新生儿肺透明膜病(课件)

NeonatalHyalineMembraneDisease(HMD)

新生儿肺透明膜病

1WhatisHMD?

alsocalled,

NeonatalRespiratoryDistressSyndrome(NRDS)-新生儿呼吸窘迫综合征

一.

Introduction2Mostcommoncauseof

respiratoryfailure

intheprematureinfants,correlatingwithimmaturestructuralandfunctionallung.

OccurringfrequentlyininfantswithGA＜34w,theincidenceisinverselyproportionaltotheGAandbirthweight.Reasonissurfactantdeficiency,inturn,leadedtoatelectasis.Thereishyalinemembraneincollapsedalveoli

byphotomicrograph.

3incidence

≤28w:50%～80%32to36w:

15%～30%＞37w:＜5%

Rarelyatfullterminfants≤4二.EtiologyandPathophysiologyWhatcausesHMD?

WhoareaffectedbyHMD?5Etiology(1)----deficiencyofPulmonary

Surfactant

（basiccause）6Pulmonary

Surfactant(PS)--肺表面活性物质acomplexcomposedoflipidsandproteinsinfluidliningofalveolarsurfacesecretedbythecellsofthe

alveolistartingin20w,butlowbefore35w,toadultlevelafter3~7dofbirthhalf-life12~24h7SurfactantCompositionProtein(SP-A,B,C,D)11.1%Totallipid85-90%

Phosphatidylcholine磷脂80-90%(Lecithin磷脂酰胆碱/卵磷脂70-80%

)

Phosphatidylethanolamine

乙醇胺

5.2%Cholesterol胆固醇

6-8%Sphingomyelin鞘磷脂1.5%Lysophosphatidylcholine溶酶体胆碱0.9%Inmaturelung,Phospholipid/Sphingomyelin(L/S)≥28PS-function

reducethesurface

tension

oflungalveolikeeplungopenatendofexpirationpreventingthealveolifromcollapsingimmunomodulatoryeffects:巨噬细胞的吞噬、杀菌和趋化活性producednaturallyinthelungstokeepthelungalveoliopen.AlveoliwithoutPSAlveoliwithPS9FunctionofPSdecreasealveolarsurfacetension(

lungcompliance),andreducesrespiratoryworkMaintainalveoliinflationandfunctionalresidualcapacity(功能残气量）Acceleratelungfluidabsorption,reducealveolareffusionPathogenOpsonization,alveolarmacrophageactivation

increaseoxygenation—增加氧合improveventilation/perfusion—促进通气anti-inflammation—抗炎作用10alveolarsurfacetension肺泡腔液面压力液体薄层表面张力11PS是保障肺泡发挥换气功能的关键PS正常时PS含量不足

/功能异常时吸气时呼气时12

IncreasedRisk

DecreasedRisk

Prematurity

早产Maternaldiabetes

糖尿病母亲Cesareandelivery

withoutlaborPerinatalasphyxia

窒息Chorioamnionitis

绒毛膜羊膜炎

Multiplegestation

多胎Caucasian,malesex

白种人,男性FamilialpredispositonChronicintrauterinestressProlongedruptureofmembranesMaternalhypertensionIUGR/SGA宫内生长迟缓Antenatalmaternalsteroidsuse母亲产前使用激素ThyroidhormoneEtiology(2)13PreterminfantsoftenhaveimmaturelungswithinadequatePS14Pathogenesis

Pulmonaryimmaturityresultsinsurfactantdeficiency

Alveolicollapseattheendofexpirationleadstorespiratoryfailure

Surfactantdeficiencymayariseafterasphyxia,shockandacidosisalveolar

surfacetensionishigherDiminishedPSPulmonaryatelectasisImpairedgasexchange(hypoxiaandacidosis)PulmonaryarteryhypertensionRight–to-leftshuntingPulmonarycapillaryleakedproteinForminghyalinemembrane15嗜伊红透明膜肺透明膜病—发病机理肺泡表面张力PS缺乏肺泡不张缺氧、酸中毒肺动脉高压，PDA肺间质水肿纤维蛋白沉着于肺泡内表面气体弥散障碍

肺泡萎陷、肺顺应性

潮气量、通气量

肺泡通透性增加16

AlveolarSurfaceTension

AtelectasisLungcompliancedecreased.Functionalresidualcapacitydecreased.Lungvolumereduced.AlveolarventilationdecreasedAirwayresistanceremainsnormal肺顺应性气道阻力功能残气量17三.Pathology18

Gross––thelungcollapsed,firm,darkred,andliver-like.decreasedlungvolume19正常肺泡RDS:水肿，血管充血，毛细血管渗出，肺泡萎陷20RDS:

Atelectasis,pulmonaryedema,collapsedalveoli

fillwith

fibrin,cellulardebrisandhyalinemembrane21RDS:

hyalinemembranes

22

四.ClinicalManifestation23ClinicalPresentationRespiratorydistress

respiratoryfailureoccurinfirstfewhoursofageandgetsprogressivelyworse

--生后进行性呼吸困难

TachypneaRR>60bpm，Cyanosis(increasedneedtooxygen)chestretractions

--三凹征nasalflaring--鼻扇

expiratorygrunting--呻吟24

Featuresofrespiratoryfailure

respiratoryfailureoccuratorsoonafterbirth,notlongerthan12h;

thesymptomsusuallypeakonthethird

day

whendiuresisstarts,

thesymptomscanresolvequicklyby

PSsupplement.25Circulatoryinsufficiency

PDA/PPHN/ypotensionHypotensionCongestiveheartfailure（duetoleft-to-rightshuntingduringrecovery

）ShockIntracranialHemorrhageOthers:pulmonaryhemorrhage,

pulmonaryinfectionscomplicationsofassistedventilation—emphysema,pneumothorax,

ventilator-associatedpneumonia，BPD--Complications26RadiographicChanges1.bell-shapedthorax(lessvolumelung)2.thelungsarehypoaerated低透亮度bilateral,diffuse,homogeneousreticulogranularopacities

弥漫、均匀的网状颗粒影3.airbronchograms-peripherallyextending支气管充气征4.unclearnessofthecardiac/diaphragmaticsilhouette

心脏、横隔轮廓不清，orwhitelung白肺27Classicrespiratorydistresssyndrome(RDS).28ModerateNRDS.

