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L Boysen et al.
Poultry science, 92(5), 1425-1429 (2013-04-11)
When assessing effects of decontamination techniques on counts of Campylobacter spp. on broiler meat, it is essential that the results reflect the variations that may exist. Decontamination studies often use high inoculation levels (10(7) to 10(8) cfu) and one or
Mainak Das et al.
Biomaterials, 30(29), 5392-5402 (2009-07-25)
The use of defined in vitro systems to study the developmental and physiological characteristics of a variety of cell types is increasing, due in large part to their ease of integration with tissue engineering, regenerative medicine, and high-throughput screening applications.
Hannah N Ladenhauf et al.
Pediatric surgery international, 28(8), 805-814 (2012-07-24)
Sodium phosphate-containing laxatives are commonly used as first-line treatment option for constipation in children and adolescents. Hyperphosphatemia is an infrequent, but potentially life-threatening complication of laxative application. We report a case series of three children exhibiting severe hyperphosphatemia and hypocalcemia
Cetin Okuyaz et al.
Journal of child neurology, 23(6), 703-705 (2008-06-10)
An 8-month-old girl was admitted to an outpatient clinic with significant hypotonia and weakness. Organic acid analysis in urine revealed a significant increase in ethylmalonic acid. A deoxyribonucleic analysis revealed the presence of a 625G>A (G-to-A substitution at nucleotide 625)
Alicia Alonso-Hernando et al.
International journal of food microbiology, 159(2), 152-159 (2012-10-18)
The potential of chemical decontaminants to cause harmful effects on human health is among the causes of the rejection of antimicrobial treatments for removing surface contamination from poultry carcasses in the European Union. This study was undertaken to determine whether
Sung-Gun Cho et al.
Journal of Korean medical science, 28(2), 336-339 (2013-02-13)
Although renal calcium crystal deposits (nephrocalcinosis) may occur in acute phosphate poisoning as well as type 1 renal tubular acidosis (RTA), hyperphosphatemic hypocalcemia is common in the former while normocalcemic hypokalemia is typical in the latter. Here, as a unique
Peter Bross et al.
Journal of human genetics, 52(1), 56-65 (2006-10-31)
Molecular chaperones assist protein folding, and variations in their encoding genes may be disease-causing in themselves or influence the phenotypic expression of disease-associated or susceptibility-conferring variations in many different genes. We have screened three candidate patient groups for variations in
Carlo Viscomi et al.
Nature medicine, 16(8), 869-871 (2010-07-27)
Ethylmalonic encephalopathy is caused by mutations in ETHE1, a mitochondrial matrix sulfur dioxygenase, leading to failure to detoxify sulfide, a product of intestinal anaerobes and, in trace amounts, tissues. Metronidazole, a bactericide, or N-acetylcysteine, a precursor of sulfide-buffering glutathione, substantially
I C Lawrance et al.
Digestive diseases and sciences, 58(4), 926-935 (2012-10-26)
Bowel-cleansing studies are frequently underpowered, poorly designed, and with subjective assessments. Consensus on tolerability of the bowel-cleansing agents is thus lacking. This study developed and validated a bowel-preparation tolerability questionnaire and used it to assess the tolerability of three bowel-cleansing
Luca Casettari et al.
Carbohydrate polymers, 91(2), 597-602 (2012-11-06)
The physicochemical and rheological properties of chitosan and two different inorganic sodium phosphate dispersions (NaH(2)PO(4) and Na(3)PO(4)) were investigated in order to elucidate the role of different factors, such as ratios between polymer and sodium inorganic phosphates, different pHs and
Maja Di Rocco et al.
Molecular genetics and metabolism, 89(4), 395-397 (2006-07-11)
A child is reported presenting with a clinical picture suggestive of genetic connective tissue disorders (vascular fragility, articular hyperlaxity, delayed motor development, and normal cognitive development), an absence of pathological ethylmalonic acid excretion during inter-critical phases and a homozygous R163W
Patrícia Fernanda Schuck et al.
Neurochemical research, 35(2), 298-305 (2009-09-17)
High concentrations of ethylmalonic acid are found in tissues and biological fluids of patients affected by ethylmalonic encephalopathy, deficiency of short-chain acyl-CoA dehydrogenase activity and other illnesses characterized by developmental delay and neuromuscular symptoms. The pathophysiological mechanisms responsible for the
C Ficicioglu et al.
Pediatric cardiology, 26(6), 881-883 (2005-08-04)
Malonyl-CoA decarboxylase deficiency is an inborn error of metabolism that may cause hypotonia and a fatal cardiomyopathy in infancy. Newborn metabolic screening programs do not include this disorder, although there is a possibility that presymptomatic treatment may attenuate the development
Karen A McGowan et al.
Archives of neurology, 61(4), 570-574 (2004-04-21)
Among patients with ethylmalonic aciduria, a subgroup with encephalopathy, petechial skin lesions, and often death in infancy is distinct from those with short-chain acyl-coenzyme A dehydrogenase deficiency or multiple acyl-coenzyme A dehydrogenase deficiency. The nature of the molecular defect in
A Bhala et al.
