bluuebunny/crossref_metadata_2025_split huggingface dataset:
HIV Infection Presenting With Motor Axonal Variant of Guillain-Barré Syndrome
Authors: Wagner Jeffrey C , Bromberg Mark B 
Published: March 2009

ABSTRACT:

Objectives:
We present a case of acute motor axonal neuropathy in a patient with previously unrecognized human immunodeficiency virus (HIV) infection.

Methods:
A 46-year-old male graduate student from Mali, Africa presented with a 3-week history of progressive weakness that began during a visit home from Utah. The symptoms stabilized by 6 weeks and gradually improved without treatment.

Results:
Electrodiagnostic studies revealed widespread fibrillation potentials and positive sharp waves with normal sensory amplitudes and no demyelinating features. Cerebrospinal fluid pleocytosis and elevated protein prompted a test for HIV infection, which returned positive.

Conclusions:
To our knowledge, this is the first case of acute motor axonal neuropathy in HIV outside of a seroconversion reaction.
Unilateral Thigh Atrophy and Weakness in Hip Osteoarthritis
Authors: DePalma Traci B , Gilchrist James M 
Published: March 2009

Abstract

Osteoarthritis is a common condition that affects a significant percentage of people in the United States. The case and discussion will serve to introduce to the neurologist the issue of hip osteoarthritis causing thigh atrophy and weakness. The pathogeneses of muscular atrophy in the setting of osteoarthritis and of muscle weakness as a cause of osteoarthritis will be explored.
Glomus Tumors in Patients of Advanced Age: A Conservative Approach
Authors: Cosetti Maura , Linstrom Christopher , Alexiades George , Tessema Belachew , Parisier Simon 
Published: March 2009

AbstractObjectives: Identify and discuss controversies in the management of paragangliomas in elderly patients.Assess and evaluate a conservative treatment strategy involving limited surgical resection and vigilant monitoring of the outcome measures of tumor control, peritreatment morbidity, symptom resolution, and hearing preservation.Study Design: Retrospective case review.Methods: All of the patients in this study were over age 60 with temporal bone glomus tumors. Primary outcome assessment included length of hospitalization, perioperative morbidity, symptom resolution, hearing preservation, and long‐term tumor control.Results: Twelve female patients with mean age of 74.5 years (range 61–85 years) with follow‐up from 24 months to 33 years (mean/median: 5/7.8 years) were identified. Nine (75%) of the patients presented with pulsatile tinnitus.Seven patients (58%) underwent surgical excision of the middle ear component of the paraganglioma. Tumors extending to the jugular foramen were purposely not resected. Five patients (45%) had relative or absolute contraindications to surgical resection and were treated with observation or primary radiation therapy. Post‐treatment audiometric evaluation confirmed stable or improved hearing. Pulsatile tinnitus resolved in all patients. No patient experienced cranial nerve deficits, extended hospitalization, or blood transfusions.All patients were followed closely with radiological imaging. The majority of patients demonstrated no disease or stable disease, while two patients demonstrated tumor growth 6 years after diagnosis.Conclusion: A prolonged natural history and the morbidity associated with surgical intervention have led to controversies in the treatment of glomus tumors in an elderly population. Our experience supports recent limited reports advocating conservative surgical excision and vigilant long‐term monitoring in this population.
Serotonin, Inflammation, and IBS: Fitting the Jigsaw Together?
Authors: Spiller Robin 
Published: March 2009

ABSTRACTUnexplained diarrhoea is a frequent indication for gastroenterologic referral, and after full investigation the most common final diagnosis is irritable bowel syndrome (IBS). Some patients with IBS describe an acute onset of symptoms following infective gastroenteritis. Postinfective IBS affects 7% to 31% of individuals infected, and appears to be a nonspecific response to injury which has been reported following Salmonella‐, Campylobacter‐, and Shigella‐related IBS. The strongest risk factor for developing postinfective IBS is severity of the initial diarrhoea illness, but toxigenicity of the infected bacteria, age <60 years, and female sex also are important risk factors. Adverse life events, hypochondriasis, and depression are also important, as is increased enteroendocrine cell and lymphocyte numbers in rectal biopsies. Postinfective IBS and IBS with diarrhoea without an infectious onset both show increased postprandial release of serotonin, whilst constipated patients show a depressed release. Several studies suggest impairment of the serotonin transporter in IBS, which in animal studies has been shown to occur following a range of inflammatory insults. Clinical conditions with an inflammatory basis, such as coeliac and Crohn disease, also are characterised by excess postprandial serotonin release. Several studies report evidence of low‐grade inflammation in IBS with diarrhoea. However, reliable markers of low‐grade inflammation that may predict response to serotonin antagonists or other anti‐inflammatory agents remain a goal for future research.
Hyperornithinemia‐Hyperammonemia‐Homocitrullinuria Syndrome (HHH) Presenting With Acute Fulminant Hepatic Failure
Authors: Mhanni AA , Chan A , Collison M , Seifert B , Lehotay DC , Sokoro AH , Huynh HQ , Greenberg CR 
Published: March 2009

