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Ethanolaminosis (1977)

Vietor, Karl W. ; Havsteen, B. ; Harms, D. ; [et al.]

Springer

European journal of pediatrics 126 (1977), S. 61-75

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ISSN: 1432-1076

Keywords: Storage disease ; Ethanolaminosis ; Cardiomegaly ; Infantile cerebral dysfunction ; Diastase resistance ; Best-positive storage substance ; Glycogenosis-like disease ; Floppy infant

Source: Springer Online Journal Archives 1860-2000

Topics: Medicine

Notes: Abstract A storage disease with cardiomegaly, generalized muscular hypotonia, cerebral dysfunction, failure to thrive and early death is described in two siblings. The first one died at the age of 10 months, the second at the age of 17 months. The symptoms were mainly due to lysosomal storage of a substance which had a positive reaction to PAS and Best's stain and which was resistant to diastase. This substance was stored in nearly all the organs, especially in the heart, liver, spleen and less in the brain and skeletal muscles. An increased renal excretion of ethanolamine, a greatly increased hepatic concentration of ethanolamine and diminished hepatic ethanolamine kinase activity could be demonstrated. Ethanolamine is essential for the synthesis of phospholipids. Both parents showed increased renal excretion of taurine. In several aspects, this syndrome is similar to the glycogenosis type II described by Pompe.

Type of Medium: Electronic Resource

URL: http://dx.doi.org/10.1007/BF00443124

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Qualitätssicherung von Chemie-Email. Vergleich verschiedener Analysenverfahren (1987)

Busse, H. ; Schindler, H.

Weinheim : Wiley-Blackwell

Materialwissenschaft und Werkstofftechnik 18 (1987), S. 195-200

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ISSN: 0933-5137

Keywords: Chemistry ; Polymer and Materials Science

Source: Wiley InterScience Backfile Collection 1832-2000

Topics: Mechanical Engineering, Materials Science, Production Engineering, Mining and Metallurgy, Traffic Engineering, Precision Mechanics

Description / Table of Contents: Quality Assurance for Vitreous and Porcelain Enamels: Comparison of Analysis MethodsEnamelled test specimens and technical parts made by West European manufacturers of chemical apparatus were investigated by ESCA, RFA, RMA and ICP for qualitative and quantiative differences of enamel composition. It was found that for reliable information concerning the identity and quantitative composition of an enamel it is necessary to use a combination of energy-dispersive X-ray microanalysis (EDX) with optical spectral analysis (ICP).

Notes: Emaillierte Prüfmuster und technische Teile westeuropäischer Hersteller von Chemie-Apparaten wurden mit ESCA, RFA, RMA und ICP qualitativ und quantitativ im Hinblick auf die Emailzusammensetzung vergleichend untersucht. Dabei zeigte sich, daß für eine sichere Aussage über Identität und quantiative Zusammensetzung eines Chemie-Emails die Verbindung von energiedispersiver Röntgenmikroanalyse (EDX) und optischer Spektralanalyse (ICP) erforderlich ist.

Additional Material: 2 Ill.