最新Approach to the patient with Myopathy：方法对患者的肌病教学讲义ppt课件.ppt

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1、ApproachtothepatientwithMyopathy：方法对患者的肌病OutlineBasicsClinicalmanifestationsDiagnosticapproachIndividualmyopathiesApproachtothepatientSkeletalmuscleComposedofmusclefibersthatcontractwhenstimulatedbyamotorneuronSkeletalmuscle:coveredbyconnectivetissuesheath(epimysium):compo

2、sedofmanycolumns(fascicles)Fascicles:surroundedbyperimysium:composedofmanymusclefibersMusclefibers:coveredbycellmembrane(sarcolemma)andsurroundedbyendomysium.:anatomicandphysiologicunit.Twotypes:1.Type-I/slowtwitch/redfibers:richerinoxidativebutpooreringlycolyticenzymes.2.

3、Type-II/fasttwitch/whitefibers:richeringlycolyticbutpoorerinoxidativeenzymes.:composedofnuclei,cytoplasm(sarcoplasm),myofibrils,ribosomes,mitochondria,storedfat,manyenzymes,glycogen,myoglobin.Cont,dMusclecontraction:slidingofthinfilamentsoverandb/nthickfilamentsbytheaction

4、ofnumerouscrossbridgesthatextendoutfromthemyosinetowardtheactin.:musclefiberstimulationbymotorneuron→ca+2movesfromitsstoresiteinthesarcoplasmicreticulumtothesarcoplasm→attachestotroponine→conformationalchangethatmovesthetroponincomplexanditsattachedtropomyosineoutoftheways

5、othatcrossbridgescanattachtoactin→slidingoffilamentsandthusmuscletensionandshortening.▪Energysource:fattyacids,glycogenandbloodglucose,aminoacids:skeletalmusclesgenerateATPthroughanaerobicandaerobicrespirationsandphosphategroupsdonatedbycreatinephosphate.ATP:servesastheimm

6、ediatesourceofenergyfor1.movementofcrossbridgesformusclecontraction2.pumpingofca+2intothesarcoplasmicreticulumformusclerelaxation.Cont,dMitochondria:playskeyroleinenergyproductionAnaerobicrespiration:majorsourceofenergyduringexerciseGlucose→Lacticacid+ATPAerobicrespiration

7、:lipids–importantsourcesofenergyduringrest&duringprolongedsubmaximalexerciseVLDLTriglycerides→fattyacids→activatedfattyacids/acyl-coA/↓CPT-Ilinkedwithcarnitine↓mitochondria↓CPT-IIATP←Betaoxidation←acyl-coA+carnitineMyopathyDefn.:disorderswithstructuralchangesorfunctionalim

8、pairmentofamuscle;unrelatedtoanydisorderofinnervationorNMJ.Classification:1.Acquired2.Her