bluuebunny/crossref_metadata_2025_split huggingface dataset:
Incidence of Dysplasia in Pelvic Pouches in Pediatric Patients After Ileal Pouch‐Anal Anastomosis for Ulcerative Colitis
Authors: Sarigol Samra , Wyllie Robert , Gramlich Terry , Alexander Frederick , Fazio Victor , Kay Marsha , Mahajan Lori 
Published: February 2024

ABSTRACTBackground:The purpose of this study was to evaluate the incidence of dysplasia and the mucosal adaptation patterns of pelvic pouches in children and adolescents who had undergone ileal pouch‐anal anastomosis for ulcerative colitis.Methods:Between 1982 and 1996, 176 pediatric patients with ulcerative colitis underwent ilial pouch‐anal anastomosis. Seventy‐six patients were followed up after surgery at the Cleveland Clinic. Pouch biopsy specimens were reviewed for dysplasia and to determine mucosal adaptation patterns. Fifty‐eight of the 76 patients had an average of three mucosal biopsies during a mean follow‐up of 5 years. Demographic and surgical data were abstracted from archives of medical records. All previously obtained pouch biopsy specimens were re‐evaluated by a single pathologist to ensure standardized interpretation.Results:No dysplasia was identified in screening specimens of 76 children and adolescents including 5 patients who showed dysplasia in resected colon specimens. The pattern of mucosal adaptation was categorized using previously reported criteria. Type A was defined as normal mucosa or mild villous atrophy with no or mild inflammation. Type B mucosa showed transient atrophy with temporary moderate inflammation followed by normalization of architecture. Type C mucosa was defined as a pattern of persistent atrophy with severe inflammation. In the study cohort, the patterns of mucosal adaptation, type A (56.9%; n = 33), type B (32.8%; n = 19), and type C (10.3%; n = 6), were comparable with those reported in adults. The rate of pouch failure and diagnosis of Crohn's disease were similar in each group and were not related to the specific adaptation pattern. Most of the patients with type C mucosa had clinical symptoms of pouchitis requiring periodic antibiotic therapy. No dysplasia was identified in any biopsy specimen reviewed.Conclusions:Similar morphologic changes can be seen in ileal pouches in pediatric and adult patients. There seemed to be no increased risk of dysplasia in children and young adults who had undergone ilial pouch‐anal anastomosis surgery for ulcerative colitis during a 5 year follow‐up. Because the long‐term risk of development of dysplasia is unknown, an initial screening should be performed 5 years after the creation of a pelvic pouch in children or when the total disease duration exceeds 7 years. Once identified, patients with Type C mucosa should have annual screening for dysplasia until further data become available.
Concentrations and Anti‐Haemophilus influenzae Activities of β‐Casein Phosphoforms in Human Milk
Authors: Kroening Terry A. , Baxter Jeffrey H. , Anderson Steven A. , Hards Robert G. , Harvey Linda , Mukerji Pradip 
Published: February 2024

ABSTRACTBackground:The distribution and concentrations of six phosphorylated forms of human β‐casein, a major source of nutrition among breast‐fed infants, have not been examined in milk samples without prior fractionation. In this study, the levels of β‐casein phosphoforms in untreated human milk samples were analyzed and their antiadhesion activities determined against Haemophilus influenzae, a pathogen implicated in middle ear infection in infants.Methods:Human milk samples were analyzed using ureapolyacrylamide gel electrophoresis of whole‐milk samples and scanning densitometry to determine the concentrations of β‐casein and its phosphoforms. A nontypable H. influenzae strain was radiolabeled to monitor its attachment to human pharyngeal cells in microtiter plates. Purified phosphoforms of β‐casein were preincubated for 15 minutes with radiolabeled bacteria to determine their antiadhesion activities.Results:The average β‐casein concentration in 151 human milk samples was 5.37 ± 2.26 mg/ml. On average, the phosphoforms in untreated milk are present in the following order ranked by concentration: tetra‐ > di‐ > non‐ > mono‐ > tri‐ > pentaphosphorylated β‐casein. The tri‐, tetra‐, and pentaphosphorylated forms of human β‐casein exhibited more than 60% inhibition of H. influenzae in the antiadhesion assay when used at a concentration of 0.6 to 0.9 mg/ml.Conclusion:The β‐casein level in untreated human milk is significantly higher than previously reported. The phosphoform distribution of β‐casein in individual donors varies widely. Anti‐H. influenzae activity was detected in vitro among human β‐casein molecules with three or more phosphate groups.
Infant Formula With Protein‐Energy Ratio of 1.7 g/100 kcal Is Adequate but May Not Be Safe
Authors: Fomon Samuel J. , Ziegler Ekhard E. , Nelson Steven E. , Rogers Ronald R. , Frantz Joan A. 
Published: February 2024

