bluuebunny/crossref_metadata_2025_split huggingface dataset:
Imaging Changes in the Pancreas in Cystic Fibrosis: A Retrospective Evaluation of 55 Cases Seen Over a Period of 9 Years
Authors: Feigelson Jean , Pécau Yvette , Poquet Myriam , Terdjman Patricia , Carrère Jacqueline , Chazalette Jean‐Pierre , Ferec Claude 
Published: February 2024

ABSTRACTBackground:Pathologic changes of the pancreas have been observed as early as the recognition of the disease termed initially “cystic fibrosis of the pancreas”. Atrophy of the gland and its fatty infiltration were considered as usual features. The aim of this study was to follow‐up the evolution of cystic fibrosis pancreas and to define its successive stages in correlation with the clinical, biochemical, and imaging findings.Methods:Fifty‐five patients were followed up during 9 years. The patients' genetic backgrounds were systematicaly performed. Blood lipase levels were analyzed systematically at each consultation of the patients and in the event of bouts of abdominal pains. Imaging using mainly echograms and tomodensitometric scans were regularly performed: echograms every 6 months, and tomodensitometric scans every 1 to 2 years. Magnetic resonance imaging was performed in four patients.Results:Five groups of patients were identified on the basis of tomodensitometric scan findings: normal pancreas (n = 4), incomplete lipomatosis of the pancreas (n = 9), complete lipomatosis of the pancreas (n = 23), cystic pancreas (n = 5), macrocystic pancreas (n = 1), atrophic pancreas (n = 13). Pancreas exocrine function was not correlated with findings. Forty episodes of pancreatitis were observed in seven patients. They had bouts of abdominal pain and elevation of lipase levels. Five of these patients were composite heterozygotes (D508/other). Incomplete lipomatosis represents an intermediate stage leading toward complete lipomatosis or toward atrophy after pancreatitis.Conclusions:Studies of pancreatic function should be performed routinely in cystic fibrosis, especially in pancreatic sufficiency or in patients with normal pancreas images. Acute pancreatitis should be diagnosed and properly identified to be differentiated from other acute abdominal syndromes occurring in cystic fibrosis.
Long‐Term Nutritional and Neurodevelopmental Outcome of Liver Transplantation in Infants Aged Less Than 12 Months
Authors: van Mourik Indra D. M. , Beath Sue V. , Brook Gill A. , Cash A. John , Mayer A. David , Buckels John A. C. , Kelly Deirdre A. 
Published: February 2024

ABSTRACTBackground:Liver transplantation is established treatment for children with end‐stage liver disease and has a 5‐year survival rate of 80% to 85%, even in infants under 12 months. Long‐term outcome in nutritional rehabilitation and normal development is unknown. This study aimed to prospectively evaluate growth and psychoneurologic performance of children who undergo liver transplantation in infancy.Methods:Twenty‐five infants (18 girls, 7 boys) who underwent liver transplantation at less than 12 months of age (median age, 9 months) were evaluated for 4 years. Growth measurements were expressed as standard deviation scores (SDSs; mean ± SEM), and psychoneurologic performance was assessed with the unrevised Griffiths Mental Ability Scales (normal range, 80–120).Results:Four children died during the study (4‐year survival, 84%). The children were malnourished before transplantation (SDSs: weight, −1.9 ± 0.2; midarm muscle area, −0.93 ± 0.3; midarm fat area, −1.52 ± 0.3; and height, −0.95 ± 0.3). Nutritional rehabilitation for all parameters occurred within 12 to 24 months after transplantation, which was most significant for weight (−1.1 ± 0.2, P = 0.001), midarm muscle area (0.74 ± 0.3, P = 0.001), and midarm fat area (−0.44 ± 0.3, P = 0.01). There was some improvement in height (−0.72 ± 0.3, P = 0.14), which was not significant, although infants who were severely stunted before transplantation (mean height standard deviation score [SDS] −2.46) showed significant catch‐up at 1 year after transplantation (mean height SDS −1.2, P = 0.003). Psychoneurologic scores were within normal limits before transplantation and were maintained for the 4‐year follow‐up period, although individual scores varied during this period. Improved nutritional status was associated with increased muscle bulk and subsequent improvement in motor scores from 90.6 at initial assessment to 97.3 at 4 years (P = 0.28). There was a temporary reduction in social skills and eye–hand coordination in the first year, which may have been an effect of the hospital environment or cyclosporine immunosuppression. Language abilities also regressed during the first year, possibly related to the effect of nasogastric tube feeding in delaying normal speech development.Conclusions:Liver transplantation in infancy has not only a successful outcome but is also associated with long‐term catch‐up growth and nutrition and maintenance of normal development.
Lactobacilli and Acidosis in Children With Short Small Bowel
Authors: Bongaerts Ger , Bakkeren Jan , Severijnen René , Sperl Wolfgang , Willems Hans , Naber Ton , Wevers Ron , van Meurs Alfred , Tolboom Jules 
Published: February 2024

