精品growth hormone deficiency 生长激素缺乏症课件.ppt

Growth Hormone Deficiency生长激素缺乏症 Xue Fan Gu, MD, PhD Xinhua Hospital Shanghai Jiao Tong University School of Medicine  Short stature Below the 3rd percentile or －2SD than that of his or her peers Short stature has many causes, the pathologic short stature should be suspected in children who have an abnormal growth velocity or who are significantly short for their family Growth Hormone Chemistry：191 animo acid，22-KDa protein Action： To growth of the epiphyseal regions of the long bones, also via induce local IGF-1 production in the epiphyseal plate at the both level of mRNA and protein. to promote the cell proliferation. A variety of metabolic effect, such as Anabolic action on protein metabolic, lipolysis, animo acid transport in diaphragm and heart. “Diabetogenic” action on large GH dose. The effects in normal aging and catabolic conditions are under investigation. Etiology of GH deficiency Genetic forms It can be transmitted as an autosomal recessive, autosomal dominat or X-linked recessive trait. Abnormalities of the genes: GHRH receptor, Pouifi, Prop1, GH1, GH receptor, IGF-1 etc. Acquired forms The GH axis is more susceptible to disruption by acquired conditions that are the other hypothalamic-pituitary axes. Craniopharyngioma, eosinophilic granuloma, tuberculosis, Trauma, radiotherapy for malignancies Clinical manifestations Short stature: slow growth in height, Congenital form: birth length is below the mean, 2-4Sd below the mean by 1 year of age. Acquired form: normal initially, growth faillure gradually appear and progress Delayed closure of the epiphyses. The head is round, the face is short and broad. The neck is short, the voice is high-pitched, the genitals are undeveloped and sexual maturation may be delayed or absent. The scalp hair is fine. Some with ACTH、TSH、GnRH and ADH deficiency. Laboratory findings (1) Definitive diagnosis: GH provocative tests: Insulin 0.05-0.1u/Kg Arginine 0.5g/kg Clonidine 4ug/kg L-dopa 10mg/k