兒科學全套課件

皮膚粘膜淋巴結(jié)綜合征(川崎病)

MucocutaneousLymphNodeSyndrome,MCLS

(KawasakiDisease,KD)

概貌Outline全身血管炎(中、小動脈)為主要病理改變Generalizedsystemicvasculitis急性發(fā)熱性出疹性疾病Anacute,febrilediseasewithrash兒童后天獲得性心臟病的主要病因之一Oneoftheleadingcausesofacquiredheartdiseaseinchildren可并發(fā)缺血性心臟病或猝死Mayleadtoischemicheartdiseaseorsuddendeath1967年日本學者首先報道FirstdescribedinJapanin1967byDr.Kawasaki

Epidemiology

發(fā)病率(Annualincidence)：

日本—108-111.7/10萬(97－98年)

澳大利亞－5.5/10萬(93-94年）

瑞典－6.2/10萬（91-92年） 智利－2.9/10萬（92-94年） 美國白種人－9/10萬

美國亞裔32.5/10萬

臨床流行病學資料

中國

北京－18.2-27.8/10萬（95-99年）

上海－16.2-36.7/10萬（85-03年）

香港－25.4/10萬(89-94年）

臺灣－24.8/10萬

(86年）

亞裔人種發(fā)病率較高

年齡：

約80％病例<5歲

About80%casesare<5yearsold性別：

男:女之比1:1.5-1.7

Boysoutnumbergirlsby1:1.5-1.7流行病學資料(2)發(fā)病有一定的家庭集聚現(xiàn)象兄弟姐妹、雙胎發(fā)病率相對較高提示：有遺傳傾向與致病或環(huán)境因素相互作用HigherratesofKDinthesiblingsandtwinssuggestapossibleroleforgeneticpredispositionthatinteractswithexposuretotheetiologicagentoragentsintheenvironments流行病學資料(3)季節(jié)：冬春季發(fā)病居多

Morecommoninwinterandearlyspringmonths流行趨勢：呈一定的區(qū)域流行

Occurringinbothendemicandcommunity-wideepidemicforms

冠狀動脈瘤或擴張發(fā)生率:15%-25%Coronaryarteryaneurysmsorectasiadevelopin15%-25%ofuntreatedcases住院病死率:約0.17%In-hospitalmortalityrateisabout0.17%再發(fā)率:1-3%Recurrencerateisreportedtobe1-3%

病因

Etiology病因仍不明確TheetiologyofKDremainsunknown臨床和流行病學資料提示感染是可能的病因或觸發(fā)因子Clinicalandepidemiologicalfeaturessuggestinfectionisetiologyortrigger

細菌毒素：超抗原學說Bacterialtoxin:ThereisahypothesisthatKDisrelatedwithsuperantigen可能與免疫異常有關(guān)ItispossiblethatKDresultsfromanimmunologicresponsetriggeredbysomemicrobialagents

病理

Pathology全身性血管炎Generalisedsystemicvasculitisinvolvingbloodvesselsthroughoutbody.動脈瘤形成Aneurysmsoccurringinarteries

病理（續(xù)）動脈重塑狹窄ArteialremodelingStenosisMCLS全身小動脈血管炎肱A、髂A(2%)腎A(1%)腎性高血壓腸系膜A(＜1%)腸梗阻休克破裂腦血管肺A

肺A高壓肺梗塞視力障礙腦梗塞川崎病心血管相關(guān)病變的病理分期（1）川崎病心血管相關(guān)病變的病理分期（2）

臨床表現(xiàn)ClinicalFindings發(fā)熱持續(xù)5天以上Feverpersistingatleast5days+雙側(cè)球結(jié)膜充血,無滲出物Bilateralnonexudativeconjunctivitis周圍肢體變化:Changesinextremities:初期：掌跖紅斑、手足硬腫Acute:Erythemaofpalms,soles;edemaofhands,feet恢復期：病程2-3周指、趾膜狀脫皮Subacute:Periungualpeelingoffingers,toesinweeks 2and3常見紅斑性、斑丘疹樣皮疹,軀干為主Theerythematous

rashormaculopapulareruptioniscommon,mainlywithinvolvementofthetrunk口唇及口腔改變:Changesofthelipsandoralcavity:口唇干裂、剝脫、出血Dryness,fissuring,peelingandbleedingofthelips