ABSTRACTBackground:In patients with a short small bowel, D‐lactic acidemia and D‐lactic aciduria are caused by intestinal lactobacilli. The purpose of this study was to obtain a detailed picture of the metabolic acidosis in young children with short small bowel.Methods:Feces, blood, and urine of children with short small bowel and acidosis were studied microbiologically and/or biochemically.Results:Previous findings were confirmed that more than 60% of the fecal flora of patients with small short bowel, who are not receiving antibiotics, consists of lactic acid‐producing lactobacilli. In blood, D‐lactic acid was the most prominent metabolite: the highest serum D‐lactate (15.5 mmol/l) was observed in a sample taken immediately after the onset of hyperventilation. The highest D‐lactate excretion was in urine collected some hours after the onset of hyperventilation, and amounted to 59 mol/mol creatinine. Acidosis in the patients with short small bowel was related to strongly increased serum D‐lactate and anion gap and to strongly decreased serum bicarbonate and pH.Conclusion:In children with small short bowel and acidosis, the common intestinal flora of mainly lactobacilli abundantly produces D‐lactic acid from easily fermentable carbohydrates. Thus, these bacteria directly cause shifts of bicarbonate, pH, and base excess and indirectly cause shifts of the anion gap, as well as hyperventilation. These kinetic parameters are strongly associated.
Prevalence of Hepatitis G Virus in Healthy Children in Liver Disease, and Human Immunodeficiency Virus‐1 Infection: Response to Interferon
Authors: Infante Dámaso , Pich Margarita , Tormo Ramon , Sauleda Silvia , Montané Carlos , Esteban Juan Ignacio , Esteban Rafael 
Published: February 2024

ABSTRACTBackground:A new virus of the Flaviviridae family, the hepatitis G virus (HGV/HGBV‐C), has been identified recently. The purpose of this study was to determine the prevalence of HGV infection in healthy children, in patients with liver disease, and in human immunodeficiency virus (HIV)‐1–infected patients. The role of HGV in the clinical course of chronic HCV, the response to interferon‐α2b, and the possible implications of intravenous γ‐globulin in the transmission of the virus were also evaluated.Methods:Fifty healthy children, 66 patients with a variety of liver diseases, 19 patients with acquired immune deficiency syndrome (AIDS), and various batches of commercial intravenous immunoglobulins were investigated. Viral HGV RNA (5´NCR‐NS5) and anti‐HGV envelope protein E2 were assayed.Results:The prevalence of HGV infection was 6% in the healthy children and 42% in the liver disease group. Viremia and anti‐E2 were found in 11% and 79% of patients with AIDS. Four (27%) of 15 patients with chronic HCV, receiving treatment with interferon, were coinfected by HGV and became HGV‐RNA negative during therapy. One year after the end of interferon therapy, three of them were again HGV RNA positive.Conclusions:The prevalence of HGV infection is high in healthy children higher in children affected with liver disease, but its potential pathologic implication is questionable, and further studies are warranted. Hepatitis G virus is sensitive to interferon therapy, although the infection often recurs after discontinuation of treatment.
Spontaneous Bowel Perforation in Infants and Young Children: A Clinicopathologic Analysis of Pathogenesis
Authors: Chen Jeng‐Chang , Chen Chiu‐Chiang , Liang Jin‐Tung , Huang Shiu‐Feng 
Published: February 2024

