http://sacslouisvuittonfr.webs.com/FIGURES AND TABLESFROM:,Sacs Louis Vuitton
Characterization of pre- and post-treatment pathology after enzyme replacement therapy for pompe diseaseBeth L Thurberg,Louis Vuitton Pas Cher, Colleen Lynch Maloney,chanel sacs à main, Charles Vaccaro,lunettes chanel, Kendra Afonso,Chaussures Louis Vuitton, Anne Chun-Hui Tsai,lunettes chanel, Edward Bossen, Priya S Kishnani and Michael O’Callaghan
Light microscopic examination of quadriceps biopsies demonstrates that patient biopsies differ in the histologic response to enzyme replacement therapy after 52 weeks of treatment,lunettes chanel. The glycogen accumulation in patient A at baseline (a) has been cleared in the majority of myocytes after 52 weeks,sac chanel pas cher; a rare myocyte appears completely replaced by glycogen and remains unaffected by ERT (asterix,sac chanel, b),boutique chanel. (c) Demonstrates the heavy glycogen load present at 3 weeks in patient C,bijoux chanel pas cher; glycogen is distributed at the periphery of many cells (red arrow, c),sac chanel pas cher. After 52 weeks of ERT (d),Chaussures Louis Vuitton, there has been little glycogen clearance,chanel pas cher, and many myocytes appear completely replaced by glycogen, similar to the appearance of the isolated cell in (b),Louis Vuitton Pas Cher. (HRLM with Richardson’s/PAS stain, magnification 400),Lunettes Louis Vuitton.
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Figure 2.
Metamorph analysis of glycogen content and correlations with clinical profiles,sac chanel. MetaMorph analysis of glycogen content was performed on all eight patients at all available timepoints (baseline, week 12 and week 52),Lunettes Louis Vuitton. ‘n’ indicates the number of blocks analyzed per biopsy at a given time point. The age of each patient at day 0 of treatment is given on the x-axis. Asterisk(*) indicates patients deceased before the 52 week biopsy. Note that baseline samples for patients B and C were delayed until week 3. No baseline biopsy was available for patient H,Sacs Louis Vuitton. The table below summarizes the motor function changes in each patient. A more detailed clinical account of these patients is described elsewhere,chanel pas cher.11 (abbreviations: age dec=age deceased,Louis Vuitton Pas Cher; wks of tx=total weeks of ERT),sac chanel pas cher.
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Figure 3,sac chanel.
Cartoon illustration of ultrastructural disease progression in five stages,chanel pas cher.
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Figure 4.
Ultrastructural disease correlates with response to ERT. (a) Myocytes with early Stage 1 or 2 ultrastructural disease predominate in the baseline biopsy of patient A,Sacs Louis Vuitton, a good histologic responder,Chaussures Louis Vuitton. Most cells are full of intact myofibrils with lysosomal glycogen tucked in between (4500 ). (b) Myocytes with late Stage 3 or 4 ultrastructural disease predominate in the baseline biopsy of patient C, a poor histologic responder,Lunettes Louis Vuitton. Most cells lack myofibrillar structure and are filled with glycogen lakes and debris ( 4500). (c) High-power view of the variation in cell damage ( 6500). The upper cell with early Stage 2 disease consists of many intact contractile elements with fraying at the fibril ends and peripheral, membrane-bound glycogen (broken arrow). The lower cell with Stage 4 disease has been completely replaced by cytoplasmic glycogen (solid arrow). Ruptured lysosomal membranes float freely in the lake of glycogen. No contractile elements or mitochondria remain. (d) High-power view of a cell with early Stage 2 disease demonstrates abnormally shaped mitochondria and cytoplasmic glycogen beginning to accumulate between contractile elements ( 8500). (e) Satellite cells (red arrow) can be seen, by electron microscopy, beneath the basement membrane adjacent to damaged myocytes (black asterix; EM, 10 500). (f) All satellite cells, active and quiescent, express m-cadherin (red cell membrane staining, yellow arrows) but only activated satellite cells express myogenin (green nuclear staining, white arrow). A blue nuclear dye was used as a counterstain for all nuclei. (confocal microscopy, magnification, 400).
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Figure 5.
Examination of autopsy tissues from patient B demonstrates abnormal glycogen accumulation in multiple organs and cell types. (a) Skeletal muscle, deltoid (HRLM with Richardson’s/ PAS stain, 400); (b) Skeletal muscle, diaphragm (HRLM with Richardson’s/ PAS stain, 400); (c) Nerve from diaphragm: glycogen can be seen in Schwann cell cytoplasm (HRLM with Richardson’s/ PAS stain, 1000); (d) Heart, interventricular septum (HRLM with Richardson’s/ PAS stain, 600); (e) Bladder, smooth muscle cells of the muscularis propria (HRLM with Richardson’s/ PAS stain, 400); (f) Large artery, smooth muscle cells of the media (HRLM with Richardson’s/ PAS stain, 1000); (g) Cerebellum: glycogen accumulation can be seen in a Purkinje cell (HRLM with Richardson’s/PAS stain, 600); (h) Frontal lobe: glycogen accumulation in a neuron (HRLM with Richardson’s/ PAS stain, 1000). (i) Spinal cord: lysosomal glycogen can be seen in motor neurons of the ventral horn (HRLM with Richardson’s/PAS stain, 400); (j) Cardiomyocyte of the interventricular septum contains multiple glycogen-filled lysosomes adjacent to the nucleus. (EM: 28 000). (k) Clusters of membrane-bound glycogen vacuoles are present in motor neurons of the ventral horn (EM: 8800),treatment pathology after enzyme replacement therapy for pompe disease. (l) Myelin sheaths of nerve fibers exhibit splitting (EM: 44 000).http://sacvuitton2013.webs.com/
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