Search results for "Excess"

showing 10 items of 225 documents

X-Linked myopathy with excessive autophagy: A new hereditary muscle disease

1988

We report on 3 brothers with a myopathy that also affected their maternal grandfather and great-uncle. Characteristic features are onset in early childhood, very slow progression, normal life expectancy, weakness of proximal limb muscles, especially in the legs, elevation of serum creatine kinase, and no cardiac or intellectual involvement. In biopsy material muscle fibers are almost never necrotic but show excessive autophagic activity and exocytosis of the phagocytosed material. We suggest that this family has an undescribed type of congenital myopathy, for which we propose the name X-linked myopathy with excessive autophagy.

WeaknessPathologymedicine.medical_specialtybusiness.industryAutophagyAnatomymedicine.diseaseCongenital myopathyX-linked myopathy with excessive autophagyMuscle diseaseNeurologySlow progressionMedicineNeurology (clinical)Biopsy materialmedicine.symptombusinessMyopathyAnnals of Neurology
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Ylenpalttisuutta ja äärimmäisyyskokemuksia : Jaakko Yli-Juonikkaan Neuromaani 2000-luvun eksessiromaanina

2019

Yli-Juonikas JaakkoeksessiromaaniexcessNeuromaanieksessipoetiikkaromaanittoisto
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Goodness-of-fit tests for parametric excess hazard rate models with covariates

2017

In this paper we propose a general methodology for testing the null hypothesis that an excess hazard rate model, with or without covariates, belongs to a parametric family. Estimating the excess hazard rate function parametrically through the maximum likelihood method and non-parametrically (or semi-parametrically) we build a discrepancy process which is shown to be asymptotically Gaussian under the null hypothesis. Based on this result we are able to build some statistical tests in order to decide wether or not the null hypothesis is acceptable. We illustrate our results by the construction of chi-square tests which the behavior is studied through a Monte-Carlo study. Then the testing proc…

[STAT]Statistics [stat]Proportional excess hazards modelSemiparametric estimation[ STAT ] Statistics [stat]Maximum likelihood estimationNonparametric estimationcolon cancer dataCovariatesExcess hazard model[STAT] Statistics [stat]
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Influence of the Oxic-Settling-Anaerobic (OSA) Process on Methane Production by Anaerobic Digestion of Sewage Sludge

2023

The present study evaluated different sludge-reduction mechanisms in the oxic-settling-anaerobic (OSA) process in terms of their effects on methane productivity by anaerobic digestion of sewage sludge. Two different layouts were investigated for the sludge return from an anaerobic side-stream reactor (ASSR) to the anoxic (scheme A) or the aerobic (scheme B) reactor of a pre-denitrification plant. Biochemical methane-potential (BMP) assays performed on the excess sludge revealed that scheme A promoted an overall increase of methane production in the OSA (20 mLCH4 gVSS−1d−1, +19%), although compared with a control CAS plant a significant decrease in the excess sludge production (3…

anaerobic digestionsewage sludgeSettore ICAR/03 - Ingegneria Sanitaria-Ambientaleanaerobic side-stream reactorGeography Planning and DevelopmentBMPAquatic Scienceexcess sludge minimizationBiochemistryWater Science and TechnologyWater
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Case Report: Insulinoma Presenting as Excessive Daytime Somnolence

2021

Currently, undiagnosed insulinomas remain a difficult clinical dilemma because its symptoms in most cases can easily be misdiagnosed as other diseases. In this article, we present the case of a 14-year-old girl who presented to our hospital with recurrent episodes of excessive daytime sleepiness and abnormal behavior during sleep that had been going on for 3 months. Insulinoma is a rare neuroendocrine tumor that causes excessive release of insulin, resulting in episodes of hypoglycemia. It usually manifests as autonomic sympathetic symptoms. These symptoms resolved rapidly with the administration of glucose. After successful removal of the tumor, daytime sleepiness and abnormal nighttime be…

business.industryEndocrinology Diabetes and Metabolisminsulinomamedicine.diseaseRC648-665Diseases of the endocrine glands. Clinical endocrinologysomnolenceEndocrinologyhypoglycemiaExcessive daytime somnolenceAnesthesiaMedicinecase reportbusinessInsulinomasleep disorderFrontiers in Endocrinology
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Effectiveness of mandibular advancement appliances in treating obstructive sleep apnea syndrome: A systematic review

