2 research outputs found
Recommendations for the use of sapropterin in phenylketonuria
Phenylketonuria (PKU) is an inherited disorder of phenylalanine (Phe) metabolism. Until recently, the only treatment for PKU was a Phe-restricted diet. Increasing evidence of suboptimal outcomes in diet-treated individuals, inconsistent PKU management practices, and the recent availability of tetrahydrobiopterin (BH(4)) therapy have fueled the need for new management and treatment recommendations for this metabolic disorder. BH(4), now available as sapropterin dihydrochloride (sapropterin), may offer the potential for improved metabolic control as well as enhanced dietary Phe tolerance in some PKU patients. A group of metabolic dietitians from North America convened in June 2011 to draft recommendations for the use of sapropterin therapy in PKU. Physicians with extensive experience in PKU management were invited at a later date to contribute to the development of these recommendations. Based on extensive clinical experience and current evidence, the present recommendations provide guidance from patient selection and determination of sapropterin response to the long-term management of patients on sapropterin therapy. Target Phe levels, nutritional adequacy, neurocognitive screening and adherence to treatment are addressed to optimize patient outcomes
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Iberian Elements in the Sonatas of Domenico Scarlatti. A Lecture Recital, Together with Three Recitals of L.v. Beethoven, J.S. Bach, J. Brahms, and Selected Works of Other Composers
The purpose of this paper is to identify Spanish elements in the sonatas of Domenico Scarlatti and to determine the extent of their use. All 555 sonatas in facsimile edition, edited by Ralph Kirkpatrick, were compared to the printed anthologies of Spanish folk music by Kurt Schindler and Felipe Pedrell as well as recordings of authentic Spanish folk music. The study concludes that Scarlatti incorporated Spanish musical elements extensively. In some sonatas, fragments of folk tunes occur, but always with some rhythmical alterations or melodic elaborations. Only K. 513 contains an entire folk tune. Scarlatti evidently wrote melodies of folk-like quality and did not merely copy the folk tunes