ABSTRACTBackground:An adequate protein‐energy ratio of infant formulas has been defined as one that permits growth similar to that of infants fed relatively generous protein‐energy ratios, and serum concentrations of albumin and urea nitrogen no less than those observed in breast‐fed infants. A safe ratio has been defined as one with no detectable adverse effects. The hypothesis was that a protein‐energy ratio of 1.7 g/100 kcal is adequate and safe.Methods:Healthy male infants were fed Formula 1.7, a milk‐based formula, as the sole source of energy from the 8th to the 112th day of life. Weight, length, and energy intake were measured; serum albumin and urea nitrogen were determined; and the results were compared with data from appropriate reference groups of infants.Results:Energy intake from 8 through 55 days was significantly higher than that of infants in the formula‐fed reference group. Gain in weight was significantly more than that of the formula‐fed reference group or of a breast‐fed reference group, whereas gain in length was similar to that of the formula‐fed reference group. Body mass index was significantly higher than that of either reference group, suggesting more fat accumulation in infants fed Formula 1.7. Plasma concentrations of albumin and urea nitrogen were similar to those of the breast‐fed reference group.Conclusion:Infants fed Formula 1.7 received adequate intakes of protein. Because of the possibility that ad libitum feeding of diets with moderately inadequate protein‐energy ratios is associated with increased food intake leading to excess weight gain, it is not possible to conclude that a protein‐energy ratio of 1.7 g/100 kcal is safe.
Comparison Between Serum α‐Glutathione S‐Transferase and Aminotransaminases in Detecting Cytolysis in Hepatitis C‐Infected Children
Authors: Namour Farès , Morali Alain , Ilardo Claudio , Abballe Xavier , Maury Florence , Guéant Jean‐Louis 
Published: February 2024

ABSTRACTBackground:α‐Glutathione S‐transferase (αGST) has been proposed as a more sensitive indicator than serum alanine aminotransferase (ALT) and aspartate aminotransferase (AST) in detecting hepatocellular damage due to chronic hepatitis C virus (HCV) infection.Methods:The accuracy of α‐GST was compared with that of ALT and AST in detecting cytolysis in 103 blood samples issued from 31 children positive for HCV RNA.Results:α‐GST had a lower sensitivity than ALT or AST (32% vs. 54.4% for each aminotransferase). The sensitivity of ALT and/or AST was 60.2%, whereas that of ALT and/or α‐GST and AST and/or α‐GST was lower (58.3% and 57.3%, respectively). Among 41 serum samples with negative ALT and AST, only 2 had positive α‐GST, whereas α‐GST failed to detect cytolysis in 31 samples with elevated ALT and/or AST. No correlation was found between α‐GST, ALT, or AST and the Knodell score.Conclusions:The combination of ALT with AST is actually the best compromise in detecting cytolysis in untreated HCV‐infected patients.
Diet Fat and Oral Insulin‐Like Growth Factor Influence the Membrane Fatty Acid Composition of Suckling Rat Small Intestine
Authors: Kit Shelley , Thomson Alan B. R. , Clandinin M. Thomas 
Published: February 2024

ABSTRACTBackground:Insulin‐like growth factor‐1 plays an important role in small intestine development. The presence of insulin‐like growth factor‐1 and the complexity of the fatty acid composition in breast milk suggests that intestinal development may be influenced by manipulating the levels of these components.Methods:To determine whether a physiological dose of insulin‐like growth factor‐1 would influence sucrase and lactase activity levels, 10‐day‐old suckling rat pups were treated with an oral gavage of insulin‐like growth factor‐1. Four diets differing in fat composition were fed to lactating dams. Brush border membranes were isolated from jejunal and ileal segments of suckling rat small intestine. Fatty acid analysis of choline and ethanolamine phospholipids was performed.Results:Insulin‐like growth factor‐1 was found to have no effect on the sucrase and lactase activities of suckling rats. Changes in the diet fat composition of the mother's diet indirectly influenced the fatty acid composition of suckling rat small intestine. Insulin‐like growth factor‐1 decreased ileal C20:4n‐6 levels. A correlation was observed between lactase activity and C20:4n‐6 and C22:6n‐3 levels. As C20:4n‐6 levels increased, lactase activity appeared to decline. Increased lactase activity was observed when C22:6n‐3 levels increased.Conclusions:The changes observed in C20:4n‐6 levels in response to oral insulin‐like growth factor‐1, combined with the apparent trend of increased lactase activity with declining levels of C20:4n‐6, may be of significance in the development of the small intestine in early life.
Gastric Emptying in Formula‐Fed and Breast‐Fed Infants Measured with the 13C‐Octanoic Acid Breath Test
Authors: Van Den Driessche Mieke , Peeters Kristel , Marien Paul , Ghoos Yvo , Devlieger Hugo , Veereman‐Wauters Gigi 
Published: February 2024