ABSTRACTWe report on two Aboriginal patients with the hyperornithinemia‐hyperammonemia‐homocitrullinuria (HHH) syndrome. Both presented with acute hepatic failure with severe hypertransaminasemia and coagulopathy, prompting evaluation for emergent liver transplantation. The diagnosis of HHH syndrome was based on the presence of typical metabolic abnormalities. A protein‐restricted diet and L‐arginine or L‐citrulline supplementation were immediately started, with rapid normalization of liver function test results and other biochemical abnormalities. Molecular analysis of the SLC25A15 gene showed that the two patients were homozygous for the common French Canadian mutation (F188Δ). The diagnosis of HHH syndrome should be considered in patients with unexplained fulminant hepatic failure. There does not appear to be a genotype‐phenotype correlation for this presentation, inasmuch as the only other reported patient presenting with this picture had two different point mutations. Early identification and prompt treatment of these patients is crucial to avoid liver transplantation and can be life saving.
Effect of Enteral IGF‐1 Supplementation on Feeding Tolerance, Growth, and Gut Permeability in Enterally Fed Premature Neonates
Authors: Corpeleijn Willemijn E , van Vliet Ineke , de Gast‐Bakker Dana‐Anne H , van der Schoor Sophie RD , Alles Martine S , Hoijer Maarten , Tibboel Dick , van Goudoever Johannes B 
Published: March 2009

ABSTRACTObjectives:The gastrointestinal tract of the premature newborn functions suboptimally with regard to digestion, absorption, and feeding tolerance. Human milk contains trophic factors, such as insulin‐like growth factor‐1 (IGF‐1), that are believed to stimulate gut growth and function. The objective of this double blind, randomized, controlled trial was to assess the effects of enteral IGF‐1 supplementation on whole body growth measured by weight gain (in grams per kilogram per day), days to regain birth weight, and anthropometrical characteristics, and gut maturation and permeability (measured by sugar absorption tests).Patients and Methods:The study included 60 premature infants (birth weight 750–1250 g) during the first month of life. Patients received either standard infant formula or standard infant formula supplemented with IGF‐1 in a concentration twice that of human colostrum (10 μg/100 mL of formula). Primary endpoints were days to full enteral feeding, days to regain birth weight, and growth rate. Sugar absorption tests were performed weekly to assess the secondary endpoints gut permeability and maturation.Results:None of the primary endpoints differed to statistical significance between groups at any point. However, gut permeability was significantly lower in the IGF‐1 supplement group on day 14 compared with the control group. At day 21, lactulose/mannitol excretion ratios were (again) comparable between the groups.Conclusions:Although gut permeability showed a faster decrease in the IGF‐1 supplement group, our data do not support IGF‐1 supplementation to infant formula.
Diagnosis of Helicobacter pylori Infection in a High‐prevalence Pediatric Population: A Comparison of 2 Fecal Antigen Testing Methods and Serology
Authors: Cherian Sarah , Burgner David P , Carson Christine F , Sanfilippo Frank M , Cook Angus G , Forbes David A 
Published: March 2009