Thereticulogranularpatternismoreprominent.Thelungsarehypoaerated.peripherallyextendingairbronchograms

arepresent.

29SevereRDS.Reticulogranularopacities,prominentairbronchograms,totalobscurationofthecardiacsilhouette.303132ComplicationofRDSarighttensionpneumothoraxwithherniationofrightupperlungacrossmidline.pneumomediastinum

33LaboratoryFindingsBloodgasanalysis:hypoxemia,hypercapnia,andrespiratoryacidosis

Phospholipid(PL)/Sphingomyelin(S)<2:1;orPhosphatidylglycerol(PG):negativeShaketestorBubblestest:

amnioticfluid(gastricaspirate),

negative

34BubblestestorShaketest:

95%alcohol1mlSample1mlShake15”Negativeamnioticfluid,gastricaspirate35五.DiagnosisDifferentialDiagnosis36DiagnosisDiagnosiscanbedecidedbyacombinationofassessments,including:

medicalhistory:GA,Diabeticmother,Asphyxia,male,Bubblestest(-)clinicalfeatures:

respiratorydistressoccuredinfirstfewhoursofageandgotprogressivelyworsechestX-ray:

confirmedbloodgases:37

Theinfantisalmostalwayspreterm.Onlyoccasionallyinterminfant.TerminfantwithHMDareusuallyborntowomenwithpoorlycontrolleddiabetes.

Bubblestestofamnioticfluidorgastricaspirateisnegative,indicatsinadequatesurfactant.Theinfantdevelopsrespiratorydistressatorsoonafterdelivery.Thesignsofrespiratordistressgraduallybecomeworseduringthefirst48hoursafterbirth.

ChestX-rayisabnormalandshowssmalllungswithreticulogranularopacities.AtypicalchestX-rayisneededtomakeadefinitediagnosisHMD.Diagnosis38DifferentialDiagnosis

PulmonarycausesofrespiratorydistressWetlungMeconiumaspirationPneumoniaExtra-pulmonarycausesofrespiratorydistressPneumothoraxHeartfailureDiaphragmaticherniaMetabolicacidosisAnaemiaPolycythaemia39

“Wetlung”orTransienttachypneaofthenewborn(TTN)---湿肺

Amnioticfluidormeconiumaspirationsyndrome

---羊水或胎粪吸入综合征

GroupBhemolyticStreptococcuspneumonia

---B组溶血性链球菌肺炎DifferentialDiagnosis40MeconiumAspiration

（MAS）

nodularnonhomogeneousdensities(bilateral,irregularcoarseinfiltrates),mayhavepleuraleffusions,usuallyincreasedlungvolume.

(不均,不规则粗大高密度影,肺容积增大,肺气肿,肺不张等）指胎儿在宫内或娩出过程吸入被胎粪污染的羊水，发生气道阻塞、肺内炎症和一系列全身症状。特点：生后出现呼吸困难，但不呈进行性发展。可合并“气漏综合征”、PPHN、ARDS、肺部感染。多见于足月儿、过期产儿，有窒息史或羊水粪染史，胸片可有不规则斑片状阴影，肺气肿明显。41MeconiumAspiration

不均匀密度增高影，肺气肿，42diffuse,coarsenodularopacities;focalemphysema.Lungsareusuallyhyperaerated.MeconiumAspiration

43FluidinthefissureWetlung

Hyperaeration；BilateralreticulogranulardensitiesarefleetinganddisappearwithventilationTransienttachypneaofthenewborn(TTN)

多见于足月儿，剖宫产。呼吸困难逐渐减轻、消失，病程较短，呈自限性，预后良好；x线：肺门纹理粗和斑点状影，常见水平线。44Wetlung

bilateral

(nonhomogeneous)densities;interstitialedemaandpleuraleffusions;hyperaeration

densitiesarefleeting

(samepatienton1daylater)45TransientTachypneaofNewbornat6hoursTheradiographontheleftshowshyperaeration,streakybilateralreticulonodularopacifications,prominentperihilarinterstitialmarkings,andmildcardiomegaly.Twodayslaterthereisnocardiomegalyandthepulmonaryparenchymalabnormalitieshavediminished.Thereisstillsomeperihilarstreakyopacities.at48hoursImagesfromEmedicine46

Group-BhemolyticStreptococcuspneumonia:Usuallyassociatedwithprematureruptureofmembranes.oftencoexistwithRDS,themimicappearance(clinicalandXfeatures)ofRDS（Hence,usallygivingantibioticstoallneonatesinthisconditionuntilbloodculturesarenegative.)47GBSNeonatalPneumoniaImagefromVirtualChildren’sHospital48阵发性呼吸急促及发绀腹部凹陷，患侧胸部呼吸音减弱甚至消失，可闻肠鸣音

X片见患侧胸部有充气的肠曲或胃泡影、肺不张，纵膈移位Diaphragmatichernia---膈疝49六.Treatmentsupplementaloxygen—correcthypoxia

continuouspositiveairwaypressure(CPAP)

mechanicalventilation

---CPAP

Indication:whenFiO2>0.6,PaO2<50mmHgorTcSO2<85%

Pressure:4~10cmH2O,flow5L/min,32°C,humidity100%

---CMV