儿科学全套课件

皮膚粘膜淋巴結綜合征(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急性發熱性出疹性疾病Anacute,febrilediseasewithrash兒童後天獲得性心臟病的主要病因之一Oneoftheleadingcausesofacquiredheartdiseaseinchildren可併發缺血性心臟病或猝死Mayleadtoischemicheartdiseaseorsuddendeath1967年日本學者首先報導FirstdescribedinJapanin1967byDr.Kawasaki

Epidemiology

發病率(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)發病有一定的家庭集聚現象兄弟姐妹、雙胎髮病率相對較高提示：有遺傳傾向與致病或環境因素相互作用HigherratesofKDinthesiblingsandtwinssuggestapossibleroleforgeneticpredispositionthatinteractswithexposuretotheetiologicagentoragentsintheenvironments流行病學資料(3)季節：冬春季發病居多

Morecommoninwinterandearlyspringmonths流行趨勢：呈一定的區域流行

Occurringinbothendemicandcommunity-wideepidemicforms

冠狀動脈瘤或擴張發生率:15%-25%Coronaryarteryaneurysmsorectasiadevelopin15%-25%ofuntreatedcases住院病死率:約0.17%In-hospitalmortalityrateisabout0.17%再發率: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因數Clinicalandepidemiologicalfeaturessuggestinfectionisetiologyortrigger

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

病理

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

病理（續）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病變的病理分期（1）川崎病心血管相關病變的病理分期（2）

臨床表現ClinicalFindings發熱持續5天以上Feverpersistingatleast5days+雙側球結膜充血,無滲出物Bilateralnonexudativeconjunctivitis周圍肢體變化:Changesinextremities:初期：掌蹠紅斑、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,軀幹為主Theerythematous

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

草莓舌“Strawberrytongue”頸部淋巴結腫大Cervicallymphadenopathy單側性、非化膿性直徑>1.5cmUnilateralNon-suppurativeDiameter>1.5cm肛周及會陰部變化其他表現Otherclinicalfindings

心血管系統：心肌炎、心包炎、心律失常、心衰Cardiovascularsystem:myocarditis,pericarditis,arrythmia, heartfailure消化系統：腹痛、腹瀉、肝功能損害Gastrointestinaltract:abdominalpain,diarrhea,hepaticdysfunction泌尿生殖系統：尿道炎Genitourinarysystem:urethritis其他表現(續）Otherclinicalfindings（continued)中樞神經系統：易激惹，無菌性腦膜炎Centralnervoussystem:extremeirritability,asepticmeningitis肌肉骨骼系統：關節炎、關節痛Musculoskeletalsystem:Arthritis,arthralgia實驗室檢查Laboratoryfindings

白細胞計數增高，粒細胞為主

Leukocytosiswithneutrophilia

血沉增快

Elevatederythrocytesedimentationrate(ESR)C反應蛋白增高

ElevatedC-reactiveprotein(CRP)

血小板計數增高

Thrombocytosis實驗室檢查Laboratoryfindings

貧血，低白蛋白血症

Anemia,Hypoalbuminemia

血清轉氨酶增高

Elevatedserumtransaminases

血清IgG,IgM,IgA,IgE,迴圈免疫複合物增高

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.不明原因發熱5天以上

Feverwithunknownreasonspersisting5+days

B.其他主要臨床表現

Otherprincipalclinicalfeatures1.周圍肢體變化：Changesinextremities:

a.掌蹠紅斑，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腫大(常為單側）

Cervicallymphadenopathywithoutsuppuration

診斷：

1.發熱+其他至少四項主要標準

Diagnosisbsaedon＞5daysfeverandatleast4principalcriteria或

2.發熱+四項以下主要標準＋冠脈病變

＞5daysfeverand＜4principalcriteriawhencoronary arteryabnormalitiesaredetectedl鑒別診斷Differentialdiagnosis

敗血症

Septicemia

猩紅熱

Scarletfever

全身型幼年類風濕關節炎

Syetemicjuvenilerheumatoidarthritis

滲出性多形紅斑

Exudative

polymorphouserythema治療Treatment一.控制炎症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生

Notloweringthefrequencyofthedevelopmentcoronaryabnormalities關於阿司匹林使用的療程DurationofAspirinadministration滿14天，熱退後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天內使用（若可能，7天內使用）

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

一般不用於初治

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,或用於

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.對症治療Symptomatictreatment併發冠狀動脈瘤的危險因素Riskfactorsforcoronaryaneurysms男孩