草莓舌“Strawberrytongue”頸部淋巴結(jié)腫大Cervicallymphadenopathy單側(cè)性、非化膿性直徑>1.5cmUnilateralNon-suppurativeDiameter>1.5cm肛周及會陰部變化其它表現(xiàn)Otherclinicalfindings

心血管系統(tǒng)：心肌炎、心包炎、心律失常、心衰Cardiovascularsystem:myocarditis,pericarditis,arrythmia, heartfailure消化系統(tǒng)：腹痛、腹瀉、肝功能損害Gastrointestinaltract:abdominalpain,diarrhea,hepaticdysfunction泌尿生殖系統(tǒng)：尿道炎Genitourinarysystem:urethritis其它表現(xiàn)(續(xù)）Otherclinicalfindings（continued)中樞神經(jīng)系統(tǒng)：易激惹，無菌性腦膜炎Centralnervoussystem:extremeirritability,asepticmeningitis肌肉骨骼系統(tǒng)：關(guān)節(jié)炎、關(guān)節(jié)痛Musculoskeletalsystem:Arthritis,arthralgia實驗室檢查Laboratoryfindings

白細胞計數(shù)增高，粒細胞為主

Leukocytosiswithneutrophilia

血沉增快

Elevatederythrocytesedimentationrate(ESR)C反應蛋白增高

ElevatedC-reactiveprotein(CRP)

血小板計數(shù)增高

Thrombocytosis實驗室檢查Laboratoryfindings

貧血，低白蛋白血癥

Anemia,Hypoalbuminemia

血清轉(zhuǎn)氨酶增高

Elevatedserumtransaminases

血清IgG,IgM,IgA,IgE,循環(huán)免疫復合物增高

ElevatedserumIgG,IgM,IgA,IgEandCIC心電圖Electrocardiogram

竇性心動過速，S-T段抬高

Sinustachycardia,elevatedS-Tsegment超聲心動圖

Echocardiogram

心包積液，瓣膜反流

Pericardialeffusion,valvularregurgitation冠狀動脈瘤（擴張）Coronaryaneurysm(ectasia)冠狀動脈造影CoronaryangiogramABC?臨床診斷標準Clinicalcriteriafordiagnosis

A.不明原因發(fā)熱5天以上

Feverwithunknownreasonspersisting5+days

B.其它主要臨床表現(xiàn)

Otherprincipalclinicalfeatures1.周圍肢體變化：Changesinextremities:

a.掌跖紅斑，手足硬腫

Erythemaofpalms,soles;edemaofhands,feet

b.指、趾膜狀脫皮

Periungualpeelingoffingers,toes臨床診斷標準Clinicalcriteriafordiagnosis

2.多形性皮疹

Polymorphousexanthem

3.雙眼球結(jié)膜充血（無滲出）

Bilateralbulbarconjunctivalinjection(withoutexudate)

4.口唇潮紅、皸裂、草莓舌、口咽粘膜充血

Erythema,dryness,cracking(,bleeding)ofthelips;strawberrytongue;diffuseinjectionoforalandpharyngealmucosae臨床診斷標準Clinicalcriteriafordiagnosis

5.非化膿性頸部淋巴結(jié)腫大(常為單側(cè)）

Cervicallymphadenopathywithoutsuppuration

診斷：

1.發(fā)熱+其它至少四項主要標準

Diagnosisbsaedon＞5daysfeverandatleast4principalcriteria或

2.發(fā)熱+四項以下主要標準＋冠脈病變

＞5daysfeverand＜4principalcriteriawhencoronary arteryabnormalitiesaredetectedl鑒別診斷Differentialdiagnosis