ABSTRACTObjectives:Accurate methods for diagnosing active Helicobacter pylori infection in children have been limited to invasive or time‐consuming techniques. Recently, fecal antigen testing has been used successfully for the diagnosis of H pylori infection in the pediatric population. We compared 2 monoclonal fecal antigen diagnostic methods in a population of children with a suspected high prevalence of H pylori infection. We also assessed the diagnostic performance of H pylori immunoglobulin G serology.Materials and Methods:In a cross‐sectional study of African refugee children (<16 years) we compared an immunochromatographic technique (ICT) and serology with a monoclonal fecal antigen enzyme immunoassay (MFAT) method for the detection of active H pylori infection. Following the manufacturer's instructions, an optical density of ≥0.190 was used as a cutoff for MFAT. Sensitivity, specificity, and positive and negative predictive values were calculated.Results:Of the 193 eligible children enrolled, active H pylori infection was detected in 149 of 182 (81.9%) in whom MFAT was performed. The prevalence of active infection increased with age; children with active infection were significantly older, and there were no sex differences. ICT and serology underperformed in comparison with MFAT (ICT sensitivity 74.6%, specificity 63.6%, positive predictive value 89.8%, negative predictive value 36.8%; and serology sensitivity 57.9%, specificity 77.4%, positive predictive value 92.0%, negative predictive value 29.9%).Conclusions:Monoclonal enzyme immunoassay fecal antigen testing is a practical and feasible alternative to traditional invasive diagnostic methods in high‐prevalence pediatric populations. Neither immunochromatography nor serology is useful for the diagnosis of active H pylori infection in these children.
Oral Delivery of L‐arginine Stimulates Prostaglandin‐dependent Secretory Diarrhea inCryptosporidium parvum–infected Neonatal Piglets
Authors: Gookin Jody L , Foster Derek M , Coccaro Maria R , Stauffer Stephen H 
Published: March 2009

ABSTRACTObjectives:To determine if oral supplementation with L‐arginine could augment nitric oxide (NO) synthesis and promote epithelial defense in neonatal piglets infected withCryptosporidium parvum.Materials and Methods:Neonatal piglets were fed a liquid milk replacer and on day 3 of age infected or not with 108C parvumoocysts and the milk replacer supplemented with L‐arginine or L‐alanine. Milk consumption, body weight, fecal consistency, and oocyst excretion were recorded daily. On day 3 postinfection, piglets were euthanized and serum concentration of NO metabolites and histological severity of villous atrophy and epithelial infection were quantified. Sheets of ileal mucosa were mounted in Ussing chambers for measurement of barrier function (transepithelial resistance and permeability) and short‐circuit current (an indirect measurement of Cl−secretion in this tissue).Results:C parvum–infected piglets had large numbers of epithelial parasites, villous atrophy, decreased barrier function, severe watery diarrhea, and failure to gain weight. L‐Arginine promoted synthesis of NO by infected piglets, which was unaccompanied by improvement in severity of infection but rather promoted epithelial chloride secretion and diarrhea. Epithelial secretion by infected mucosa from L‐arginine‐supplemented piglets was fully inhibited by the cyclooxygenase inhibitor indomethacin, indicating that prostaglandin synthesis was responsible for this effect.Conclusions:Results of these studies demonstrate that provision of additional NO substrate in the form of L‐arginine incites prostaglandin‐dependent secretory diarrhea and does not promote epithelial defense or barrier function ofC parvum–infected neonatal ileum.
Natural Evolution of Infantile Regurgitation Versus the Efficacy of Thickened Formula
Authors: Hegar Badriul , Rantos Rastra , Firmansyah Agus , De Schepper Jean , Vandenplas Yvan 
Published: March 2009

ABSTRACTBackground:Regurgitation is frequent in infants. We evaluated changes in regurgitation among patient groups fed standard formula, standard formula subsequently thickened with cereal, or formula manufactured with bean gum as a thickening agent.Patients and Methods:A prospective, blinded, randomised 1‐month intervention trial evaluating the efficacy of parental reassurance of the regurgitating child in combination with 3 formula interventions—standard infant formula (group A); 5 g of rice cereal added to 100 mL standard formula (group B); and formula manufactured with bean gum as a thickening agent (group C)—was performed in 60 infants presenting with more than 4 episodes of regurgitation and/or vomiting per day during the week before inclusion. Formula intake, infant comfort, stool aspects, and weight gain were evaluated. All of the infants and data recorded by parents in a diary were evaluated weekly by a blinded health care professional.Results:At baseline, groups A, B, and C were similar for all of the parameters. After the 1‐month intervention, regurgitation/vomiting decreased significantly in all 3 groups (P < 0.0005). Although the decrease was largest in group C (−4.2 ± 2.1 episodes/day), the incidence did not differ significantly with groups A or B. At no evaluation interval was there a difference in volume of formula intake, infant comfort, stool frequency, or aspect. After 1 month, weight gain was significantly greater in group C compared with group A (19.9% vs 16.4%; P < 0.001).Conclusions:Thickening of formula decreases regurgitation, but not significantly. Parental reassurance remains the cornerstone of the treatment of infant regurgitation.
Capsule Endoscopy May Reclassify Pediatric Inflammatory Bowel Disease: A Historical Analysis
Authors: Cohen Stanley A , Gralnek Ian M , Ephrath Hagit , Saripkin Larry , Meyers William , Sherrod Olga , Napier Angela , Gobin Tamara 
Published: March 2009

