retrospectively reviewed. Responses to treatment were graded as excellent (cessation of recurrent abdominal pain), fair (persistence of symptoms but milder and less frequent), or poor (no response). Follow‐up data were available in 38 patients. Twenty‐four patients were treated with propranolol and 12 with cyproheptadine. Four were not treated because of mild and infrequent symptoms.Results:Among the children treated with propranolol, 18 (75%) had an excellent response, 2 (8%) had a fair response, and 4 (17%) had no response. In those treated with cyprohepatadine, 4 (33%) had an excellent response, 6 (50%) had a fair response, and 2 (17%) had no response. Patients were instructed to continue medication for 6 months or until cycles had stopped. However, 11 of 24 patients (46%) in the propranolol group took medication for less than 6 months and the remaining patients from 6 months 3 years. Six patients in the cyproheptadine group (50%) took medication less than 10 months and the remaining patients for 10 months to 3 years.Conclusion:Patients with abdominal migraine may benefit from prophylactic treatment with propranolol or cyproheptadine.
Dietary Lipids Influence Intestinal Adaptation After Massive Bowel Resection
Authors: Kollman Kathryn A. , Lien Eric L. , Vanderhoof Jon A. 
Published: February 2024

ABSTRACTBackground:Certain lipids, primarily long chain fatty acids and especially long chain polyunsatured fatty acids (LCPUFAs) from marine oils, stimulate gut adaptation after resection. The goal of this study was to define the degree of resection that provides an optimal model for adaptation and to determine if dietary LCPUFAs improve intestinal adaptation after resection.Methods:One hundred and fifty‐g male Sprague‐Dawley rats were divided into groups receiving 60%, 70%, and 80% bowel resection. After resection, each group was subdivided into two dietary groups and pair fed diets containing either safflower oil or docosahexaenoic acid (DHA) and arachidonic (AA).Results:After 2 weeks, mucosal mass, protein, DNA, and disaccharidase activity were measured in the remaining intestine. Rats receiving 80% resection responded with the highest level of intestinal adaptation. Within the 80% resection group, diet containing DHA and AA stimulated adaptation significantly more than safflower diet. A second study further evaluated the effect on LCPUFAs on intestinal adaptation. Diets included a control group 10% soy oil, and three diets differing in their AA‐DHA fat blend ratio at 5% AA and 3.3% DHA, 15% AA and 10% DHA, and 45% AA and 30% DHA. The addition of LCPUFAs to diets enhanced intestinal adaptation in a linear, dose‐dependent manner after an 80% small bowel resection. Rats fed a diet containing 30% DHA‐45% AA had significantly enhanced mucosal mass compared to rats fed a diet containing 10% soy oil, and considerably higher compared to rats fed 3.3% DHA‐5% AA.Conclusions:These studies suggest that modification of dietary LCPUFAs may enhance intestinal adaptation in short bowel syndrome.
Clinical Applications of Technetium Tc 99m Hexamethyl Propylene Amine Oxime Leukocyte Scan in Children with Inflammatory Bowel Disease
Authors: Del Rosario Marian A. , Fitzgerald Joseph F. , Siddiqui Aslam R. , Chong Sonny K. , Croffie Joseph M. , Gupta Sandeep K. 
Published: February 2024

ABSTRACTBackground:Labeled leukocyte imaging is a helpful diagnostic tool in the detection of inflammation and sepsis. The technetium TC 99m hexamethyl propylene amine oxime (99mTc HMPAO)‐labeled leukocyte scan has been found to be more sensitive than the Indium‐111 labeled leukocyte scan in detecting inflammatory bowel disease, with reported sensitivities of 95% to 100%. Experience with the 99mTc HMPAO‐labeled leukocyte scan was examined and its clinical applications evaluated in the immediate treatment of patients with inflammatory bowel disease.Methods:A retrospective chart review was undertaken that included pediatric patients who underwent 99mTc HMPAO‐labeled leukocyte scan at the James Whitcomb Riley Hospital for Children. The disease activity of patients with inflammatory bowel disease was assessed. The leukocyte scan was performed according to the manufacturer's specifications, and images were obtained 30 minutes and 2 hours after administration of the radiopharmaceutical.Results:During the period of July 1996 through November 1997, 41 scans were performed in 35 patients. Twenty‐nine patients had histologically proven inflammatory bowel disease: 24 with Crohn's disease, 4 with ulcerative colitis, and 1 with indeterminate colitis. Active inflammatory bowel disease was suspected in 24 patients when the leukocyte scan was performed. Twenty of the 24 patients (83% sensitivity) had abnormal findings in leukocyte scans that prompted more aggressive management in 15 (75%). Six of the 15 who were receiving maximum medical therapy underwent surgical resection of severely affected bowel segments, and medical treatment was intensified in the other 9. The remaining 5 patients were receiving optimal medical therapy, instituted at their recent visit, and did not require further medication adjustments. Four of the 24 patients with active inflammatory bowel disease had normal leukocyte scans (17% false‐negative rate), 3 of whom were receiving corticosteroid therapy at the time the scans were performed. All of the 11 patients in whom inflammatory bowel disease was in remission and 6 patients who did not have inflammatory bowel disease had normal findings in leukocyte scans (100% specificity).Conclusions:Although a tissue diagnosis is still recommended, obtained during upper and lower gastrointestinal endoscopic examinations, and contrast radiography of the small bowel for the initial work‐up of patients with suspected inflammatory bowel disease, the 99mTc HMPAO‐labeled leukocyte scan is a safe and useful diagnostic adjunct for subsequent evaluation of patients known to have inflammatory bowel disease. The results of 99mTc HMPAO‐labeled leukocyte scans directly influenced treatment of 75% of the study patients with active inflammatory bowel disease, which included the decision to refer patients for surgical intervention.