敗血癥

Septicemia

猩紅熱

Scarletfever

全身型幼年類風濕關(guān)節(jié)炎

Syetemicjuvenilerheumatoidarthritis

滲出性多形紅斑

Exudative

polymorphouserythema治療Treatment一.控制炎癥Anti-inflammation

1.阿司匹林Aspirin

30-100mg/kg.d每日3-4次（qid)

具抗炎、抗血小板作用

Withactivitiesofanti-inflammationandanti-platelet

不能降低冠脈病變的發(fā)生

Notloweringthefrequencyofthedevelopmentcoronaryabnormalities關(guān)于阿司匹林使用的療程DurationofAspirinadministration滿14天，熱退后2-3天減量至3-5mg/kg.d

(Highdose)redusedto3-5mg/kg.dafter14daysofillnessandthechildhasbeenafebrilefor48-72hrs

a.無冠脈病變，維持6-8周

maintainedfor6-8weeksifnocoronaryabnormalitiesdetacted

b.有冠脈病變，維持至恢復

Continuedindefinitelytillrecover

注意瑞氏（肝腦脂肪變性）綜合征

Reyesyndrome

消化道不良反應2.丙種球蛋白靜脈滴注IVIG

2g/kg.d一次靜脈滴注8-12小時

2g/kg.dinasingleinfusionfor8-12hrs

與阿司匹林合用

Togetherwithaspirin

病程10天內(nèi)使用（若可能，7天內(nèi)使用）

Startedwithinfirst10daysofillness.(Ifpossible,within7daysofillness)3.皮質(zhì)激素Steroids

一般不用于初治

NotusedfortheinitialtreatmentofKD

對冠脈病變的療效不確切

Theeffectsoncoronaryarteryabnormalitiesstilluncertain

與靜脈丙球和阿司匹林合用,或用于

UsedtocombindwithIVIGandaspirinor

初治失敗的病例

Tothecasesfailedtorespondtoinitialtherapy二.抗血小板、抗凝治療

Anti-plateletandanticoagulanttherapy

雙嘧達莫(潘生丁)

Dipyridamole(Persantin)

華法林

Warfarin

低分子肝素

Low-molecular-weightheparin三.對癥治療Symptomatictreatment并發(fā)冠狀動脈瘤的危險因素Riskfactorsforcoronaryaneurysms男孩

Malesex年齡<6月或>3歲

Age>6monthsor>3yrs發(fā)熱持續(xù)2周以上

Fevermorethan2wks血沉>

100mm/hESR>100mm/h血紅蛋白<80g/L

Hemoglobin<80g/L白細胞>16x109/L

WBC>16x109/LAdolescenceAdolescence,pubertybetweenchildhoodandadulthood

featurestressfuldevelopmentalmajorchanges

physicalmaturitysexualitycognitive認知emotionalfeelingsrelationshipswithothers

Range:boy11～19girl10～21AdolescentMedicine

Objectiveteenagerjuvenileyoungsteraddressingidentifiedhealthconcernsthecomplicatedinteractionsofdevelopmentalchangesonhealthcareneedstheeffectivenessoftreatmenthealtheducationhealthpromotion

Contents

PreclinicalMedicineClinicalMedicinePedagogyorEducationPsychologyHygieneSocialMedicineBehaviorMedicine

NormaldevelopmentalperiodPuberty

PhysiologicalbasisDevelopment&changesSexualcharacterbystagesPhysicalbuild,constitution,statueMentalitybehavior

ClassificationofSexMaturity

StagesinGirls

Tannerbreastpubichairsstages

乳房陰毛P1prepubertalB1PH1P2earlystageB2PH2P3metaphaseB3PH3P4anaphaseB4PH4P5adultstageB5PH5