ABSTRACTBackground:The 13C‐octanoic acid breath test, a noninvasive method for measuring gastric emptying, was used to compare the gastric‐emptying rate of formula‐fed and breast‐fed infants. Octanoic acid, a medium‐chain fatty acid marked with the stable isotope 13C is immediately absorbed in the duodenum. Because gastric emptying is the rate‐limiting step for the absorption of medium‐chain fatty acids, the fraction of 13C expired in the breath indicates the rate of gastric emptying.Methods:Twenty‐nine newborn infants (16 boys, 13 girls) were investigated, with parental consent. The infants had a mean gestational age at birth of 34.5 weeks (range, 27‐41 weeks) and a birth weight of 2148 g (range, 960‐4100 g). Their mean weight on the day of the test was 2496 g (range, 1998‐4140 g), and their mean age was 23 days (range, 7‐74 days). Each infant received a test meal after a maximum fasting period of 3 hours. Fourteen infants were fed formula milk (Nutrilon Premium, NV Nutricia, Zoetermeer, The Netherlands) with 13C‐octanoic acid and 15 infants received expressed mother's milk mixed with 13C‐octanoic acid. After obtaining two basal breath samples and the feeding, breath samples were collected using a nasal prong, every 5 minutes during the first half hour and every 15 minutes during the next 3.5 hours. Analysis of the expired 13C fraction in the breath samples was performed using isotope‐ratio mass spectrometry, and the gastric emptying curve and gastric emptying parameters were determined.Results:The mean half‐emptying time determined by the 13C‐octanoic acid breath test was 65 minutes (range, 27‐98 minutes) for the formula fed infants and 47 minutes (range, 16‐86 minutes) for the breast‐fed infants. The difference between the half‐emptying times is significant (t‐test, p < 0.05).Conclusions:The results of the 13C‐octanoic acid breath test indicated faster gastric emptying of human milk than formula. Our findings are in accordance with those in earlier studies, using the invasive‐dilution technique; noninvasive and detailed ultrasonography, which is not easily used because it is operator dependent and the observation time is short; or cineesophagogastroscintigraphy, which is less suitable for infants (because of the radiation involved). The 13C‐octanoic acid breath test is a safe and noninvasive method for measuring gastric emptying in small infants and allows comparison of various feeding methods.
Systemic Bartonella henselae Infection with Hepatosplenic Involvement
Authors: Ventura Alessandro , Massei Francesco , Not Tarcisio , Massimetti Mauro , Bussani Rossana , Maggiore Giuseppe 
Published: February 2024

ABSTRACTBackground:Systemic manifestations of Bartonella henselae infection are rare in the immunocompetent host. The infection generally has initial symptoms of prolonged fever and multiple granulomatous lesions in liver and spleen.Methods:Retrospective analysis of the records of all patients with hypoechogenic lesions in the liver and/or spleen diagnosed from 1990 through 1996 in three pediatric clinics in northern Italy,Results:Among the 13 patients reviewed, 9 had evidence of B. henselae infection and hepatosplenic involvement: five had prolonged and unexplained fever lasting from 3 to 16 weeks, and four had typical cat‐scratch disease and peripheral lymphadenitis. All patients had increased sedimentation rate and normal aminotransferase serum activity. Five children had a liver biopsy, by laparotomy in three and by needle in two. In all, the predominant liver lesion was a necrotizing granuloma. All patients were treated with broad‐spectrum antibiotics. Fever lasted from 3 to 16 weeks, and hepatic and splenic lesions resolved in all with residual splenic calcification in one.Conclusions:Systemic B. henselae infection represents an important cause of inflammatory hypoechogenic hepatosplenic lesions in children. Serology provides rapid diagnosis, avoiding multiple and invasive investigations. Hepatosplenic involvement can be found even in children with typical cat‐scratch disease without apparent systemic manifestations. The frequency of liver and/or splenic involvement in cat‐scratch disease is probably underestimated.
Immunological and Nutritional Composition of Human Milk in Relation to Prematurity and Mothers' Parity During the First 2 Weeks of Lactation
Authors: Montagne Paul , Cuillière Marie Louise , Molé Claire , Béné Marie Christine , Faure Gilbert 
Published: February 2024

