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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
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