ABSTRACTBackground:In children beyond the neonatal stage, spontaneous bowel perforation with no specific cause is rare. It has attracted little notice and does not fit into any established clinical category. This often results in treatment delays. To determine the underlying pathogenesis, a clinicopathologic analysis of this group of patients was performed.Methods:From 1984 through 1997, 15 previously healthy children (10 boys and 5 girls) with bowel perforations were enrolled in this study. Children in whom the specific cause was established were excluded. The ages of the patients ranged from 3 months to 5 years, 5 months (average age: 2 years, 4 months). Data on clinical course, surgical and pathologic findings, and outcomes were analyzed.Results:All the patients had high fever and acute, watery diarrhea with clinical dehydration. Abdominal distension developed and bowel perforation ensued 5 to 30 days after the onset of diarrhea. In all cases, the perforation consisted of either an isolated patch of bowel (n = 8) or patchy lesions in a segment (n = 7), which was confined to one of the following three anatomic watershed areas: the splenic flexure (n = 7); the lower sigmoid (n = 3); and the ileocecal region (n = 5). One patient died, and three had major sequelae including intra‐abdominal abscess, adhesion ileus, and peristomal fistula. Specimens from 13 patients were submitted for histopathologic evaluation. All specimens had acute suppurative inflammation diffusely around the perforation site. Seven had areas of coagulation necrosis of the muscularis propria, especially in the inner circular muscle layer.Conclusion:In acute diarrheal diseases, dehydration may cause bowel ischemia and lead to perforation. Physicians should be alert to the possibility of this abdominal catastrophe when progressive abdominal distension followed by acute diarrheal episodes occurs in children.
Effects of Liver Transplantation on Long‐Chain Polyunsaturated Fatty Acid Status in Infants with Biliary Atresia
Authors: Lapillonne Alexandre , Hakme Christine , Mamoux Valérie , Chambon Michelle , Fournier Vincent , Chirouze Véronique , Lachaux Alain 
Published: February 2024

ABSTRACTBackgroundThe long‐chain polyunsaturated fatty acid (LC‐PUFA) status of infants with untreated biliary atresia (BA) is known to be poor and is correlated to the severity of the liver disease. Liver transplantation (LT) markedly increases survival of patients with BA but the extent to which this reverses poor LC‐PUFA status is not known.MethodsTo explore this question, the erythrocyte (red blood cell, RBC) phospholipid content of eight infants with BA who underwent LT was determined 2 months after an initial portoenterostomy, immediately before LT, and 6 and 12 months after LT. Before LT, all infants were fed a protein hydrolysate formula containing medium‐chain triglycerides and essential fatty acids. Afterward, they were fed a normal diet for age. The RBC phospholipid content at each time point was compared with that of 28 age‐matched control infants.ResultsJust before LT, median RBC phospholipid content of C20:4n‐6, C20:5n‐3, and C22:6n‐3 was 25%, 48%, and 30% lower, respectively, than that observed in age‐matched control infants. After LT, the RBC phospholipid content of most fatty acids reached normal values by 6 months. However, that of C20:4n‐6 and C22:6n‐3 contents remained 5% and 15% lower, respectively, than in normal control infants. Twelve months after LT, C20:4n‐6 content remained lower than in normal children, but that of C22:6n‐3 did not differ. The ratio of C20:3n‐6/C20:4n‐6, a reflection of Δ‐5 desaturase activity, was abnormal compared with normal children before LT (0.17 vs. 0.10, P < 0.009) but normalized by 6 months after LT (0.11 vs. 0.10, not significant).ConclusionsThese data show that the abnormal LC‐PUFA status of children with BA improves after LT but is not entirely reversed within a year after surgery. They suggest that the abnormal status before LT may be secondary, in part, to low Δ‐5 desaturase activity. The extent to which a different pre‐and/or post‐LT diet can prevent PUFA deficiency and/or hasten recovery of PUFA status remains to be determined.
Lactulose–Mannitol Intestinal Permeability Test in Children With Diarrhea Caused by Rotavirus and Cryptosporidium
Authors: Zhang Ying , Lee Ben , Thompson Matthew , Glass Roger , Lee Rosa Cama , Figueroa Dante , Gilman Robert , Taylor David , Stephenson Charles ,   Diarrhea Working Group Peru
Published: February 2024