ABSTRACTBackground:Data are sparse on the role of capsule endoscopy (CE) in classifying disease type, affecting patient management, and altering health outcomes in pediatric inflammatory bowel disease (IBD). In a retrospective cohort analysis of symptomatic pediatric patients with previously diagnosed IBD, we evaluated whether data from CE would result in the recognition of new disease locations that could alter the current diagnosis and provide information to better manage the underlying disease.Patients and Methods:CE evaluation was performed in 28 patients an average of 4.2 ± 3 years after original IBD diagnosis, and was prompted by exacerbation of underlying disease, growth failure/weight loss, or presurgical evaluation. Of the patients, 7 of 28 (25%) were originally diagnosed as having ulcerative colitis (UC) (n = 5) or indeterminate colitis (IC) (n = 2), while 21 of 28 (75%) were diagnosed as having Crohn disease (CD).Results:Following CE examination, 4 of 5 patients with UC and 1 of 2 patients with IC (total 5 of 7, 71% of UC/IC patients) had their disease reclassified to CD based upon newly diagnosed small bowel mucosal lesions. Moreover, 13 of 21 (62%) patients with CD were found at the time of CE examination to have more extensive small bowel disease with newly diagnosed jejunal disease found in 12 of 13 (92%) patients. In the 5 newly diagnosed patients with CD, all of them had therapeutic changes made. One capsule retention occurred.Conclusions:Capsule endoscopy may lead to reclassification of IBD from UC/ IC to definitive CD. In addition, previously diagnosed patients with CD may be found to have a more significant burden of small bowel disease. Taken together, this information may facilitate more targeted and effective therapies and potentially lead to better patient outcomes.
Growth and Final Height After Liver Transplantation During Childhood
Authors: Scheenstra Rene , Gerver Willem Jan , Odink Roelof J , van Soest Hanneke , Peeters Paul MJG , Verkade Henkjan J , Sauer Pieter JJ 
Published: March 2009

ABSTRACTObjective:To evaluate the effect of end‐stage pediatric liver disease and liver transplantation on growth and final height.Patients and Methods:We evaluated growth at 2 years (n = 101) and 5 years (n = 63) after pediatric liver transplantation (LTx). Twenty‐three children reached final height. Height was expressed as a standard deviation score of the target height (zTH score) of each patient.Results:At the first 2 years after LTx, the zTH score was significantly increased from −1.7 to −1.3 SD (P < 0.05). Growth at 2 or 5 years after LTx, expressed as ΔzTH score, was positively correlated with pretransplant growth retardation (P < 0.05). In comparison with patients with noncholestatic primary liver disease, patients with cholestatic primary liver disease were more severely growth retarded before LTx (zTH score −2.0 vs −1.2 SD, P < 0.05) and had better growth in the first 2 years after LTx (ΔzTH score +0.6 vs −0.1 SD, P < 0.05). Twelve of the 23 patients had a final height below −1.3 SD of their target height.Conclusions:Growth retardation is common in children before LTx, particularly in children with an underlying cholestatic disease. After LTx, catch‐up growth was partial and was prominent only in cholestatic children who had been severely growth retarded before LTx. After LTx during childhood, ∼50% of patients reach a final height lower than −1.3 SD of their genetic potential
Necrotising Enterocolitis in Very Low Birth Weight Infants in Italy: Incidence and Non‐nutritional Risk Factors
Authors: Gagliardi Luigi , Bellù Roberto , Cardilli Viviana , De Curtis Mario ,   Network Neonatale Lombardo
Published: March 2009

ABSTRACTIncidence and non‐nutritional risk factors were estimated for necrotising enterocolitis in 2035 very low birth weight infants, admitted to 14 tertiary‐level neonatal intensive care units in Lombardy, northern Italy. There were 62 necrotising enterocolitis cases, with an overall incidence of 3.1%. After adjustment for gestational age and centre, the risk factors were mechanical ventilation, patent ductus arteriosus, and late‐onset sepsis, whereas surfactant treatment was associated with decreased risk. Significant variations in necrotising enterocolitis incidence among hospitals were found.
Feeding Short-Tailed Fruit Bats (Carollia perspicillata)
Authors: Rasweiler John J. , Cretekos Chris J. , Behringer Richard R. 
Published: March 2009

