نتایج جستجو برای: lamy
تعداد نتایج: 314 فیلتر نتایج به سال:
Pycnodysostosis (the Toulouse-Lautrec syndrome) is a rare autosomal-recessive disorder of osteoclast dysfunction. This disorder was first described by Maroteaux and Lamy in 1962. We describe anaesthetic management of a 35-year-old female having pyknodysostosis with fracture shaft left femur with anticipated difficult intubation. Therefore, spinal anesthesia was planned for her fracture fixation...
For perfect fields k satisfying [k ¯:k]>2, we construct new normal subgroups of the plane Cremona group and provide an elementary proof its non-simplicity, following melody recent by Blanc, Lamy Zimmermann that rank n over (subfields of) complex numbers is not simple for n≥3.
The fine structure of rotifer coronal cilia has been studied by Lansing & Lamy (1961), and Scholtyseck & Danneel (1962), who reported that they possessed the familiar structures common to all cilia. Cilia are also present in the gut of rotifers, where they stir the contents, and drive them along. This note describes an unusual structure in the tips of the intestinal cilia of the rotifer, Philod...
Mucopolysaccharidosis type VI (MPS VI, Maroteaux-Lamy syndrome, McKusick #253200) is a lysosomal storage disorder that is caused by a deficiency in the lysosomal exohydrolase N-acetylgalactosamine-4-sulphatase (4-sulphatase, EC 3.1.6.1). We report a patient with no obvious clinical signs of MPS VI that has 5% of normal 4-sulphatase catalytic capacity. This patient represents an index case for t...
In continued efforts to develop enzymatic assays for lysosomal storage diseases appropriate for newborn screening laboratories we have synthesized novel and specific enzyme substrates for Maroteaux-Lamy (MPS VI) and Morquio A (MPS IVA) diseases. The sulfated monosaccharide derivatives were found to be converted to product by the respective enzyme in blood from healthy patients but not by blood ...
Hervé Avet-Loiseau,1 Florent Malard,1 Loic Campion,2 Florence Magrangeas,1 Catherine Sebban,3 Bruno Lioure,4 Olivier Decaux,5 Thierry Lamy,6 Laurence Legros,7 Jean-Gabriel Fuzibet,8 Mauricette Michallet,9 Bernadette Corront,10 Pascal Lenain,11 Cyrille Hulin,12 Claire Mathiot,13 Michel Attal,14 Thierry Facon,15 Jean-Luc Harousseau,16 Stephane Minvielle,1 and Philippe Moreau,17 for the Intergroup...
Topics include chemical applications of lasers, molecular spectroscopy, electronic structure of molecules, photochemistry and diffraction. Outcomes, Students. Handbook of Enhanced Spectroscopy (Marc Lamy de la Chapelle, Pietro Giuseppe Gucciardi, Nathalie Lidgi-Guigui) on Amazon.com. *FREE* shipping. Is it possible to find out the phase confirmation from XPS spectra? wh. see: Handbook of Spectr...
Mucopolysaccharidosis type VI or Maroteaux-Lamy syndrome is an autosomal recessive lysosomal storage disorder caused by deficiency of arylsulfatase B (ARS-B) enzyme activity. It results in mild to severe multi-organ system failure from accumulation of undigested glycosaminoglycans (GAGs); dermatan sulfate and chondroitin-4-sulfate. We have developed a single-step enzyme assay using a fluorescen...
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