儿科学-培训课件PPT
儿科学-培训课件PPT,儿科学,培训,课件,PPT
RicketsofVitaminDDeficiency 刘喜红刘喜红 Liu Liu XihongXihong 广州市妇女儿童医疗中心临床营养科广州市妇女儿童医疗中心临床营养科Clinic Nutrition Department Guangzhou Women Clinic Nutrition Department Guangzhou Women Children Medical Centre Children Medical Centre Cryingbabyatnight夜哭郎夜哭郎VitDsDiscoveryl1650,FrancisGlissonfoundRicketscommonamongtherichpersonl1822,reported:Ricketsrelatedtolackofsunexposurel1824,found:fishliveroilcouldtreatRicketsl1918,aEnglishmanmistakeforricketscausedbythelackofVitAl1921,wasnamedVitD,the4thVitl1936,chemicalcharacteristicsofVitDascertainedThecourseofVitDsdiscoveryistheresultsofpeopletofightagainstricketsObjective&Requirement VitDssourceandmetabolicprocessVitD来源、代谢过程生理功能Etiologyandpathogenesis发病机理、病因Symptomsandsigns各期临床表现 PreventionandTreatment治疗原则和方法VitDsfunction生理功能DefinitionRicketsisthesofteningandweakeningofbones(failuretoassimilateandusecalciumandphosphorusnormally)inchildren,usuallybecauseofanextremeandprolongedvitaminDdeficiency.WheredoesVitaminDcomefrom?AmainsourceofvitaminDismadebyourownbodies.madeintheskinbyexposuretosunlight insomefoods:certainlybehelpful,notsatisfactory,notadailydoseOilyfishFortifiedfoods:infantformulamilkEggyolk,liver,andwildmushroomscontainonlysmallquantitiesofvitaminD.maternal-fetaltranship:infantsThesunshinevitaminSkinproducevitaminDwhenexposedtosunlight10-15minutesofdirectsunlightcangenerate10,000to20,000IU lNote:it is not the same as sunbathing;the skin simply needs to be exposed to sunlight.But,the sunlight has to fall directly on to bare skin(through a window is not enough).lNote:too much exposure to the suns rays can be damaging.Sunburn should be avoided(it can increase the risk of skin cancer).absorb more ultraviolet-B(UVB)in the melanin(黑色素)of their skinsunlightfood25-OHase1-OHasefeedbackregulation RegulationofWitDfeedback:bloodcalcium&phosphorusconcentration,parathyroidglandsandCalcitonin血钙、磷浓度与甲状旁腺、降钙素自身反馈作用WhydoweneedVitaminD?lAmainactionofvitaminDistohelpcalciumandphosphorusinourdiettobeabsorbedfromthegut.lIncreaserenaltubularreabsorptionofcalciumphosphate,especiallyphosphorus lbonecalciummobilization:releasecalciumfromoldbone,calciumdepositesonosteoblast l Calciumandphosphorusareneededtokeepboneshealthyandstrong.lInaddition,vitaminDseemstobeimportantformusclesandgeneralhealth.ThereisalsosomeevidencethatvitaminDmayalsohelptopreventotherdiseasessuchascancer,diabetesandheartdisease Adults:osteoporosis,hypertension,coronaryheartdisease,immunedisease,diabetes,kidneydisease,etc.When/WhyVitaminDDeficiencyHappen?VitaminDdeficiencymeansthatthereisnotenoughvitaminDinyourbody.Broadlyspeaking,thiscanoccurinthreesituations:lThebodyhasanincreasedneedforvitaminDgrowtoofastlThebodyisunabletomakeenoughvitaminDgastrointestinaltractdisease,hepaticandgalldiseases,medicine,etc.lNotenoughvitaminDisbeingtakenmother,food,sunlightCausesofrickets-lack of Vitamin DlSunlight:children now tend to spend less time outdoors.