Malesex年齡<6月或>3歲

Age>6monthsor>3yrs發熱持續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

特發性(體質性)OvarianT.單純性乳房早發育2.CNSDisordersLeydigcellT.2.Prematureadrenarche*space-occupying2.adrenaldisorders單純性陰毛早現

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

單純性乳房早發育PrematureAdrenarche

單純性陰毛早現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

神經遞質去甲腎上腺素

GABA5-HT

MedianEminence(MLT前體)

下丘腦神經內分泌細胞

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/雙髂脊距

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(乳房初發育)Benignprematuremenarche(月經初潮)Boys:Benigngynecomastia(男性乳房發育)FamilialgynecomastiaActions1.Observation/reevaluation2.Consideradditionalassessmenttoconfirmdiagnosis

Treatment

ReassuranceforpatientandparentsAbnormalClinicalevaluationisabnormalorthenormalpubertalmilestonesequenceisdisturbedBoneagemayormaynotbeappropriateforage(prematureassociatedwithcentrallesionsmayappearnormal)

DifferentialDiagnosis

Centralcauses:CNSorpituitarylesionGonadotropin-secretingtumorProfoundhypothyroidismChronicadrenalinsufficiencyPeripheralcauses:McCune-AlbrightsyndromeAdrenalhyperplasia/tumorDifferentialDiagnosis

Girls:Ovariantumor/cystsExogenousestrogenBoys:TesticulartumorAutonomousLegdigfunctionExogenousandrogens

DifferentialDiagnosis

Contrasexualdevelopment(異性化發育）

Girls：

Virilizingadrenaltumors（男性化腎上腺腫瘤）

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

Functionalhyperandrogenism(功能性雄激素過多症）

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.肝鬱腎虛型治法：滋陰降火，疏肝解鬱方藥：知柏地黃丸合丹梔逍遙散2023-12-22118疾病症狀體征輔助檢查＋實驗室檢查診斷鑒別診斷治療預後傳統的授課方式單線式2023-12-22119合理的解释合理的推断足够的证据解决的方法发现的临床问题提出的临床问题???2023-12-22120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……2023-12-22121咳嗽心衰生理性呛咳肿瘤……肺炎上呼吸道感染ProblemBasedLearning2023-12-22122ProblemBasedLearning教學主題What?2023-12-221231。什麼是先天性心臟病？

胚胎發育過程中形成的心血管畸形2。先天性心臟病的發生率是多少？

7‰左右3。先心病是怎樣發生的？

遺傳因素＋環境因數4。先心病主要發生在什麼時候？

胚胎發育早期，4～8周5。通常把先心病分成哪些類型？

肺血、紫紺、分流2023-12-221246。最常見的先心病症狀和體征是什麼？

雜音、紫紺、心衰症狀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。常用於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。大多數能治療。10。先心病有哪些治療方法？

藥物治療、介入治療、手術糾治、心臟移植2023-12-22125症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2023-12-22126男，2歲入幼稚園前體檢發現心臟雜音到醫院就診

怎麼診斷？？？功能性和器質性雜音的鑒別

功能性 器質性年齡 兒童和青少年 任何年齡症狀 無 有雜音部位 肺動脈瓣區、心尖區 特定聽診區，心尖－胸骨區時間、時限 收縮早中期 全收縮期、舒張期或連續性響度

I～II級 II級以上傳導方向 無傳導 沿血流方向與呼吸,體位關係 臥位或運動後明顯 主動脈瓣區雜音坐位前傾時明顯 二尖瓣區雜音左側臥位明顯實驗室檢查 無陽性發現 ECG、X-ray、ECHO有特定表現2023-12-22128雜音3LSBSMIV功能性病理性胸骨左緣心尖區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AVSDECHOECHO2023-12-22129女，4歲 有紫紺

怎麼診斷？？？

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後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]2023-12-22131男孩，1歲4月生後發現動脈導管未閉，家長問：什麼時候治療最好？2023-12-22132藥物治療介入治療手術治療適應症禁忌症治療方案併發症後遺症家長意見療效?RheumatoidDisease

Connectivetissuediseases

（包含有120種與關節、骨骼、肌肉等相關的疾病）

過敏性紫癜（anaphylactoidpurpura）類風濕性關節炎（JRA）SLE（systemiclupuserythematosis）皮肌炎（dermatomyositis）硬皮病（scleroderma）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.BothillnesseshaveelevatedserumIgAlevelsandidenti