INTRODUCTIONThe short-tailed fruit bat Carollia perspicillata is predominantly frugivorous in the wild and will therefore accept a variety of non-citrus fruits in captivity (e.g., bananas, apples, melon, and peaches). To promote cage cleanliness, however, these bats are routinely fed a fruit-based liquid diet that cannot be carried away from the food dishes. By intention, this diet is simple and inexpensive to prepare, and it can also be prepared in advance and refrigerated or frozen for later use. This protocol describes the preparation and storage of the diet, as well as the feeding of the bats.
A Nuclear Gene Encoding the Iron-Sulfur Subunit of Mitochondrial Complex II Is Regulated by B3 Domain Transcription Factors during Seed Development in Arabidopsis
Authors: Roschzttardtz Hannetz , Fuentes Ignacia , Vásquez Marcos , Corvalán Claudia , León Gabriel , Gómez Isabel , Araya Alejandro , Holuigue Loreto , Vicente-Carbajosa Jesús , Jordana Xavier 
Published: March 2009

Abstract
Mitochondrial complex II (succinate dehydrogenase) is part of the tricarboxylic acid cycle and the respiratory chain. Three nuclear genes encode its essential iron-sulfur subunit in Arabidopsis (Arabidopsis thaliana). One of them, SUCCINATE DEHYDROGENASE2-3 (SDH2-3), is specifically expressed in the embryo during seed maturation, suggesting that SDH2-3 may have a role as the complex II iron-sulfur subunit during embryo maturation and/or germination. Here, we present data demonstrating that three abscisic acid-responsive elements and one RY-like enhancer element, present in the SDH2-3 promoter, are involved in embryo-specific SDH2-3 transcriptional regulation. Furthermore, we show that ABSCISIC ACID INSENSITIVE3 (ABI3), FUSCA3 (FUS3), and LEAFY COTYLEDON2, three key B3 domain transcription factors involved in gene expression during seed maturation, control SDH2-3 expression. Whereas ABI3 and FUS3 interact with the RY element in the SDH2-3 promoter, the abscisic acid-responsive elements are shown to be a target for bZIP53, a member of the basic leucine zipper (bZIP) family of transcription factors. We show that group S1 bZIP53 protein binds the promoter as a heterodimer with group C bZIP10 or bZIP25. To the best of our knowledge, the SDH2-3 promoter is the first embryo-specific promoter characterized for a mitochondrial respiratory complex protein. Characterization of succinate dehydrogenase activity in embryos from two homozygous sdh2-3 mutant lines permits us to conclude that SDH2-3 is the major iron-sulfur subunit of mature embryo complex II. Finally, the absence of SDH2-3 in mutant seeds slows down their germination, pointing to a role of SDH2-3-containing complex II at an early step of germination.
Expression of genes encoding for proteins involved in heparan sulphate and chondroitin sulphate chain synthesis and modification in normal and malignant plasma cells
Authors: Bret Caroline , Hose Dirk , Reme Thierry , Sprynski Anne‐Catherine , Mahtouk Karène , Schved Jean‐François , Quittet Philippe , Rossi Jean‐François , Goldschmidt Hartmut , Klein Bernard 
Published: March 2009

SummarySyndecan‐1 is a proteoglycan that concentrates heparin‐binding factors on the surface of multiple myeloma cells, and probably plays a major role in multiple myeloma biology. As heparan sulphate and chondroitin sulphate are the bioactive components of syndecan‐1, we analysed the signature of genes encoding 100 proteins involved in synthesis of these chains, i.e. from precursor uptake to post‐translational modifications, using Affymetrix microarrays. The expression of enzymes required for heparan sulphate and chondroitin sulphate biosynthesis was shown to increase in parallel with syndecan‐1 expression, throughout the differentiation of memory B cells into plasmablasts and normal bone marrow plasma cells. Sixteen genes were significantly different between normal and malignant plasma cells, nine of these genes –EXT2, CHSY3, CSGALNACT1, HS3ST2, HS2ST1, CHST11, CSGALNACT2, HPSE, SULF2 – encode proteins involved in glycosaminoglycan chain synthesis or modifications. Kaplan–Meier analysis was performed in two independent series of patients: B4GALT7, CSGALNACT1, HS2ST1 were associated with a good prognosis whereas EXT1 was linked to a bad prognosis. This study provides an overall picture of the major genes encoding for proteins involved in heparan sulphate and chondroitin sulphate synthesis and modifications that can be implicated in normal and malignant plasma cells.