日照不足lFood:breast feeding食物中补充不足lNeedmore:grow rapidly需要增加,生长速度快lMothersstoreofVitD:twins,preterm围生期不足lAbsorptionproblems:diseases,drugs:diarrhea Inflammatory bowel disease Cystic fibrosis Kidney problems疾病影响RiskFactorslAge:3-36 months lDarkskinlNorthernlatitudeslPrematurebirthlAnti-seizuremedicationslExclusivebreast-feedingpathogenesis发病机制发病机制长骨钙化带消失长骨钙化带消失Diagnosis:ClinicalStageslEarlystage/Initialstage(初期/早期):2ypillowbald 枕秃squarehead方颅 pressurealopeciaSkeletalchanges-headlCraniotabes(颅骨软化颅骨软化):Ping-Pong-ballsensationisfeltlDelayederuptionofmilkteeth(出牙延迟出牙延迟):lDelayedclosureofanteriorfontanelle(前囟闭合延迟)前囟闭合延迟):lRachiticrosary(肋骨串珠):lChickenbreast(鸡胸):lFunnelbreast(漏斗胸):lHarrisongroove(郝氏沟):Skeletalchangesthorax(chest)lRachiticbracelet(手镯、脚镯)lGenuvarum(膝内翻)knock knees(O形腿)lGenuvalgum(膝外翻)bow legs(X形腿)SkeletalchangesextremitiesRachiticbraceletKnockkneesBowlegsX-raycuppingandfrayingthedistalendsoftheradiusandulna,Theepiphysealendcalcifyisdisappear,likeacupandbrush.Thebonebecomethinning.Greenstickfracturesoccurinthelongbones,therearenoclinicalsymptoms,maybeonlylateinstandingandwalking.骨钙化带消失,干骺端呈毛刷状、杯口状改变骨质稀疏,骨皮质变薄可有青枝骨折,而无临床症状recoveryphase:2-3weekslater恢复期恢复期12dstreatment:Zonesofpreparatorycalcification出现不规则的钙化线18dstreatment:thezonesofpreparatorycalcificationarewelldefined形成钙化带29ds:zonesofpreparatorycalcificationbecomedenseandshaftshavebecomeunited.钙化带致密增厚Kyphosis驼背Scoliosis脊柱侧弯SkeletalchangesSpinalcolumThediseaseischaracterizedbydeformitiesofthelongbones,enlargementofthewristsandcostochondraljunctions,hypotoniaandcraniotabesanddelayedfontanelleclosure.会坐或站立后,因韧带松弛可致脊柱畸形OtherSymptomsandSigns Poordevelopmentandgrowth:usuallystartwalkingverylate 生长发育落后:生长发育落后:站立、行走晚Vulnerabletoinfections:especiallyrespiratorysystem 容易感染容易感染:特别是呼吸系统Muscleweakness,musclecramps,breathingdifficultiesandseizures 全身肌肉松弛、肌力和肌张力降低Cardiomyopathy:very rare.DiagnosisHistory:inadequate intake of vit DSymptomsandsigns:clinical observationLaboratorytests:the level of 25-(OH)-D,X-rays:can reveal bone deformities.goldenstandardDiagnosis:Biochemicalchangesl25-(OH)D3:decreasedlSerumcalcium,phosphorus:normalorlowlAlkalinephosphatase(ALP):elevatedlBonealkalinephosphatase(BALP):elevatedDifferentialDiagnosislX-linkedhypophosphatemicrickets:sex-linkedinheritance,somethingwrongwiththephosphorusreabsorptionontherenaltubule低低血磷抗维生素血磷抗维生素D D佝偻病佝偻病lVitaminD-dependentrickets:autosomalrecessiveinheritance,type1and2,veryseriousVitDVitD依赖性佝偻病依赖性佝偻病lDistalrenaltubularacidosis:secondaryparathyroidglandhyperfunction,metabolicacidosis远端肾小管酸中毒远端肾小管酸中毒lRenalrickets:congenitalorAcquiredchronicRenaldysfunctionlHepaticrickets:hepaticfunctiondamage lChondrodystrophyachondroplasia软骨营养不良软骨营养不良Mucopolysacharidosis(MPS)黏多糖病黏多糖病Hydrocephalus脑积水脑积水TreatmentlTreatment is simple:Most cases of rickets can be treated with vitamin D or calcium supplements,which may vary with the child age.Too much vitamin D can be dangerous.lNatural and artificial light are effective therapeutic.lRickets due to a genetic condition may require additional medications or other treatment.lSome skeletal deformities caused by rickets may need corrective