ClassificationofSexMaturity

StagesinBoysTannerGenitaliaPubichairsstages外生殖器陰毛P1prepubertalG1PH1NoneP2earlystageG2PH2Scanty,long,slightlypigmentedP3metaphaseG3PH3Darker,starttocurlsmallinamountP4anaphaseG4PH4Resemblesadulttypelessinquantity,curlyP5adultstageG5PH5adultdistributionrhombusinshape

PrecociousPuberty

Definition

Onsetofsecondarysexualcharacteristicsbefore8yingirlsand9Yinboys

GlobalIncidence

0.6～1％boys∶girls＝1∶51998Shanghai30,0001%ClassificationandConditionsCausing

PrecociousPubertyTrueP.P.Pseudo.P.PIncompleteP.P.1.Idiopathic1.Tumors1.Prematurethelarche

特發(fā)性(體質(zhì)性)OvarianT.單純性乳房早發(fā)育2.CNSDisordersLeydigcellT.2.Prematureadrenarche*space-occupying2.adrenaldisorders單純性陰毛早現(xiàn)

lesions*Congenitaladrenal3.Prematuremenarche*congenitalCNShyperplasia孤立性早潮

anomalies*Secondaryadrenal*postinfectionorhyperplasiatramua*adrenaltumor3.Hypothyroidism3.GnRHinducedTumor4.Exogenoussexhormones5.McCune-AlbrightSyndrome

Gonadotrophin-dependentP.P.

(TrueP.P.)

IdiopathicP.P.EncephalicTumorCongenitalHypothyroidism

Gonadotrophin-independentP.P.

(pseudoP.P.)OvarianTumorCongenitalAdrenalHyperplasiaSecondaryAdrenalHyperplasiaGnRHinducedTumorExogenoussex-steroidsMcCune-AlbrightSyndrome

IncompletePrecociousPuberty

(partialP.P.)

PrematureThelarche

單純性乳房早發(fā)育PrematureAdrenarche

單純性陰毛早現(xiàn)PrematureIsolatedmenarche

孤立性早潮LaboratoryTest

BoneageGP（Greulich-Pyle)/TW2/TW3PelvicUltrasonography

SexSteroidsGnRHStimulationTestMRIRadiographytubularboneDifferentialDiagnosisDifferentialDiagnosis

TreatmentofP.P.

MedicationTraditionalChineseMedicine

TheEndocrineChangesofPuberty

Hypothalamus-Pituitary-Gonads,AdrenalGlandsAxis

neurotransmittersNoradrenalin

C.N.S

神經(jīng)遞質(zhì)去甲腎上腺素

GABA5-HT

MedianEminence(MLT前體)

下丘腦神經(jīng)內(nèi)分泌細胞

GnRH

(-)(+)Pituitary

Gn:LH、FSH

Gonads

Ovary（E2)testicle(T)SecondarySexualDevelopment

Normalpuberty(average)girls10y～11yboys12～13yFactorsindividual,hereditary,nutritional,morbid,psychologicalNormalSexualDevelopmentSequenceGirls:breast－labia–pigmentation-vaginalsecretion-pubichair-menarche13yboys：

Testis-penis-scrotum-pubic&axillaryhair–beard-Adam'sapple-spermatorrhea15ys

Changesinphysicalbuildandstatue

1.LinearitygrowthThepubertalgrowthspurt2Y

增長高峰（PHV）

Girls:10Y-18Y25cm16YBoys:12Y-20y28cm17YFoot-calf-thigh-trunkTop/lowerpartPreadolescence=1.1Postadolescence=0.9

Changesinphysicalbuildandstatue

2.Acromion/IliacCrest雙肩峰距/雙髂嵴距

male1.37female1.35-1.27boysViscerocranium-frontalsinusmandible3.Bodycompositionleanbodymass(LBM)Prepubertyboys≈girlsPubertyboys’LBM,bone≈1.5timesofgirls’Girls’bodyfat≈2.0timesofboys’

TannerStaging

Girls(B1)

Prepubertal,elevationofpapillaonly

TannerStaging

Girls(B2)

Breastbudappearsunderenlargedareola

TannerStaging

Girls(B3)