ABSTRACTBackground:To investigate the effect of prematurity and parity on the dynamics of the major immunologic and nutritional proteins of human milk over the first 2 weeks of lactation.Methods:Microparticle‐enhanced nephelometric immunoassays were developed for the quantification of α‐lactalbumin, β‐casein, serum albumin, lactoferrin, and lysozyme in human milk. These components, immunoglobulin A, and total proteins were assayed in 368 individual samples collected from 74 mothers.Results:The dynamics of the major immunologic and nutritional proteins in early lactation presented similar patterns in preterm and term human milks. In comparison with term milk, preterm milk was globally characterized by higher concentrations of immune proteins and lower concentrations of nutritive proteins. These differences were increased by the degree of prematurity, which, however, influenced the absolute and relative protein concentrations differently, depending on the stage of lactation. The protein composition of term milk was similar, whatever the mother's parity. Conversely, the influence of prematurity on the levels of milk proteins during the first days of lactation was even greater in primiparous mothers.Conclusions:This precise description of the composition of preterm and term milk, regarding the main nutritional and immunologic proteins, confirms the influence of both prematurity and parity on milk components and demonstrates the combined effect of these two conditions.
Effects of Red Blood Cell Transfusion on Resting Energy Expenditure in Adolescents with Sickle Cell Anemia
Authors: Harmatz Paul , Heyman Melvin B. , Cunningham John , Lee Philip D. K. , Styles Lori , Quirolo Keith , Kopp‐Hoolihan Lori , Ghiron Jim , Hintz Raymond L. , Vichinsky Elliott 
Published: February 2024

ABSTRACTBackground:Previous studies indicate that resting energy expenditure is elevated in children with sickle cell anemia, possibly caused in part by hemolysis and increased erythropoietic activity. The purpose of the present investigation was to determine whether erythrocyte transfusion normalizes resting energy expenditure in sickle cell anemia.Methods:Five adolescents with sickle cell anemia (12‐16 years old; 4 boys, 1 girl) were studied before and 1 week after erythrocyte transfusion before elective surgery or at the initial transfusion for growth failure. Resting energy expenditure was measured by indirect calorimetry, and laboratory measures were determined by routine, validated methods. Data comparisons were by nonparametric analysis.Results:After erythrocyte transfusion, total hemoglobin levels increased (difference (D = 15 g/l; p < 0.05), whereas hemoglobin S (D = ‐0.36; p < 0.05) and reticulocyte count (D = ‐0.12; p < 0.05) decreased. Mean pretransfusion resting energy expenditure was elevated to 124% above predicted levels (p < 0.05) and increased further to 134% above prediction (p < 0.05) vs. pretransfusion levels). Plasma triiodothyronine (T3) levels increased (D = 0.17 nmol/l; p < 0.05), reverse T3 (rT3) levels tended to decline (D = ‐0.04 nmol/l; p = 0.14), and rT3/T3 decreased (D = ‐0.03; p < 0.05). Plasma insulin‐like growth factor‐I (IGF‐I) levels were low‐normal before transfusion and did not change, despite the change in resting energy expenditure.Conclusions:The results confirm that resting energy expenditure is elevated in patients with sickle cell anemia. However, resting energy expenditure further increased after transfusion, despite decreased erythropoietic activity. A posttransfusion decrease in rT3/T3 may contribute to the increased resting energy expenditure. That there was no change in IGF‐I implies that the growth hormone‐IGF system is not involved in posttransfusion regulation of resting energy expenditure. Therefore, our data are not consistent with the hypothesis that increased resting energy expenditure in sickle cell anemia is directly related to erythropoietic activity. The mechanisms by which resting energy expenditure increases after transfusion in sickle cell anemia require additional investigation.
Prevalence of Protein‐Energy Malnutrition at Diagnosis in Children with Acute Lymphoblastic Leukemia
Authors: Reilly John J. , Weir Jennifer , McColl John H. , Gibson Brenda E. S. 
Published: February 2024