surgery.lVitD:1)VitDdaily2000-4000IUor1,25-(OH)2D30.5-2.0gfor1m,400IU/d(1y,600IU/d)2)VitD3200000-300000IUim,singledose,after3m,400IU/dTreatmentPrognosislExcellent:ifsufficientamountofvitDareadiministered,healingbeginswithinafewdaysandprogressesslowlyuntilthenormalbonystructureisrestored.lIsnotafataldisease,butcomplicationsandintercurrentinfectionssuchaspneumonia,tuberculosis,andenteritisaremorelikelytocausedeathofrachiticchildrenthannormal.lOntheotherhand,deformitiessuchasbowedlegsandtherachiticrosarypersistthroughoutadultlife.PreventionlCanbepreventedbyexposuretosunlightorbyoraladministrationofVitD.lThedailyrequirementofVitDis400IU.lPrematureinfantsorbreast-fedinfantswhosemothersarenotexposedtoadequatesunlightshouldreceivesupplementalVitDdailypreterminfants:800IU/d,3monthslaterchangetoprophylacticdosesTerminfants:beginafter2weeksuntilto2yearsoldlVitDshouldalsobeadministeredtopregnantandlactatingmothers.维生素维生素D缺乏性手足搐搦症缺乏性手足搐搦症 TetanyofVitaminDDeficiencylclinicalmanifestation,diagnosis,treatmentandprevention掌握本病的临床表现、诊断和防治原则掌握本病的临床表现、诊断和防治原则letiology,pathogeny病因和发病机理ldifferentialdiagnosis鉴别诊断Objective&Requirement 发病机制发病机制长骨钙化带消失骨矿化受阻骨矿化受阻lTetanyduetodeficiencyofvitDoccasionallyaccompaniesrickets.lProbablyasaresultofdeficientabsorptionofbothvitDandcalcium.lOccursmostfrequentlybetweentheagesof4mand3yr.especially6monthsEtiologyandPathogenesislVitDdeficiencylBloodcalciumdecreaselparathyroidglandsincompensation BloodcalciumTotalcalcium1.75-.88mmol/LIoncalcium1.0mmol/LNeuromuscularexcitability神经肌肉兴奋性增高tetany抽搐抽搐parathyroidglandstired?ClinicalFeatures临床表现临床表现lLatenttenancy隐匿型隐匿型:theserumcalciumlevelis1.75-1.88mmol/L,symptomsarenotevident,buttheycanbeelicitedbymeansof:chvosteksigh面神经征面神经征Trousseausigh陶瑟征陶瑟征pernealreflex腓反射腓反射ClinicalFeatureslManifesttenancy典型发作典型发作:theserumcalciumlevelis1.75mmol/L,spontaneousclinicalmanifestationsinclude:convulsions惊厥惊厥:withoutfever,mostcommonseencarpopedalspasm手足搐搦手足搐搦laryngospasm喉痉挛喉痉挛DiagnosislBasedonthecombinedpresenceofrickets,lowserumcalciumlevel,andsymptomsoftetany.lTheserumphosphoruslevelisusuallylowlALPlevelisincreased.OutburstrepeatedattacksoberafterattacknosignofnervoussystemRicketshypocalcemiaDifferentialDiagnosisHypoglycemia低血糖症Hypomagnesiumia低血镁症infantilespasms婴儿痉挛症primaryparathyroidfunctiondecline原发性甲状旁腺功能减退centralnervoussysteminfection中枢神经系统感染acutelaryngitis急性喉炎TreatmentlOxygeninhalationisindicatedduringconbulsiveseizures.lCalciumsupplements:calciumchloride,calciumlactate,calciumgluconate:canbeadministeredintravenously,5-10ml.lSodiumphenobarbitalmaybegivenintramuscularly,whencalciumgluconatedoesnotquicklycontroltheattacks.lProlongedattacksoflaryngospasmareusuallycontrolledbysedationandbycalciumsalts.lAftertheacutemanifestationshavebeencontrolled,VitDindailydosesshouldbestartedwiththeoraladministrationofcalcium.lWhenthericketsishealed,thedoseofVitDshouldbedecreased.TreatmentlEmergentTreatment:OxygenInhalation吸氧Controlconvulsionsorlaryngospasm控制惊厥或喉痉挛:chloralhydrate,diazepam 10%水合氯醛保留灌肠、地西泮0.1-0.3mg/kg肌注或静注lTreatmentwithcalcium:补钙10%calciumgluconate5-10ml,intravenousinjectionslowlyafterdilution lVitDtreatment:补VitD:E-mail:http:/
收藏