先天性甲状腺功能减低症 课件讲座

1、2021/8/121烦恼有何惧怕，既然躲不掉，就调好心态与它共存。心向阳光，何惧风霜。茫茫人海你我相遇就是缘分，欢迎下载！MIT DIT DIT MITMIT T4 T3 DIT甲状腺激素的生物学作用（一）对代谢的影响 1、产热效应 2、对蛋白质、糖和脂肪的影响（二）对生长发育的影响（三）对神经系统的影响DEFINITIONA congenital disorder of thyroid metabolism characterized by a lower than normal end organ effect of thyroid hormone resulting in the sig

2、ns and symptoms of hypothyroidism. Etiology1、甲状腺不发育或发育不全、甲状腺不发育或发育不全Thyroid dysgenesis Etiology2、新生儿暂时性甲状腺功能减低症TSH-Binding Inhibitory Antibody (TBIAb)Etiology3、 甲状腺素合成途径缺陷Defective synthesis of thyroxine(thyroid hormone)Etiology4、促甲状腺激素缺乏Thyrotropin deficiency (Thyroid-stimulating hormone, TSH)2021/

3、8/12129、 人的价值，在招收诱惑的一瞬间被决定。2022-3-172022-3-17Thursday, March 17, 202210、低头要有勇气，抬头要有低气。2022-3-172022-3-172022-3-173/17/2022 10:47:07 PM11、人总是珍惜为得到。2022-3-172022-3-172022-3-17Mar-2217-Mar-2212、人乱于心，不宽余请。2022-3-172022-3-172022-3-17Thursday, March 17, 202213、生气是拿别人做错的事来惩罚自己。2022-3-172022-3-172022-3-1720

4、22-3-173/17/202214、抱最大的希望，作最大的努力。2022年3月17日星期四2022-3-172022-3-172022-3-1715、一个人炫耀什么，说明他内心缺少什么。2022年3月2022-3-172022-3-172022-3-173/17/202216、业余生活要有意义，不要越轨。2022-3-172022-3-17March 17, 202217、一个人即使已登上顶峰，也仍要自强不息。2022-3-172022-3-172022-3-172022-3-17Etiology5、甲状腺或靶器官反应性低下Thyroid hormone unresponsivenessEt

5、iology6、碘缺乏Defect of iodide CLINICAL FEATURESCLINICAL FEATURES Neurological manifestationsinfantile hypotonia lack of crying (good baby) psychomotor delay sluggish/disinterested somnolence Respiratory ManifestationsRespiratory Manifestationsapnea choking spells with feeds nasal obstruction noisy res

6、pirations respiratory distress syndrome Cardiovascular ManifestationsCardiovascular Manifestationscardiomegaly murmers slow heart rate Gastrointestinal ManifestationsGastrointestinal Manifestationsconstipation delayed dentation feeding difficulties high birth weight large abdomen macroglossia poor a

7、ppetite prolonged jaundice unbilical hernia Cutaneous ManifestationsCutaneous Manifestationscoarse, brittle, scanty hair cold and mottled skin deep, hoarse voice dry, scaly skin edema of genitals & extremities low hairline myxedema Musculoskeletal ManifestationsMusculoskeletal Manifestationsnorm

8、al body weight but decreased length normal or increased head size broad, short fingers pseudohypertrophy (calf) short limbs short thick neck Endocrine ManifestationsEndocrine Manifestationsgoiter (with error of thyroid metabolismINVESTIGATIONSINVESTIGATIONSScreeningScreeningall neonates are screen a

9、t 48 hours for TSH (and/or T4) from a blood drop placed on filter paper if result is 0.20 mIU/L see patient immediately for history/physical, to draw a serum T4 and TSH, and to start thyroid hormone therapy (see below) SerumSerumelevated TSH and reduced T4 anemia unconjugated hyperbilirubinemia Radi

10、onuclide ScintigraphyRadionuclide Scintigraphyradioactive technetium to detect developmental defects of the thyroid (i.e., dysgenesis, ectopia, etc.) Skeletal X-RaysSkeletal X-Raysfor baseline bone age retardation of osseous development Differential diagnosis佝偻病21-三体症先天性巨结肠骨骼发育障碍的疾病MANAGEMENTMANAGEM

11、ENTThyroid Replacement start thyroid hormone at 6 ug/kg po qd Maintenance see initially at 3 and 6 months of age then q6m moniter TSH, T4 levels, growth parameters, clinically .PrognosisPrognosisnormal growth, intelligence, and life span if detected within the first few weeks of life short stature,

12、mental retardation, and delayed puberty with delays in detection, progressive ataxia 地方性呆小病的主要病因是A 母亲孕期饮食中缺碘B 垂体分泌TSH减少C 甲状腺发育异常D 甲状腺激素合成障碍E 母亲在妊娠期应用抗甲状腺药物甲状腺组织的散发性 呆小病出现症状的最早年龄通常是？A 13个月B 46个月C 78个月D 910个月E 1112个月9、 人的价值，在招收诱惑的一瞬间被决定。22.3.1722.3.17Thursday, March 17, 202210、低头要有勇气，抬头要有低气。*3/17/2022

13、 10:47:07 PM11、人总是珍惜为得到。22.3.17*Mar-2217-Mar-2212、人乱于心，不宽余请。*Thursday, March 17, 202213、生气是拿别人做错的事来惩罚自己。22.3.1722.3.17*March 17, 202214、抱最大的希望，作最大的努力。2022年3月17日星期四*22.3.1715、一个人炫耀什么，说明他内心缺少什么。2022年3月*22.3.17*March 17, 202216、业余生活要有意义，不要越轨。*3/17/202217、一个人即使已登上顶峰，也仍要自强不息。*22.3.17谢谢大家谢谢大家2021/8/12399、

14、 人的价值，在招收诱惑的一瞬间被决定。2022-3-172022-3-17Thursday, March 17, 202210、低头要有勇气，抬头要有低气。2022-3-172022-3-172022-3-173/17/2022 10:47:07 PM11、人总是珍惜为得到。2022-3-172022-3-172022-3-17Mar-2217-Mar-2212、人乱于心，不宽余请。2022-3-172022-3-172022-3-17Thursday, March 17, 202213、生气是拿别人做错的事来惩罚自己。2022-3-172022-3-172022-3-172022-3-173/17/202214、抱最大的希望，作最大的