ABSTRACTBackground:The relationship between intestinal permeability and acute secretory diarrheal syndromes caused by rotavirus and Cryptosporidium parvum in infants less than 36 months of age was studied using the lactulose–mannitol excretion assay.Methods:An oral solution containing 0.4 g/kg lactulose and 0.1 g/kg mannitol was administered to 15 infants with rotavirus, 7 with Cryptosporidium infection and a control group of 7 with secretory diarrhea admitted to the Oral Rehydration Unit of the National Children's Hospital in Lima, Peru. Urinary sugar excretion was measured using an enzymatic spectrophotometric method. The ratio of urinary excretion of lactulose to mannitol was used to measure intestinal mucosal permeability, with higher ratios indicative of increased intestinal permeability. Infants in all three groups were retested 20 days after the initial test.Results:The (mean ± SE) lactulose:mannitol (L:M) excretion ratios during the acute phase (day 1) of diarrhea in infants with rotavirus or Cryptosporidium and control infants were 0.67 ± 0.1, 0.76 ± 0.16, and 0.26 ± 0.04, respectively. In the convalescent phase (day 20) the ratios were 0.19 ± 0.02, 0.28 ± 0.05, and 0.29 ± 0.07, respectively. Significant reductions in L:M ratios were noted in rotavirus patients between days 1 and 20 (paired t‐test;P < 0.01), Cryptosporidium patients between days 1 and 20 (paired t‐test;P < 0.05), and between control subjects on day 1 and rotavirus patients on day 1 and Cryptosporidium patients on day 1 (unpaired t‐tests;P < 0.05 for both). There were no significant differences in control subjects between days 1 and 20, control subjects and rotavirus patients on day 20, or control subjects and Cryptosporidium patients on day 20.Conclusions:The results indicate that increased intestinal permeability caused by rotavirus or cryptosporidium infections in Peruvian infants less than 36 months of age is a significant but reversible phenomenon. The temporal relationship observed in the current study and the contribution of such alterations in intestinal mucosal integrity to the burden of diarrheal disease and the development of malnutrition in developing countries is discussed.
Direct Measurement of Pancreatic Enzymes After Stimulation With Secretin Versus Secretin Plus Cholecystokinin
Authors: Del Rosario Marian A. F , Fitzgerald Joseph F , Gupta Sandeep K , Croffie Joseph M 
Published: February 2024

ABSTRACTBackground:Direct measurement of pancreatic enzymes after administration of pancreatic secretagogues is the gold standard in the assessment of exocrine pancreatic function. Recent experience at the authors' institution showed that endoscopic collection of pancreatic secretions 5, 10, and 15 minutes after intravenous administration of secretin is useful in screening for pancreatic insufficiency. Concomitant administration of intravenous cholecystokinin has been a subject of debate. The purpose of this study was to compare pancreatic enzyme levels after administration of secretin versus secretin plus cholecystokinin and to validate the timing of collection of duodenal fluid.Methods:A prospective, randomized, double‐blind study was conducted from September 1997 through September 1998. Patients scheduled for pancreatic enzyme sampling were randomly assigned to receive intravenous secretin (2 U/kg) plus placebo (group 1) or intravenous secretin (2 U/kg) plus cholecystokinin (0.02 μg/kg [Group 2]). Duodenal fluid was collected 5, 10, and 15 minutes later and placed in dry ice. Samples were measured for the levels of trypsin, amylase, lipase, and chymotrypsin.Results:Twenty patients were assigned to each group. The age range was similar in both groups: 12 months to 16 years, 8 months in group 1 (median, 2.1 years) and 15 months to 13 years, 7 months in group 2 (median, 2.5 years). Group 2 had a greater number of patients with all four enzymes at normal levels during at least one of the time points, 75% versus 50% (P = 0.102). The difference in enzyme levels at the 5‐, 10‐, and 15‐minute collections was statistically significant. For all four enzymes in both groups, values varied from highest to lowest with time (P = 0.0001). The enzyme levels at 10 minutes were close to the enzyme levels at 5 minutes; the lowest values occurred at 15 minutes.Conclusions:Higher pancreatic enzyme levels were obtained after administration of secretin plus cholecystokinin, although the trend did not reach statistical significance. Pancreatic enzyme levels are highest at 5 and 10 minutes, indicating that collections of duodenal fluid should be completed within 10 minutes of the administration of the secretagogues.
Deep Sedation With Inhaled Sevoflurane for Pediatric Outpatient Gastrointestinal Endoscopy
Authors: Montes Ramon G , Bohn Roger A 
Published: February 2024