Breasttissuegrowsbeyondareolawithoutcontourseparation

TannerStaging

Girls(B4)

Projectionofareolaandpapillaformsasecondarymound

TannerStaging

Girls(B5)

Adultbreastcontourwithprojectionofpapillaonly

TannerStaging

Girls(PH1)

Prepubertalvillushaironly

TannerStaging

Girls(PH2)

Sparsegrowthofslightlypigmentedhairalongthelabia

TannerStaging

Girls(PH3)

Hairiscoarser,curledandpigmentedspreadsacrossthepubes

TannerStaging

Girls(PH4)

Adult-typehairbutnospreadtomedialthigh

TannerStaging

Girls(PH5)

Adult-typehairwithspreadtomedialthighbutnotuplineaalba

TannerStaging

Boys(G1,PH1)

PrepubertalTestes:<2.5cmvillushaironly

TannerStaging

Boys(G2,PH2)

ThinningandreddeningofscrotumTestes:2.5to3.2cmSparsegrowthofslightlypigmentedhairatbaseofpenis(12.3years)

TannerStaging

Boys(G3,PH3)

Growthofpenis,especiallylength(13.2years)Testes:3.3to4.0cmThicker,curlierhairspreadstothemonspubis

TannerStaging

Boys(G4,PH4)

Growthofpenisandglands,darkeningofscrotumTestes:4.1to4.5cmAdult-typehairbutnospreadtomedialthigh

TannerStaging

Boys(G5,PH5)

AdultgenitaliaTestes:>4.5cmAdult-typehairwithspreadtomedialthighsbutnotuplineaalba

TrueP.P.GnRH-independentHypothalamus-Pituitary-Gonad-AxisPrematureactivation&hyperfunctionReproductivecapacity

PseudoP.P.GnRH-IndependentEndogenous/ExogenousHypothalamus-pituitary-axis↙Nonereproductivecapacity

Exogenous

Intakeofexogenoussexsteroids

contraceptive避孕藥royaljelly蜂王漿

chickembryo雞胚silkwormchrysalis蠶蛹

colostrum動物初乳

pollen花粉etc.

IdiopathicP.P.

80%TrueP.P.TheNeuroendocrineFunctionalFactors①NutritionalFactors②EnvironmentalFactors

detergent(alkylatedphenol)（烷基化苯酚類）

agrochemicals(organochlorine)（有機氯殘留）

plasticmaterial(plasticizer-santicizer218a,bisphenolA)(鄰苯二甲酸酯類及雙酚A）③Socialfactor

IntracranialTumor

Man>WomenEncephalicspaceoccupyinglesion:Symptoms:polydipsia,polyuriahyperphagiaobesenessDisorders:hamartoma,

astrocytoma,ependymocytoma,pinealoma

錯構(gòu)瘤、星形細胞瘤、室管膜瘤、松果體瘤

CongenitalHypothyroidism

Prolonged/UntreatedT4

-Feedback

HypothalamusTRH

PituitaryPRL

TSH

FSH、LH

McCune-AlbrightSyndromeSomaticcellmutationsNonegermlineGeneticbutnotheritableMutationsstimulatoryG-proteinsubunitadenylcyclasesystemaffectedcellsmembranereceptor

PrematureThelarcheTransientcondition<2yNoneProgressiveIsolatedbreastdevelopmentunilateralBenign–?firstsignTrueP.P.orPseudoP.P.?Exogenousexposuretoestrogen

PrematureAdrenarche

pubicandaxillaryhair

？nonclassic21-hydroxylasedeficiencyBoneage/growthvelocitySomegirlsmildinsulinresistancepolycysticovarysyndromenotabenignconditionIdiopathicPrecociousPuberty

