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The treatment of maple syrup urine disease
The Journal of Pediatrics, 1969Summary The early management of 3 infants with maple syrup urine disease is described with particular reference to variations in daily requirements for branched-chain amino acids. The use of a new dry base mix consisting of a dextrimaltose-corn oil-mineral mix, an iron-vitamin mix, and an amino acid mix as a formula base has facilitated earlier home ...
Shlomo Pollak +3 more
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Journal of Pediatric Endocrinology & Metabolism (JPEM), 2020
Objectives Accurate management of metabolic decompensation in maple syrup urine disease (MSUD) has a crucial role, as acute attacks can cause neurological sequels and can be life threatening.
T. Zubarioglu +5 more
semanticscholar +1 more source
Objectives Accurate management of metabolic decompensation in maple syrup urine disease (MSUD) has a crucial role, as acute attacks can cause neurological sequels and can be life threatening.
T. Zubarioglu +5 more
semanticscholar +1 more source
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
Charles D. Hancock, Norman C. Woody
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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
Charles D. Hancock, Norman C. Woody
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Implications of Maple Syrup Urine Disease in Newborns
Nursing for Women's Health, 2017Maple syrup urine disease (MSUD) is an inherited metabolic disorder that affects the body's ability to metabolize amino acids. If left untreated, it places newborns at risk for life-threatening health problems, including episodes of illness called metabolic crisis.
Pamela Harris-Haman +3 more
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La Radiologia medica, 2001
PubMed: 11677467 [No abstract available]
Incesu L. +4 more
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PubMed: 11677467 [No abstract available]
Incesu L. +4 more
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Maple Syrup Urine Disease in Negro Infant
Archives of Pediatrics & Adolescent Medicine, 1963Since 1954, fourteen infants have been described as having an inborn error of metabolism manifested by signs of central nervous system (CNS) derangement in association with either maple-syrup-scented urine or demonstrable elevations of branched-chain amino acids (or their α-keto acids) in urine, blood, or spinal fluid (Table 1).
Thomas D. Tilden +2 more
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Treatment of maple syrup urine disease: Benefits, risks, and challenges of liver transplantation
International Journal of Developmental Neuroscience, 2023M. Deon +4 more
semanticscholar +1 more source
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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Cerebral Edema in Maple Syrup Urine Disease
Archives of Pediatrics & Adolescent Medicine, 1982Mantovani et al1have recently reported the occurrence of pseudotumor cerebri and computed tomographic (CT) abnormalities in two patients with classic maple syrup urine disease (MSUD). We have observed the same CT abnormalities and clinical evidence of cerebral edema in a 12-day-old boy with MSUD, and suggest that this complication may in fact be more ...
M S Lungarotti +3 more
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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 ...
openaire +3 more sources