2015

Objectives/Hypothesis Mandibular advancement devices are an alternative to continuous positive airway pressure for patients with mild or moderate obstructive sleep apnea/hypopnea syndrome (OSAHS). The main aim of this review was to assess the effectiveness of different devices in treating OSAHS, based on polysomnographic measurements such as the apnea/hypopnea index (AHI) and oxygen saturation, and on changes in the upper airway and improvements in the most common symptoms: snoring and somnolence. Their adverse effects were also noted. Study Design Systematic review. Methods Following an exhaustive search in the Medline, Scopus, and Cochrane Library databases, 22 articles published in the p…

business.industrymedicine.medical_treatmentDentistryApneaExcessive daytime sleepiness030206 dentistryCochrane Librarymedicine.diseaserespiratory tract diseasesObstructive sleep apnea03 medical and health sciences0302 clinical medicinestomatognathic systemOtorhinolaryngologymedicineContinuous positive airway pressuremedicine.symptomAirwaybusinessHypopnea030217 neurology & neurosurgerySomnolenceThe Laryngoscope
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Enantioselective Synthesis of α-Quaternary Amino Acids by Alkylation of Deprotonated α-Aminonitriles.

2015

A series of α-quaternary arylglycines were prepared in high optical purity (up to 98% ee) by α-alkylation of deprotonated α-aminonitriles derived by the Strecker reaction from (4S,5S)-5-amino-2,2-dimethyl-4-phenyl-1,3-dioxane. The procedure includes only chromatographic purification of the final products and is devoid of chromatography or crystallization operations on intermediates to raise the optical purity.

chemistry.chemical_classificationAlkylationMolecular StructureChemistryOrganic ChemistryStrecker amino acid synthesisEnantioselective synthesisStereoisomerismAlkylationCatalysislaw.inventionAmino acidDioxanesDeprotonationlawNitrilesOrganic chemistryCrystallizationAmino AcidsProtonsEnantiomeric excessThe Journal of organic chemistry
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ChemInform Abstract: Enantioselective Synthesis of α-Quaternary Amino Acids by Alkylation of Deprotonated α-Aminonitriles.

2015

An α-alkylation of deprotonated α-amino nitriles, prepared from chiral phenyldioxanamine (I) by Strecker reaction, leads to α-quaternary arylglycines with high optical purity after hydrolysis and oxidative cleavage of the chiral auxiliary.

chemistry.chemical_classificationChiral auxiliarychemistry.chemical_compoundHydrolysisDeprotonationchemistryStrecker amino acid synthesisEnantioselective synthesisGeneral MedicineAlkylationEnantiomeric excessMedicinal chemistryAmino acidChemInform
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Determination of optical purity of phosphonic acid analogues of aromatic amino acids by capillary electrophoresis with alpha-cyclodextrin.

2000

A simple and efficient method for the determination of enantiomeric purity of structurally diverse phosphonic and phosphinic acid analogues of phenylalanine and phenylglycine using capillary electrophoresis is presented. These preliminary studies indicated that the enantiomer separation is strongly dependent on the structure of the aminophosphonic acid.

chemistry.chemical_classificationCyclodextrinsalpha-CyclodextrinsChromatographyCyclodextrinOrganic ChemistryOrganophosphonatesElectrophoresis CapillaryPhenylalanineGeneral MedicineBiochemistryAnalytical ChemistryAmino acidchemistry.chemical_compoundCapillary electrophoresischemistryBenzene derivativesAromatic amino acidsphosphonic acidsEnantiomerenantiomer separationAmino AcidsEnantiomeric excessJournal of chromatography. A
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(S)-Mandelic acid enolate as a chiral benzoyl anion equivalent for the enantioselective synthesis of non-symmetrically substituted benzoins

2011

A strategy for the enantioselective synthesis of non-symmetrically substituted benzoins from (S)-mandelic acid and aromatic aldehydes has been developed. This strategy is based on a diastereoselective aldol reaction of the lithium enolate of the 1,3-dioxolan-4-one derived from (S)-mandelic acid and pivalaldehyde with aromatic aldehydes, which gives the corresponding aldols in good yields. Subsequent hydroxyl group protection as MEM ethers, basic hydrolysis of the dioxolanone ring, oxidative decarboxylation of the α-hydroxy acid moiety, and hydroxyl group deprotection provides chiral non-symmetrically substituted benzoins with high enantiomeric excesses.

chemistry.chemical_classificationDecarboxylationorganic chemicalsOrganic ChemistryEnantioselective synthesisMandelic acidBiochemistryAldehydechemistry.chemical_compoundAldol reactionchemistryDrug Discoverypolycyclic compoundsOrganic chemistryAldol condensationEnantiomeric excessOxidative decarboxylationTetrahedron
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