医学精品课件:12.先甲低.ppt
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- 医学 精品 课件 12. 先甲低
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1、(Cretinism)Prof Feng XiongPediatric Endocrinology DepartmentChildrens Hospital of CMUCongenital HypothyroidismLearning objectivesYou should know:The clinical features of congenital hypothyroidism in neonates and childrenBe able to discuss the diagnosis and managementMajor contentsOverview of congeni
2、tal hypothroidismEtiologyClinical manifestationLaboratory examination Diagnosis and differential diagnosisTreatment Abbreviation CH congenital hypothyroidismTRH thyrotropin-releasing hormoneTSH thyrotropic-stimulating hormoneT4 thyroxineT3 triiodothyronineMIT monoiodotyrosine(单碘酪氨酸)单碘酪氨酸)DIT diiodot
3、yrosine(双碘酪氨酸)(双碘酪氨酸)TG thyroglobulinTBG thyroxine-binding globulinTH thyroid hormone OverviewHypothyroidism Congenital hypothyroidism acquired hypothyroidism Iodine deficiency Autoimmune disease Hashimoto thyroiditisOverviewHypothyroidism Congenital hypothyroidism acquired hypothyroidism Iodine def
4、iciency Autoimmune disease Hashimoto thyroiditisOverviewCongenital Hypothyroidism(CH)is common disease of pediatric endocrinology.Congenital factors cause the insufficiency of thyroid hormone,which result in the low metabolism,growth retardation,and mental impairment.The onset during inborn or newbo
5、rn period,will lead to irreversible mental impairment.CH is one of preventable causes of mental retardation,early treatment will improve the prognosis thoroughly.Incidence 1 in 4000 live births(in America and Europe)Females:Male is 2:1In China:Incidence of Congenital hypothyroidism(From the neonatal
6、 screening):1 in 3624 -the nationwide neonatal screening from clinical examination center of Health Bureau in 1999 ClassificationSporadical CHMajority CH patients are sporadical,a few is familial.Endemic CHPregnant women live in iodine insufficient areas,which result in iodine deficiency.Along with
7、fortifying iodine salt in dietary,incidence of endemic CH is much lower.Embryology of ThyroidEmbryology of Thyroid Thyroid is the first endocrine gland to develop in embryo Begins to form in 24 days after fertilization Forms from primordial pharyngeal floor(原始咽底部)forming thyroid diverticulum(原基)As e
8、mbryo and tongue grow,thyroid descends in anterior neck and reaches its normal site:the opposite cervical vertebra 5-7 by 7 weeks of gestationPharynxPharynxfloorfloorRole of Placenta SubstancePlacental permeability I-+TRH +IgG Antibodies +T3 +T4 +TSH 0pituitary-thyroid hormone production after birth
9、 Hormones in fetal Hormones after birth(Hour)Within 30 minutes of delivery,TSH abrupt rise occurs-as a protected results of change in environmental temperature.TSH levels:first 12h as high as 70mU/L;after 48h:20;after 120h:95 percent neonates with CH have few clinical manifestations(maternal T4 cros
10、ses the placenta,so even in athyrosis thyroid hormone umbilical cord serum T4 level are about 25 to 50 percent of those of normal infants).Clinical findings:(happens in 5%of neonatal CH)Post-term birth(42 weeks);large for date infant Wt 4 kg Hypothermia,acrocyanosis手足发绀手足发绀,respiratory distress,leth
11、argy and hoarse cry.poor feeding,constipation.Jaundice often prolongs over 2 weeks.Oedema,large posterior fontanel,macroglossia,abdominal distension,umbilical hernia脐疝脐疝,mottled skin.bone age is often delayed at birth.Neonate with Congenital HypothyroidismJaundice,macroglossia,dry skin,and hoarse cr
12、y.Laboratory examination An elevated TSH is most sensitive and specific test to confirm diagnosis Remember that TSH surge occurs in first 12 hours of life and may remain high until 48 hoursLaboratory examination Newborn screening Suitable Time:23 days after birthMothed:use dry blood slip and detect
13、TSH level.Results:positive:TSH 20uU/ml then detect T4 and TSH for further determineLaboratory examinationEvaluate thyroid function primarily hypothyriodism:T4 T3 TSH secondary hypothyriodism:T4 T3 TSHTRH stimulating test objective:suspected insufficient TSH or TRH objectives mothed:use TRH 7ug/kg,IV
14、,detect TSH level results:normal:at 30 min appear TSH peak,at 90 min retum to basic value.abnormal:No TSH peak appear-pituitary disorder Delayed TSH peak appear-hypothalamic disorderLaboratory examinationRadiographinfant and child CHEvaluation bone ageObviously delayed bone age,almost lags 3 years o
15、ldPosition of detect:younger than 6 month:take knee X-rayolder than 6month:take wrist X-ray Neonatal distal femoral epiphysis ossification center Normal neonate Neonate with CHWrist ossification center Normal children CH childrenNormal children CH children Diagnosis as early as possiblediagnostic cr
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