ABSTRACTBackground:Sevoflurane is an inhaled anesthetic agent with ideal properties for achieving deep sedation during pediatric outpatient gastrointestinal endoscopy. This is a comparison of experience with this gas and other sedation methods used in the authors' hospital.Methods:Retrospective chart review and statistical analysis of data from children receiving inhaled sevoflurane administered by an anesthesiologist through laryngeal insufflation, intravenous propofol, or intravenous midazolam‐fentanyl‐ketamine in any combination to achieve deep sedation for outpatient gastrointestinal endoscopy. Anesthesia was administered in a dedicated procedure room. The intravenous drugs were administered by pediatric intensivists in the intensive care unit. The same endoscopist performed all the procedures.Results:A total of 248 procedures were reviewed (midazolam‐fentanyl‐ketamine 67, propofol 114, and sevoflurane 67). All patients were adequately sedated with sevoflurane, and no intravenous access was required. Time (in minutes) to awakening (midazolam‐fentanyl‐ketamine 47.15, propofol 36.12, sevoflurane 5.70), discharge (midazolam‐fentanyl‐ketamine 141.99, propofol 91.20, sevoflurane 53.34), and total time, including induction and procedure (midazolam‐fentanyl‐ketamine 163.97, propofol 119.40, sevoflurane 73.93), were significantly lower for sevoflurane (P < 0.01). The complication rate for sevoflurane (4.5%) was lower (P < 0.05) than for midazolam‐fentanyl‐ketamine (13.4%) and for propofol (17.5%). Charges for room use and medications were also lower for sevoflurane (P < 0.01). The total charges for sedation (U.S.$) were comparable for sevoflurane (688.10) and propofol (723.08) but were higher for midazolam‐fentanyl‐ketamine (855.10, P < 0.01).Conclusions:Deep sedation with inhaled sevoflurane for pediatric outpatient gastrointestinal endoscopy is as safe as conventional sedation techniques, potentially less expensive, increases endoscopy unit productivity, and eliminates the inconvenience associated with obtaining intravenous access in children.
The Euro‐Growth Study: Why, Who, and How
Authors: van't Hof Martin A. , Haschke Ferdinand ,   Euro‐Growth Study Group
Published: February 2024

ABSTRACTBackground:Assessment of growth is important in health management of infants and children. Evaluation of growth performance requires anthropometric measurements, with proper interpretation depending on the use of appropriate references. Europe‐specific growth references have not been available. The purpose of the present study was to generate such references for infants and children from birth to 3 years of age. The study was further intended to assess the influence of nutrition and lifestyle factors on growth.Methods:The Euro‐Growth Study was designed as a multicenter longitudinal cohort study. This report describes the study design, the sample (cohort), and the methods used. Quality control measures included standardized measurement techniques with ongoing cross‐sectional and longitudinal consistency checks. Selectivity in participation and discontinuation and reproducibility of data over time were evaluated.Results:Of 2245 infants who were enrolled in the study at 22 study sites in 11 countries, 1746 (78%) provided longitudinal data until 12 months of age, 1205 (57%) until 24 months of age, and 1071 (48%) until 36 months of age. Anthropometric measurements were performed on 21,773 occasions. As a result of cross‐sectional and longitudinal data checks, 209 data points (0.09% of all data points) were rejected. Comparison of participants's demographic data with those of nonparticipating local subjects indicated that the cohort was selective in maternal age (higher), maternal education level (higher), household location, and family structure. The withdrawal rate during the study was low (24%), and withdrawals occurred at random. Analysis of reproducibility over time resulted in the elimination of the data from one study site.Conclusion:The Euro‐Growth Study provided longitudinal growth data from a large cohort of normal European children. Because of the chosen sampling method, the study cohort ws somewhat selective relative to the local background population. Internal validity was satisfactory in that the reproducibility of anthropometric measurements was high in 21 sites and the withdrawal rate was low and random.
Iodine Supply in Children From Different European Areas: The Euro‐Growth Study
Authors: Manz Friedrich , van't Hof Martin A. , Haschke Ferdinand ,   Committee for the Study of Iodine Supply in European Children: The Euro‐Growth Study Group
Published: February 2024

ABSTRACTBackground:In many areas of Europe iodine deficiency disorders still exist. Urinary iodine excretion is a valuable tool in characterizing the iodine supply of a population. However, there is a continuing discussion on the most appropriate parameter to measure urinary iodine excretion.Methods:In 247 children (ages 3–5 years) from six European centers, spot urine samples were analyzed for urinary iodine concentration and urinary iodine‐creatinine ratio.Results: The median values of urinary iodine concentration from the six centers were very similar (90–106 μg/l) corresponding to a normal or marginal iodine supply according to the criteria of the World Health Organization. However, the median values of the urinary iodine‐creatinine ratio from the six centers differed significantly (143–445 μg/g, P < 0.001). The different results of the two urinary parameters to assess urinary iodine supply were due to large significant differences in the urinary creatinine concentration reported by the various centers, which probably reflect marked differences in water intake.Conclusions:Iodine deficiency is still a significant health problem in many European countries. In comparable and homogeneous population subgroups (even from different countries), the urinary iodine‐creatinine ratio appears to be a much better parameter for assessing iodine supply than urinary iodine concentration.