Results 181 to 190 of about 5,954 (223)
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American Journal of Diseases of Children, 1963
More than 20 instances of maple syrup urine disease (MSUD) have been described since 1954. Most were infants, who manifested in the first month of life a maple syrup odor in their urine and a clinical pattern of fits, episodic rigidity, lethargy, and poor suck. When measured, their blood and urine levels were found to be elevated for the branched-chain
N C, WOODY, C D, HANCOCK
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More than 20 instances of maple syrup urine disease (MSUD) have been described since 1954. Most were infants, who manifested in the first month of life a maple syrup odor in their urine and a clinical pattern of fits, episodic rigidity, lethargy, and poor suck. When measured, their blood and urine levels were found to be elevated for the branched-chain
N C, WOODY, C D, HANCOCK
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Cerebral lipids in maple syrup disease
The Journal of Pediatrics, 1965A combined neuropathological and neurochemical study of a patient who succumbedto maple syrup disease is presented. The principal anatomic abnormalities were in the white matter and included generalized cerebral edema, astrocytosis, oligodendroglial swelling, and a decreased amount of myelin.
J H, MENKES, M, PHILIPPART, R E, FIOL
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Journal of Food Science, 1982
ABSTRACT Selected grades of maple syrup (34% moisture) were dehydrated by two dissimilar methods. Differences in color and flavor of reconstituted dried products and the maple syrups from which they were derived were minimal. Moisture content, bulk density, hygroscopicity and ease of reconstitution of the dehydrated products showed ...
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ABSTRACT Selected grades of maple syrup (34% moisture) were dehydrated by two dissimilar methods. Differences in color and flavor of reconstituted dried products and the maple syrups from which they were derived were minimal. Moisture content, bulk density, hygroscopicity and ease of reconstitution of the dehydrated products showed ...
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Gastronomica, 2006
This article examines the organic maple syrup industry in Canada, providing personal anecdote, a brief history of maple production and the underlying practice and philosophy of the organic movement as it applies to maple products. Often thought as inherently organic, modern, industrial maple syrup production includes a number of ways in which concerns ...
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This article examines the organic maple syrup industry in Canada, providing personal anecdote, a brief history of maple production and the underlying practice and philosophy of the organic movement as it applies to maple products. Often thought as inherently organic, modern, industrial maple syrup production includes a number of ways in which concerns ...
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Inflammation in maple syrup urine disease
Clinica Chimica ActaMaple syrup urine disease (MSUD), also called leucinosis, is a rare inborn error of metabolism characterized by the deficiency of an enzyme complex: branched-chain alpha-keto acid dehydrogenase (BCKAD). This complex is located in the mitochondria and is responsible for metabolizing the three essential branched-chain amino acids (BCAA): isoleucine ...
Ana Kalise, Böttcher +6 more
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Archives of Neurology, 1967
MAPLE syrup disease is a rapidly progressive familial cerebral degenerative condition marked by the excretion of a urine possessing a sweet, maple syrup-like odor. 1 The enzymatic basis for this disease is a failure in the oxidative decarboxylation of three branched-chain keto acids, the derivatives of leucine, valine, and isoleucine.
J H, Menkes, H, Solcher
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MAPLE syrup disease is a rapidly progressive familial cerebral degenerative condition marked by the excretion of a urine possessing a sweet, maple syrup-like odor. 1 The enzymatic basis for this disease is a failure in the oxidative decarboxylation of three branched-chain keto acids, the derivatives of leucine, valine, and isoleucine.
J H, Menkes, H, Solcher
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Scoliosis and Maple Syrup Urine Disease
Journal of Pediatric Orthopaedics, 1984The intermediate variant of maple syrup urine disease produced frequent infections and significant mental retardation in a young female patient recently treated for scoliosis. There were no problems with infection, wound healing, or fusion with a regimen consisting of a low protein diet, perioperative antibiotics, good hydration, and early ...
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The Therapy of Maple Syrup Urine Disease
Archives of Pediatrics & Adolescent Medicine, 1967BOTH THE clinical picture and the metabolic abnormality in maple urine disease pose special problems in its management. The rapid progression of symptoms (from feeding problems and apathy to the onset of central nervous system signs with periods of hypertonicity alternating with hypotonia, loss of Moro's reflex, difficulties with respiration to ...
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Pure Maple Syrup: Nutritive Value
Science, 1964Variations in concentrations of sugar, nitrogen, phosphorus, potassium, calcium, and magnesium of sap from sugar maple ( Acer saccharum , Marsh.) trees are related to the time of sap collection and result in variation of the same components in pure maple syrup.
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