PrecociousPuberty

PrecociousPuberty

Definition

Onsetofsecondarysexualcharacteristicsbefore8yingirlsand9yinboys

GlobalIncidence

0.6～1％boys∶girls＝1∶51998Shanghai30,000/1%ClassificationandConditionsCausing

PrecociousPuberty(P.P)TrueP.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*congenitalCNS

hyperplasia

孤立性早潮

anomalies*Secondaryadrenal*postinfectionorhyperplasia

tramua*adrenaltumor3.Hypothyroidism3.GnRHinducedTumor

4.Exogenoussexhormones

5.McCune-AlbrightSyndrome

Gonadotrophin-dependentP.P.

(TrueP.P.)

IdiopathicP.P.EncephalicTumorCongenitalHypothyroidism

TrueP.P.GnRH-dependentHypothalamus-Pituitary-Gonad-Axis

Prematureactivation&hyperfunctionReproductivecapacity

IdiopathicP.P.

TrueP.P----80%TheNeuroendocrineFunctionalFactorsNutritionalFactorsEnvironmentalFactors

detergent(alkylatedphenol)（烷基化苯酚類）

agrochemicals(organochlorine)（有機(jī)氯殘留）

plasticmaterial(plasticizer-santicizer218a,bisphenolA)(鄰苯二甲酸酯類及雙酚A）SocialfactorGirls-80%,boys-40%,somefamilialOnset4-8ys/InfancyNormalpubertalsequence

Girls:breast-pubicandaxillaryhair-menacheBoys:testicular-penile-pubicandaxillaryhair-spermatorrheaEpiphysealclosureearlyMatureintoshortadults

IntracranialTumor

Man>WomenEncephalicspaceoccupyinglesionSymptoms:polydipsia,polyuriahyperphagiaobesenessDisordershamartoma,

astrocytoma,ependymocytoma,pinealoma

錯構(gòu)瘤、星形細(xì)胞瘤、室管膜瘤、松果體瘤

Gonadotrophin-independentP.P.

(precocioupseudopuberty)OvarianTumorCongenitalAdrenalHyperplasiaSecondaryAdrenalHyperplasiaGnRHinducedTumorExogenoussex-steroidsMcCune-AlbrightSyndromePrecociouPseudopubertyGnRH-independentEndogenous/ExogenousHypothalamus-pituitary-axis

Noreproductivecapacity

CongenitalHypothyroidism

Prolonged/UntreatedT4

-Feedback

HypothalamusTRH

PituitaryPRL

TSH

FSH、LH

Congenitaladrenalhyperplasia

SimpleVirilizing11-hydroxylase（11-羥化酶缺陷）

Boys：

isosexualprecociouspubertyGirls:

heterosexualprecociouspubertypseudohermaphrodism假兩性畸形CongenitalHypothyroidism

Prolonged/UntreatedGirls:breastdevelopmentBoys:testisenlargementOthersymptomscoarsedfacies,lethargy,hypotonia,constipation,anemia,mentalretardation,dullresponseGrowthandMaturationofBonelaggardbehindMcCune-AlbrightSyndromeSomaticcellmutationsNonegermlineGeneticbutnotheritableMutationsstimulatoryG-proteinsubunitadenylcyclasesystemaffectedcellsmembranereceptor

Intakeofexogenoussexsteroids

SymptomsofprecociouspseudopubertyGirlsbreastenlargermentareola乳暈

nympha小陰唇

pigmentation色素沉著

vaginadischargeirregularvaginalbleedingBoysbreastenlargermentareolapigmentationtestis/penisnoenlargerment

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PrematureThelarche

Transientcondition<2yNoneProgressiveIsolatedbreastdevelopmentorunilateralBenign–?firstsigntrueP.Porprecociouspseudopuberty?Exogenousexposuretoestrogen

PrematureAdrenarche

pubicandaxillaryhair

？nonclassic21-hydroxylasedeficiencyBoneage/growthvelocitySomegirlsmildinsulinresistancepolycysticovarysyndromenotabenignconditionLaboratoryTest

BoneageGP（Greulich-Pyle)/TW2/TW3PelvicUltrasonography

SexSteroidsGnRHStimulationTestMRIRadiographytubularbone

DiagnosisandDifferentialDiagnosis

InitialassessmentClinicalhistoryandphysicalexaminationPubertalmilestoneandgrowthchartRadiographofleftwristforboneageUnremarkableNormalVariationAbnormalDiagnosisPossiblediagnosesDifferentialdiagnosisActionsActionsActionsTreatmentTreatment

TreatmentofP.P.

MedicationTraditionalChineseMedicineTreatment1.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(dá)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