ABSTRACTBackground:The purpose of the present study was to test the hypothesis that protein‐energy undernutrition is common in patients with acute lymphoblastic leukemia at diagnosis. Previous studies have failed to establish whether undernutrition is a common feature at diagnosis.Methods:Body mass index (BMI, weight/height2), expressed as a standard deviation score (SDS) relative to contemporary United Kingdom reference data, was used as the index of nutritional status. The index was calculated in a national cohort of standard‐risk patients (n = 1019) treated in the same protocol in the United Kingdom.Results:Prevalence of undernutrition (defined as BMI SDS <‐2.0) exceeded expected frequencies in boys (7.6%) and girls (6.7%). These differences were statistically significant (p < 0.001), with a 95% confidence interval for the prevalence of undernutrition of 5.8% to 9.0%.Conclusions:Undernutrition is relatively common in patients with newly diagnosed acute lymphoblastic leukemia, with a threefold excess of patients below the cutoff used to define undernutrition. Screening for undernutrition at diagnosis of acute lymphoblastic leukemia is indicated, and the BMI SDS is a simple index of nutritional status that could be readily calculated using measurements routinely made at diagnosis. The same simple screening technique could also be used clinically to detect and manage or prevent overnutrition (obesity), which is common in these patients after diagnosis.
Primary Gastric Plasmacytoma: A Rare Cause of Hypertrophic Gastritis in an Adolescent
Authors: Goyal Alka , Langer Jacob C. , Zutter Mary , Swanson Paul , Kraus Madeleine D. , Bartlett Nancy , Shackelford Gary D. , Longtine Janina A. , Perlmutter David H. 
Published: February 2024

ABSTRACTBackground:This report describes a 16‐year‐old patient with gastric rugal hypertrophy caused by a primary gastric plasmacytoma. She had a 3‐month history of nausea and burning abdominal pain. Radiographic studies showed giant rugal hypertrophy. Superficial endoscopic gastric biopsies showed mild inflammation with plasma cells of polyclonal origin in the mucosa. When symptoms persisted, she underwent laparoscopic full‐thickness gastric biopsy. There was monoclonal plasma cell infiltration histologically diagnostic of plasmacytoma and inconsistent with Helicobacter pylori‐associated mucosa‐associated lymphoid tissue (MALT) lymphoma. There was no evidence for involvement of the bone marrow or regional lymph nodes. The tumor did not respond to radiotherapy, necessitating total gastrectomy.Methods:Blood samples were analyzed for interleukin (IL)‐6 by enzyme‐linked immunosorbent assay. Gastric biopsy and gastrectomy specimens were subjected to immunophenotyping for κ and λ light chains, CD45, CD20, and LN1 and to polymerase chain reaction analysis for herpes virus HHV8.Results:There was no elevation in circulating IL‐6 levels, militating against a pathogenesis akin to that of Castleman's disease. There was no evidence for infection with the Kaposi's sarcoma‐associated herpes virus HHV8, which has recently been found in patients with multiple myeloma.Conclusions:This diagnosis and the characteristics of the tumor are very unusual, if not unique, for a patient of this age. The diagnostic evaluation of this patient also demonstrates the importance of deep endoscopic or full‐thickness biopsies in some children with hypertrophic gastritis.
Variable Morbidity in Alagille Syndrome: A Review of 43 Cases
Authors: Quiros‐Tejeira Ruben E. , Ament Marvin E. , Heyman Melvin B. , Martin Martin G. , Rosenthal Philip , Hall Theodore R. , McDiarmid Sue V. , Vargas Jorge H. 
Published: February 2024

ABSTRACTBackground:Alagille syndrome is one of the most common inherited disorders that cause chronic liver disease in children. Early reports suggested a benign course in these patients. Subsequent reports showed significant morbidity and mortality. This study was designed to analyze the long‐term clinical course on Alagille syndrome.Methods:The records of children with Alagille syndrome seen during a 20‐year period were reviewed.Results:Forty‐three patients were identified. Liver disease was diagnosed before 12 months of age in 95%. The frequencies of renal anomalies (50%) and intracranial hemorrhage (12%) were significant. The high incidence of chronic otitis media (35%) has not been reported previously. One patient had a renal transplant. Vascular compromise as a pathologic mechanism for some characteristics of the syndrome is also suggested by the presence of small bowel stenosis and atresia, tracheal and bronchial stenosis, renal artery stenosis, middle aortic syndrome, and avascular necrosis of the humeral and femoral heads. Twenty (47%) patients underwent liver transplantation. Five of six who underwent Kasai procedure required liver transplantation. Twelve died (28%), five after liver transplantation. One patient died of intracranial bleeding. Sixteen (37%) without liver transplantation and 15 (35%) who underwent liver transplantation are alive.Conclusions:Some patients with early‐onset and more severe liver disease can benefit from liver transplantation. Careful and complete assessment should be made of infants with a cholestatic syndrome, to avoid misdiagnosis and unnecessary Kasai procedures. Our observation of vascular compromise in various organ systems suggests that notch signaling pathway defects affect angiogenesis in Alagille syndrome.