The Journal of pediatrics, 126(6), 910-915 (1995-06-01)
We identified two additional patients with short-chain acyl-coenzyme A (CoA), further characterized the clinical and biochemical features of this defect, and compared it with other fatty acid oxidation defects. We have measured the in vitro short-chain acyl-coenzyme A dehydrogenase (SCAD)
Arun Sreekumar et al.
Nature, 457(7231), 910-914 (2009-02-13)
Multiple, complex molecular events characterize cancer development and progression. Deciphering the molecular networks that distinguish organ-confined disease from metastatic disease may lead to the identification of critical biomarkers for cancer invasion and disease aggressiveness. Although gene and protein expression have
D I Zafeiriou et al.
Neuropediatrics, 38(2), 78-82 (2007-08-23)
Ethylmalonic encephalopathy (EE) is a rare, recently defined inborn error of metabolism which affects the brain, gastrointestinal system and peripheral blood vessels and is characterized by a unique constellation of clinical and biochemical features. A 7-month-old male, who presented with
Levinus A Bok et al.
Pediatrics, 112(5), 1152-1155 (2003-11-05)
To understand the expanding clinical and biochemical spectrum of short-chain acyl-CoA dehydrogenase (SCAD) deficiency, the impact of which is not fully understood. We studied a family with SCAD deficiency and determined urinary ethylmalonic acid excretion, plasma C(4)-carnitine, SCAD enzyme activity
Tandem mass spectrometric determination of malonylcarnitine: diagnosis and neonatal screening of malonyl-CoA decarboxylase deficiency.
René Santer et al.
Clinical chemistry, 49(4), 660-662 (2003-03-26)
M-H Read et al.
Journal of inherited metabolic disease, 28(6), 1149-1150 (2006-01-26)
We report the fourth case of combined D-and L-2-hydroxyglutaric aciduria presenting with neonatal encephalopathy and subependymal cysts.
Valeria Tiranti et al.
American journal of human genetics, 74(2), 239-252 (2004-01-21)
Ethylmalonic encephalopathy (EE) is a devastating infantile metabolic disorder affecting the brain, gastrointestinal tract, and peripheral vessels. High levels of ethylmalonic acid are detected in the body fluids, and cytochrome c oxidase activity is decreased in skeletal muscle. By use
Fatal electrolyte abnormalities following enema administration.
Dominika Szoke et al.
Clinical chemistry, 58(11), 1515-1518 (2012-10-30)
Patrícia Fernanda Schuck et al.
Synapse (New York, N.Y.), 67(3), 111-117 (2012-11-20)
Ethylmalonic acid (EMA) accumulates in tissues of patients affected by short-chain acyl-CoA dehydrogenase deficiency and ethylmalonic encephalopathy, illnesses characterized by variable neurological symptoms. In this work, we investigated the in vitro and in vivo EMA effects on Na(+), K(+)-ATPase (NAK)
Cameron P Brewer et al.
International journal of sport nutrition and exercise metabolism, 23(2), 187-194 (2012-11-02)
Research into supplementation with sodium phosphate has not investigated the effects of a repeated supplementation phase. Therefore, this study examined the potential additive effects of repeated sodium phosphate (SP) supplementation on cycling time-trial performance and peak oxygen uptake (VO2peak). Trained
Samuel Adler et al.
Gastrointestinal endoscopy, 76(6), 1170-1174 (2012-10-03)
Colon capsule endoscopy (CCE) is a noninvasive technique for the detection of colorectal lesions. However, for CCE to be offered as an out-of-clinic procedure, the system needs to automatically alert the patient when to ingest the laxative (booster). We tested
Sunaro C Ngourn et al.
Langmuir : the ACS journal of surfaces and colloids, 28(33), 12151-12158 (2012-07-27)
A quartz crystal microbalance (QCM) sensor was developed for the quantitation of calcium phosphate mineralization and the assessment of DNA as a template molecule. Inherent advantages of QCM, such as nanogram sensitivity, temporal resolution, surface-based measurements, and flow capabilities, were
Colonoscopy preperations.
The Medical letter on drugs and therapeutics, 55(1408), 6-6 (2013-01-26)
Pei-Yu Liu et al.
Electrophoresis, 33(19-20), 3079-3086 (2012-09-22)
A CD-modified CE method was established for quantitative determination of seven hydroxy acids in cosmetic products. This method involved chemometric experimental design aspects, including fractional factorial design and central composite design. Chemometric experimental design was used to enhance the method's
Kaisorn L Chaichana et al.
Journal of neuroscience methods, 180(1), 116-125 (2009-05-12)
The discoveries of neural (NSCs) and brain tumor stem cells (BTSCs) in the adult human brain and in brain tumors, respectively, have led to a new era in neuroscience research. These cells represent novel approaches to studying normal phenomena such
C D Capo-Chichi et al.
The American journal of clinical nutrition, 71(4), 978-986 (2000-03-25)
Riboflavin, flavin mononucleotide (FMN), and flavin adenine dinucleotide (FAD) concentrations have been little studied in cases of malnutrition. Our objective was to investigate the effects of malnutrition on riboflavin status and riboflavin's relation with thyroid hormones and concentrations of urinary
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