蔡宏歆骨科课件2014读片会case osteomalacia cai

病例报告

CaseReport浙江大学附属邵逸夫医院骨科OrthopedicsDepartmentofSRRSHHospitalAffiliatedbyZheJiangUniversity蔡宏歆CaiHongxinM.DCaihongxin.zju@声明在此专业会议公开和讨论患者病情已得到患者允许病史HistoryA32-year-oldIndiawomanRighthippain4month,lefthippain10daysLowerlimbweaknessanddifficultytostandupfromsitting,WaddlingGait.ROMofleftHipwaslimitedNotraumahistoryPMHunremarkableMRIRight第一次就诊2013-10月RightDiagnosis诊断DDHOsteoarthritisInfectionorTuberculosisFractureAnkylosisspondylitisRheumatoidarthritisBonemarrowedema（femurheadnecrosis）LabTests化验CBC:wbc13000/ESR：20mm/HRF:normalASO：normalUricacid：normalCRP:normalTreatment治疗NSAIDsAntibioticMuchMuchBetter第二次就诊2014-5Askingneurologicalexpertconsult.Righthippainincreasedintendays.Difficultytostandupfromsitting.WaddlingGait.LowerextremitymuscleMRCscale:M4X-RayRightFractureDiagnosisFractureAtypicalfracturePathologicalfractureStressfracturePseudo-fracture1yearalendronate2009.72010.32010.52012.3Whatisdifferentbetweentwocase?Diagnosis诊断Reasonsfor“Fractures”?ReasonsforMuscleweakness?BMD骨密度股骨颈-3.5Wards三角-3.2LabTestsCBC:normalESR:30mm/hRF:NegtiveUricacid:normalCRP:28.2mg/LT3T4:normalACA&AKA:normalHLA-B27:normalASO:normalLabTestsPTH:145ng/L(15-65)Ca:2.4P:0.54mmpl/L(0.8-1.6)Alkalinephosphatase:305mmol/L(35-135)25-hydroxyVitaminD:<7.5nmol/L(>75)VitaminDDeficiency维生素D缺乏CommonCauses原因InadequateintakeDecreasedexposuretosunlightGastricbypassDrugRiskfactersVegetarian(noegg&milk)IndiapeopleimmigratetohighlatitudeareaDarkskin高危人群VitaminD/symptomsVidaminDValleyvalleyimpordanddomusclosgelendonBonepain(Hydrationofbonematrix)MuscleweaknessDifficultyinwalkingTBinfection(<50mmol/L)Myopathy病理Osteomalaciacanpresentfirstasseveremuscleweaknessanddifficultywalking13%.Boneturnoverabout5%/year,patientsmaydevelopmyopathyinsidioustooveryears.Ca++iscriticallyimportantformusclecontractilitySkeletalmusclecontain1-25vitaminDreceptorsthatDiagnosisAgainFractureAtypicalfracturePathologicalfractureStressfracturePseudo-fracture讨论Osteoporosis骨质疏松Osteomalacia骨质软化Osteoporosis骨质疏松WHOBMD-2.5TInosteoporosis,thebonesareporousandbrittle.硬-量小Osteomalacia骨软化Thesofteningofthebonesduetodefectivebonemineralization（骨量大–软）;Tscore<3VitaminDdeficiencyRenaltubularacidosisTumor-inducedosteomalaciaFGF-23ChronicrenalfailureGeneticdiseasePathologyDDxAtypicalfracturePseudo-fractureAtypicalFractureAtypicalFracturePseudo-fractureAtypicalFractureSeveresuppressionofboneturnoverLeadstotheaccumulationofbonemicrodamageandthedevelopmentofaninsufficiencyfractureatthepointofmaximal,Weight-bearingstress,namelyatthesubtrochantericordiaphysealfemurLooserPseudo-fractureTheselesionsarecomposedofpoorlymineralizedosteoidmatrixandareNOTtruefracturesorstressfractures.Occurontheconcaveside(compressiveaspect).Osteomalacia骨软化OsteomalaciaReddyMunagalaVV,TomarV.NEnglJMed2014;370:e10.DifferentMicrodamageStressfracture微骨折累积的疲劳骨折BonesoftenPseudo-fracture骨软化造成假骨折最终诊断VitaminDdeficiencyOsteomalaciawithbilateralproximalfemoralpseudo-fractures(Looser-Milkmansyndrome)ProximalmyopathyNEXTTetracyclinelabeledbonebiopsyRuleoutKidneydiseaseBonescanSimpleTest25-VitaminDhalflife15Days1-25VitaminDhalflife15h.总结Tips成年人的维生素D缺乏并非罕见，容易被忽视2case/May。维生素D缺乏不仅可以最终导致骨的软化，而且早期造成近躯干肌肉无力，容易误诊。骨质疏松和骨软化具有不同病理基础。骨软化多发生在骨的压力侧。不典型应力骨折发生在骨的张力侧。参考文献OsteomalaciaasaResultofVitaminDDeficiency;ArtiBhan,MBBSa,AjayD.Rao,MDb,D.SudhakerRao,MBBSc,*EndocrinolMetabClinNAm39(2010)321–331VitaminDDeficiency;MichaelF;NEJM2007357:266-81Proximalmyopathyinla