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10.1002/mus.24495

http://scihub22266oqcxt.onion/10.1002/mus.24495
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suck abstract from ncbi


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pmid25363903
      Muscle+Nerve 2015 ; 51 (3 ): 449-54
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  • Progressive polyradiculoneuropathy due to intraneural oxalate deposition in type 1 primary hyperoxaluria #MMPMID25363903
  • Berini SE ; Tracy JA ; Engelstad JK ; Lorenz EC ; Milliner DS ; Dyck PJ
  • Muscle Nerve 2015[Mar]; 51 (3 ): 449-54 PMID25363903 show ga
  • INTRODUCTION: A 24-year-old man with primary hyperoxaluria type 1 (PH1) presented with a rapidly progressive axonal and demyelinating sensorimotor polyradiculoneuropathy shortly after the onset of end-stage renal disease. His plasma oxalate level was markedly elevated at 107 µmol/L (normal<1.8 µmol/L). METHODS: A sural nerve biopsy was performed. Teased fiber and paraffin and epoxy sections were done and morphometric procedures were performed on this sample and on an archived sample from a 22-year-old man as an age- and gender-matched control. Embedded teased fiber electron microscopy was also performed. RESULTS: The biopsy revealed secondary demyelination and axonal degeneration. Under polarized light, multiple bright hexagonal, rectangular, and starburst inclusions, typical of calcium oxalate monohydrate crystals, were seen. CONCLUSIONS: The proposed mechanisms of nerve damage include disruption of axonal transport due to crystal deposition, toxic effect of oxalate, or nerve ischemia related to vessel occlusion from oxalate crystal deposition.
  • |*Disease Progression [MESH]
  • |Calcium Oxalate/*metabolism [MESH]
  • |Humans [MESH]
  • |Hyperoxaluria, Primary/complications/diagnosis/*metabolism [MESH]
  • |Male [MESH]
  • |Polyradiculoneuropathy/diagnosis/etiology/*metabolism [MESH]
  • |Sural Nerve/*metabolism/pathology [MESH]


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