小儿疾病ppt课件.ppt

PrecociousPuberty 1 PrecociousPuberty DefinitionOnsetofsecondarysexualcharacteristicsbefore8yingirlsand9yinboysGlobalIncidence0 6 1 boys girls 1 51998Shanghai30 000 1 2 ClassificationandConditionsCausingPrecociousPuberty P P 3 Gonadotrophin dependentP P TrueP P IdiopathicP P EncephalicTumorCongenitalHypothyroidism 4 TrueP P GnRH dependentHypothalamus Pituitary Gonad Axis Prematureactivation hyperfunctionReproductivecapacity 5 IdiopathicP P TrueP P 80 TheNeuroendocrineFunctionalFactorsNutritionalFactorsEnvironmentalFactorsdetergent alkylatedphenol 烷基化苯酚类 agrochemicals organochlorine 有机氯残留 plasticmaterial plasticizer santicizer218a bisphenolA 邻苯二甲酸酯类及双酚A Socialfactor 6 Girls 80 boys 40 somefamilialOnset4 8ys InfancyNormalpubertalsequenceGirls breast pubicandaxillaryhair menacheBoys testicular penile pubicandaxillaryhair spermatorrheaEpiphysealclosureearlyMatureintoshortadults 7 IntracranialTumor Man WomenEncephalicspaceoccupyinglesionSymptoms polydipsia polyuriahyperphagiaobesenessDisordershamartoma astrocytoma ependymocytoma pinealoma错构瘤 星形细胞瘤 室管膜瘤 松果体瘤 8 Gonadotrophin independentP P precocioupseudopuberty OvarianTumorCongenitalAdrenalHyperplasiaSecondaryAdrenalHyperplasiaGnRHinducedTumorExogenoussex steroidsMcCune AlbrightSyndrome 9 PrecociouPseudopuberty GnRH independentEndogenous ExogenousHypothalamus pituitary axis Noreproductivecapacity 10 CongenitalHypothyroidismProlonged Untreated T4 Feedback HypothalamusTRH PituitaryPRL TSH FSH LH 11 Congenitaladrenalhyperplasia SimpleVirilizing11 hydroxylase 11 羟化酶缺陷 Boys isosexualprecociouspubertyGirls heterosexualprecociouspubertypseudohermaphrodism假两性畸形 12 CongenitalHypothyroidism Prolonged UntreatedGirls breastdevelopmentBoys testisenlargementOthersymptomscoarsedfacies lethargy hypotonia constipation anemia mentalretardation dullresponseGrowthandMaturationofBonelaggardbehind 13 McCune AlbrightSyndrome SomaticcellmutationsNonegermlineGeneticbutnotheritableMutationsstimulatoryG proteinsubunitadenylcyclasesystemaffectedcellsmembranereceptor 14 Intakeofexogenoussexsteroids SymptomsofprecociouspseudopubertyGirlsbreastenlargermentareola乳晕nympha小阴唇pigmentation色素沉着vaginadischargeirregularvaginalbleedingBoysbreastenlargermentareolapigmentationtestis penisnoenlargerment 15 IncompletePrecociousPuberty partialP P PrematureThelarche单纯性乳房早发育PrematureAdrenarche单纯性阴毛早现PrematureIsolatedmenarche孤立性早潮 16 PrematureThelarche Transientcondition 2yNoneProgressiveIsolatedbreastdevelopmentorunilateralBenign firstsigntrueP Porprecociouspseudopuberty Exogenousexposuretoestrogen 17 PrematureAdrenarche pubicandaxillaryhair nonclassic21 hydroxylasedeficiencyBoneage growthvelocitySomegirlsmildinsulinresistancepolycysticovarysyndromenotabenigncondition 18 LaboratoryTest BoneageGP Greulich Pyle TW2 TW3PelvicUltrasonographySexSteroidsGnRHStimulationTestMRIRadiographytubularbone 19 DiagnosisandDifferentialDiagnosis InitialassessmentClinicalhistoryandphysicalexaminationPubertalmilestoneandgrowthchartRadiographofleftwristforboneageUnremarkableNormalVariationAbnormalDiagnosisPossiblediagnosesDifferentialdiagnosisActionsActionsActionsTreatmentTreatment 20 TreatmentofP P MedicationTraditionalChineseMedicine 21 Treatment 1 GnRHanalog 促性腺激素释放激素拟似剂 2 Cyproteroneacetate 环丙孕酮 色普龙 3 Danazol 达那唑 22 DifferentkindsofGnRHanalogs Brandstructurerelativeefficiencydosage 天然GnRH 1 g kg LueprolinD Leu61520 50H亮丙瑞林140 300MBuserelinD Ser6201200 1800喷鼻布舍瑞林20 40HTriptorelinD Trp63620 40H曲普瑞林50 100MDesl