80%girls,40%boys,somefamilialOnset4-8ys/InfancyNormalpubertalsequencegirlsbreast-publicandaxillaryhair-menacheBoystesticular-penile-publicandaxillaryhairEpiphysealclosureearlyMatureintoshortadultsIdiopathicPrecociousPubertyEncephalicTumorCongenitalHypothyroidismProlonged/UntreatedGirls:BreastdevelopmentBoys:testisenlargementOthersymptomsCoarsedfacies,lethargy,hypotonia,constipation,anemia,mentalretardation,dullresponse,etc.GrowthandMaturationofBonelaggardbehind

Congenitaladrenalhyperplasia

SimpleVirilizing11-hydroxylase（11-羥化酶缺陷）

Boys：

isosexualprecociouspubertyGirls:

heterosexualprecociouspubertypseudohermaphrodism假兩性畸形Intakeofexogenoussexsteroids

SymptomsofpseudoP.P.Girlsbreastenlargermentareola乳暈nympha小陰唇

pigmentation

vaginadischargeirregularvaginalbleedingBoysbreastenlargermentareolapigmentationtestis/penisNoenlargermentPrecociousPubertywith

PolyostoticFibrousDysplasia

andAbnormalPigmentationRadiographicAtlasoftheHandandWristAdditionalAssessmentT3、T4、TSH

CongenitalHypothyroidismT、E2

OvarianTumor/LedigCellTumor17-OHP/（17－KS)

CongenitalAdrenalHyperplasia

GnRHStimulationTest

LHRH:0’-30’-60’-90’-120’pituitarycellreservingfunctionDifferentionofTrueP.P.withPseudoP.P.

MRIMRI

DiagnosisandDifferentialDiagnosis

InitialassessmentClinicalhistoryandphysicalexaminationPubertalmilestoneandgrowthchartRadiographofleftwristforboneageUnremarkableNormalVariationAbnormalDiagnosisPossiblediagnosesDifferentialdiagnosisActionsActionsActionsTreatmentTreatmentUnremarkableClinicalevaluationisunremarkableandrevealsanearlybutotherwisenormalpubertywithnormalmilestonesequenceBoneageisgreaterthanchronologicageDiagnosisTrueIdiopathicPrecociousPubertyActions1.Observation/reevaluation2.Consideradditionalassessment(FSH,LH,Estradialortestosterone,TSH,thyroidhormoneandhCGlevelsaGnRHstimulationtest)IdiopathicPrecociousPubertyMRItoexcludeapathologiccause(e.g.CNSlesion)Treatment1.Psychologicsupport2.ConsidersuppressionofFSHandLHwithGnRHanaloginselectedpatientsNormalVariationClinicalevaluationisunremarkableexceptforapatternconsistentwithanormalvariationofpubertyBoneageinconsistentwithchronologicagePossiblediagnosesBenignprematureadrenarcheGirls:Benignprematurethelarche(乳房初發(fā)育)Benignprematuremenarche(月經(jīng)初潮)Boys:Benigngynecomastia(男性乳房發(fā)育)FamilialgynecomastiaActions1.Observation/reevaluation2.Consideradditionalassessmenttoconfirmdiagnosis

Treatment

ReassuranceforpatientandparentsAbnormalClinicalevaluationisabnormalorthenormalpubertalmilestonesequenceisdisturbedBoneagemayormaynotbeappropriateforage(prematureassociatedwithcentrallesionsmayappearnormal)

DifferentialDiagnosis

Centralcauses:CNSorpituitarylesionGonadotropin-secretingtumorProfoundhypothyroidismChronicadrenalinsufficiencyPeripheralcauses:McCune-AlbrightsyndromeAdrenalhyperplasia/tumorDifferentialDiagnosis

Girls:Ovariantumor/cystsExogenousestrogenBoys:TesticulartumorAutonomousLegdigfunctionExogenousandrogens

