七年制医学课件 儿科 18先天性甲低.ppt

 （呆小病 克汀病） (Cretinism) Overview Congenital Hypothyroidism is common disease of pediatric endocrinology. Congenital factors cause the insufficience of thyriod hormone, which result in the low metabolism, retardation of growth,and mental impairment. The onset during inbore or newborn peroid,can lead to irreversible mental impairment. Disease incidence In American and Europe: Incidence of neonatal screening: 1/3750 In China: Incidence of neonatal screening: 1/3624 ------the nationwide neonatal screening from clinical examination center of Health Bureau in 1999 Category Sporadical congenital hypothyroidism accounting for most patients, a few as family. Endemic congenital hypothyroidism Pregnant women live in iodine insufficient areas, which result in iodine deficiency. along with the population of iodine salt in dietary, the incidence is much lower. Etiological factor Thyriod dysgenesis Athyreotic or hypothyreotic Ectopic Goitrous enzyme defect (thyriod hormone dysgenesis) Iodine deficiency or Iodine transporter defect peroxidase defct thyroglobulin synthetic defect Thyroid-stimulating hormone defect TSH defect or TSH resistance Peripheral thyriod hormone inactivation Transient hypothyriodism maternal antithyriod medications or maternal antithyriod antibodies The role on metabolism of human body The role on growth and development The role on function of organs and systems The role on organ and system Clinical manifestation typical symptom 典型症状 Special manifestation of neonatal hypothyroidism 新生儿甲低的特殊表现 Typical symptome(典型症状) Retardation of growth and development 生长发育障碍 unusual facies and body carriage 特殊面容体态 mental disable 智能低下 Physiologic Hypofunction 生理功能低下 Typical symptome(典型症状) Retardation of g