Congenital Hypothyroid sm 先天性甲状腺功能减低症 Xue Fan gu. Md. Phd Xinhua Hospital Shanghai jiao Tong University School of medicine
Congenital Hypothyroidism 先天性甲状腺功能减低症 Xue Fan Gu, MD, PhD Xinhua Hospital Shanghai Jiao Tong University School of Medicine
Incidence Thyroid hormone deficiency may: or acqulre d Congenital: most cases are hypoplasia or aplasia of the thyroid gland World:1:3000~5000 China: 1: 3 200
Incidence • Thyroid hormone deficiency may: or acquired • Congenital:most cases are hypoplasia or aplasia of the thyroid gland World: 1:3 000～5 000 China: 1:3 200
Thyroid Ontogenesis 0 8th gestational weeks: synthesis of thyroglobulin 0 10 12th gestational weeks: pitutary gland begins to secrete TSh, thyroid gland synthesis of T3, T4 30th gestational weeks: hypothalamic pitutary-thyroid axis is functioning and independent of the maternal axis
⚫ 8th gestational weeks: synthesis of thyroglobulin ⚫ 10~12th gestational weeks: pitutary gland begins to secrete TSH,thyroid gland synthesis of T3、T4 ⚫ 30th gestational weeks: hypothalamicpitutary-thyroid axis is functioning and independent of the maternal axis Thyroid Ontogenesis
After delivery .14 TSH rapidly 300 10 rise reaching 250 60~80uU/ml 10 levels, and then200、 8 slowly decline 150 over the next 6 few days(5-7d) 100 to <suU/ml 50 T3 2 levels 30 Birth 2 4 6 GESTATIONAL POSTNATAL AGE (wks) AGE(wks)
• After delivery, TSH rapidly rise reaching 60~80 uU/ml levels, and then slowly decline over the next few days(5~7d) to <5 uU/ml levels
Thyroid hormone synthesis and metabolism The thyroid follicle is stimulated by tsh by increase with TSH receptor Iodine from the circulation is concentrated and rapidly oxidized by peroxidase to iodine Iodine incorporated into tyrosyl residures on thyroglobulin Iodothyrosines are couple an ether linkage to form T4 and T3
Thyroid hormone synthesis and metabolism • The thyroid follicle is stimulated by TSH by increase with TSH receptor • Iodine from the circulation is concentrated and rapidly oxidized by peroxidase to iodine • Iodine incorporated into tyrosyl residures on thyroglobuline • Iodothyrosines are couple an ether linkage to form T4 and T3