DifferentialDiagnosis

Contrasexualdevelopment(異性化發(fā)育）

Girls：

Virilizingadrenaltumors（男性化腎上腺腫瘤）

Congenitaladrenalhyperplasia(先天性腎上腺皮質(zhì)增生）

Functionalhyperandrogenism(功能性雄激素過多癥）

Boys:Feminizingadrenaltumor(女性化腎上腺腫瘤）Actions1.Assessmentforexposuretoexogenoussexsteroids2.AdditionaltestingdirectedatunderlyingcausesuggestedbyinitialclinicalassessmentTreatment1.GnRHanalog促性腺激素釋放激素擬似劑2.Cyproteroneacetate(環(huán)丙孕酮，色普龍）3.Danazol(達那唑）

DifferentkindsofGnRHanalogs

Brandstructurerelativeefficiencydosage

（天然GnRH=1）（μg/kg）LueprolinD-Leu615

20-50H亮丙瑞林140-300MBuserelinD-Ser6201200-1800噴鼻布舍瑞林20-40

HTriptorelinD-Trp636

20-40

H曲普瑞林50-100MDeslorelinD-Trp6144

4-8

H色氨瑞林NafarelinD-Nal6200800-1600噴鼻那法瑞林4

HHistrelinD-His6210

8-10H

組氨瑞林TraditionalChineseMedicine

①.陰虛火旺型治法：滋陰降火方藥：知柏地黃丸（湯）加減②.肝郁腎虛型治法：滋陰降火，疏肝解郁方藥：知柏地黃丸合丹梔逍遙散2023/12/16118疾病癥狀體征輔助檢查＋實驗室檢查診斷鑒別診斷治療預后傳統(tǒng)的授課方式單線式2023/12/16119合理的解釋合理的推斷足夠的證據(jù)解決的方法發(fā)現(xiàn)的臨床問題提出的臨床問題???2023/12/16120咳嗽反射疾病呼吸道心血管胸腔消化道……感染異物腫瘤咽喉氣道肺部……2023/12/16121咳嗽心衰生理性嗆咳腫瘤……肺炎上呼吸道感染ProblemBasedLearning2023/12/16122ProblemBasedLearning教學主題What?2023/12/161231。什么是先天性心臟??？

胚胎發(fā)育過程中形成的心血管畸形2。先天性心臟病的發(fā)生率是多少？

7‰左右3。先心病是怎樣發(fā)生的？

遺傳因素＋環(huán)境因數(shù)4。先心病主要發(fā)生在什么時候？

胚胎發(fā)育早期，4～8周5。通常把先心病分成哪些類型？

肺血、紫紺、分流2023/12/161246。最常見的先心病癥狀和體征是什么？

雜音、紫紺、心衰癥狀7。最常見的先心病畸形有哪些？各占比例多少？

VSD30-50%，ASD20%，PDA10％，TGA5%，TOF5%8。常用于先心病診斷的輔助檢查有哪些？

ECG、X線、心超、心導管和心血管造影9。先心病能治好嗎？

因病而異，因人而異。大多數(shù)能治療。10。先心病有哪些治療方法？

藥物治療、介入治療、手術(shù)糾治、心臟移植2023/12/16125癥狀體征有病無病不清楚檢查＋觀察不清楚檢查＋觀察疾病定位GHC必要的檢查明確診斷治療方案疾病診斷和鑒別診斷過程2023/12/16126男，2歲入幼兒園前體檢發(fā)現(xiàn)心臟雜音到醫(yī)院就診

怎么診斷？？？功能性和器質(zhì)性雜音的鑒別

功能性 器質(zhì)性年齡 兒童和青少年 任何年齡癥狀 無 有雜音部位 肺動脈瓣區(qū)、心尖區(qū) 特定聽診區(qū)，心尖－胸骨區(qū)時間、時限 收縮早中期 全收縮期、舒張期或連續(xù)性響度

I～II級 II級以上傳導方向 無傳導 沿血流方向與呼吸,體位關(guān)系 臥位或運動后明顯 主動脈瓣區(qū)雜音坐位前傾時明顯 二尖瓣區(qū)雜音左側(cè)臥位明顯實驗室檢查 無陽性發(fā)現(xiàn) ECG、X-ray、ECHO有特定表現(xiàn)2023/12/16128雜音3LSBSMIV功能性病理性胸骨左緣心尖區(qū)心肌炎心肌病風濕性心臟病先心病2y，有震顫肺血少肺血正常肺血多X片：肺充血，無紫紺紫紺型非紫紺型VSDASDPDA其他AVSDECHOECHO2023/12/16129女，4歲 有紫紺

怎么診斷？？？

周圍性 中央性部位 四肢末梢、面頰、鼻尖、耳廓 全身（舌、粘膜）37

C10分鐘 減輕 無變化吸100％氧 減輕或消失 不變或減輕吸O2后PaO2 >150mmHg <150mmHg*SaO2

正常 <94％*Hb<80g/L時，SaO2需<63％，才顯示青紫。

Hb>240g/L時，SaO2

88％，即顯示青紫。[青紫]2023/12/16131男孩，1歲4月生后發(fā)現(xiàn)動脈導管未閉，家長問：什么時候治療最好？2023/12/16132藥物治療介入治療手術(shù)治療適應癥禁忌癥治療方案并發(fā)癥后遺癥家長意見療效?RheumatoidDisease

Connectivetissuediseases

（包含有120種與關(guān)節(jié)、骨骼、肌肉等相關(guān)的疾?。?/p>

過敏性紫癜（anaphylactoidpurpura）類風濕性關(guān)節(jié)炎（JRA）SLE（systemiclupuserythematosis）皮肌炎（dermatomyositis）硬皮?。╯cleroderma）風濕熱（rheumaticfever）川崎病(Kawasakidisease)RheumatoidDiseaseinChildrenThemostcommondiseases：JuvenileRheumatiodArthritisHenoch-SchonleinpurpuraKawasakiDisease(MucocutaneousLymphNodeSyndrome,MCLS)RheumaticFever冠狀動脈造影CoronaryangiogramHenoch-SchonleinpurpuraAlternativenames:AnaphylactoidpurpuraVascularpurpuraDefinitionHenoch-Schonleinpurpuraisadiseasethathasthesymptomspurplespotsontheskinjointpaingastrointestinalsymptomsglomerulonephritis(atypeofkidneydisorder)HistorystorySchonlein'sPurpura

(In1837,JohanSchonlein)theskinandjointsareaffectedbutthegastrointestinaltractisnot

Henoch'sPurpura(Later,hisstudent,EduardHenoch)purpuraspotsontheskinandacuteabdominalproblemsnotaffectedbyjointdiseaseGeneralconditionsatypeofhypersensitivityvasculitisandinflammatoryresponsewithinthebloodvesselItiscausedbyanabnormalresponseoftheimmunesystemTheexactcauseforthisdisorderisunknown.Thesyndromeisusuallyseeninchildren,butpeopleofanyagemaybeaffectedItismorecommoninboysthaningirlsManypeoplewithHenoch-SchonleinpurpurahadanupperrespiratoryillnessinthepreviousweeksCausesandpathogenesisAninflammatorydiseaseofthesmallbloodvessels(capillaries)

usuallyaself-limiteddiseaseItisthemostcommonformofchildhoodvascularinflammation(vasculitis)andresultsininflammatorychangesinsmallbloodvesselsCommonallergensincludeMedications(suchascertainantibiotics,antihistaminesandthiazidediuretics)Pathogens(bacterium/virus/parasite,suchasgroupAstrep,varicella,parvovirusandhepatitisB)AnacuterespiratoryinfectionCommonallergensincludefoods(suchasberries,shellfish,fish,nuts,eggs,milk,andothers)pollen

animaldander(scalesofshedskin)insectbites

Certainvaccinations,suchasformeasles,yellowfeverandcholera

Pathophysiology

asmall-vesselvasculitischaracterizedbyimmunoglobulinA(IgA),C3,andimmunecomplexdepositioninarterioles,capillaries,andvenulesHSPandIgAnephropathyarerelateddisorders.BothillnesseshaveelevatedserumIgAlevelsandidenticalfindings