急性腎小球腎炎（agn）

1、Disorders of puberty,Department of PediatricsSoochow University Affiliated Children’s Hospital,Aim and claim,Understanding the normal development of childFamiliar with the causes of precocious pubertyGet hold of the

2、 clinical features and diagnosis and management of precocious,General concepts,Puberty is defined as the acquisition of reproductive capability.In clinical practice it is marked by the acquisition of secondary sexual c

3、haracteristicsBetween the age of 8-18 years children acquire secondary sexual characteristic and undergo the bodily changes which we loosely call pubertySome do earlier than othersSome go through the changes in about

4、2 years,while others may take three times as long,General concepts,it is important to recognize the apparently Beneficial changes which take place at puberty such as rapid increase in size, strength and endurance; the

5、development of gonads from their infantile to mature state. All these changes and there interactions constitute puberty.,Normal puberty,Secondary sex characteristics in girls: Breast development at the age ab

6、ort 8-13 years, the vagina（陰道） begins to increase in length, the uterus （子宮）and ovarian（卵巢） follicles（卵泡） increase in size, the pubic hair（陰毛） appears,menarche（月經(jīng)） occurs. Secondary sex characteristics in boys: the

7、 external genitalia begin to enlarge at the age about 9-14 years from infantile to mature, represented by enlargement of the testes（睪丸） and scrotum（陰囊） and penis（陰莖）,pubic hair appears, voice change,sperm produce.,Featur

8、es of puberty,Femalesbreast development is first sign,height spurt reaches maximum before menarche,menarche occurs between 11 and 15 yearsMalestesticular growth is first sign,maximum height spurt reached 2 years

9、 after females,The stages of female and male sexual development(1-5）,乳房發(fā)育的分期Stage 2 - Breast bud with areola wideningStage 3 - Enlargement of breast and widening of areola. No separation of contoursStage 4 - Second

10、ary mound develops with separationStage 5 - Mature breast,睪丸測量裝置,Between early childhood and approximately 8-9 yr of age (prepubertal stage), the hypothalamic-pituitary-gonadal axis（下丘腦-垂體-性腺軸） is dormant, as reflected

11、 by undetectable serum concentrations of luteinizing hormone (LH) and sex hormones (estradiol in girls, testosterone in boys). In this phase, the activity of the hypothalamus and pituitary may be suppressed by poorly cha

12、racterized neuronal restraint pathways.,The feature of puberty,At about same time as secondary sex characteristics are developing, children experience a dramatic acceleration of their growth—growth spurt.Change in body

13、 proportions(legs trunk and sitting height)The skull forward growth, skeletal maturetion, lymphoid tissues developmentGnRH (from hypothalamus),FSH,LH (from pituitary) have sure pulsatile secretionSex steroids modulat

14、e the adolescent growth spurt and ultimately bring about cessation of growth,Disorder of puberty,During normal puberty,secondary sex characteristics are acquired and reproductive capacity is attained.Puberty may be

15、 precocious or delayed.,Disorder of puberty,Definition of precocious puberty:Precocious puberty refers to the development of secondary sexual characteristics before the age of 8 year in girls or 9 year in boys.,Cau

16、se of precocious puberty,Gonadotrophin-dependent/ True precocious puberty （促性腺激素依賴性） idiopathic, familial, CNS lesions, e.g. postirradiation, surgery, tumours,hydrocephalus.Gonadotrophin-independent / precocious

17、 pseudopuberty （促性腺激素非依賴性） McCune-Albright syndrome , (polyostotic fibrous dysplasia of bone, café-au-lait spots ,Excessive oestrogen in girls or excess testorsterone in boys isseen together with no LH or FS

18、H response to LHRH) tumours of adrenals or gonads , CAH.,True precocious puberty is always isosexual and stems from hypothalamic-pituitary-gonadal activation. The gonadotropin-mediated increase in the size and activ

19、ity of the gonads leads to increasing sex hormone secretion and progressive sexual maturation. In precocious pseudopuberty, some of the secondary sex characteristics appear, but there is no activation of the normal hypot

20、halamic pituitary-gonadal interplay. In this latter group, the sex characteristics may be isosexual（同性） or heterosexual（異性） (“contrasexual”),Conditions causing precocious puberty:GONADOTROPIN-DEPENDENT PUBERTY (TRUE PRE

21、COCIOUS PUBERTY)IdiopathicOrganic brain lesionsHyoothalamic hamartomaBrain tumors, hydrothephalus, severe head trauma, myelomeningoceleHypothyroidism, prolonged and untreatedCOMBINED GONADOTROPIN-DEPENDENT AND GONA

22、DOTROPIN-INDEPENDENT PUBERTYTreated congenital adrenal hyperplasiaMcCune-Albright syndrome, lateFamilial male precocious puberty, late,GONADOTROPIN-INDEPENDENT PUBERTY(PRECOCIOUS PSEUDOPUBERTY)Femalesisosexuol (femi

23、nizing) conditionsMcCune-Albright syndromeAutonomous ovarian cysts0varian tumorsGranulosa-theca cell tumor associated with 0llier diseaseTeratoma, chorionepithelioma,,Sex-cord tumor with annular tubules (SCTAT) asso

24、ciated with Peutz-Jeghers syndromeFeminizing adrenocortiral tumorExogenous estrogensHeterosexuol (masculinizing) conditionscongenital adrenal hyperplasiaAdrenal tumorsOvarian tumorsGlucocorticoid receptor defectE

25、xogenous androgens,Malesisosexuol (norulinizing) conditionsCongenital adrenal hyperplasiaAdrenocortical tumorLeydig cell tumorFamilial male precocious pubertyisolatedAssoriated with pseudohypoparathyroidismHCG-se

26、creting tumorsCentral nervous system,,HepatoblastomaMedjastinal tumor associated with Klinefelter syndromeTeratomaGlucocorticoid receptor defectExogenous androgenHeterosexual (feminizing) conditionsFeminizing adre

27、nocortical tumorSCTAT associated with Peutz-leghers syndromeExogenous estrogens,INCOMPLETE (PARTIAI) PREOCIOUS PUBERTYPremature thelarche（單純?nèi)榉堪l(fā)育） Premature adrenarche（單純腎上腺初現(xiàn)）Premature menarche（單純月經(jīng)來潮）,Ce

28、ntral precocious puberty(CPP),Precocious puberty is termed ‘central’ when it is caused by the premature activation of the hypothalamo-pituitary- gonadal axis.It is also to describe this condition as idiopathic precocious

29、 puberty(also not all causes of central precocious puberty are idiopathic).,Clinical manifestation,Sexual development may begin at any age and generally follows the sequence observed in normal puberty. In girls, the firs

30、t sign is development of the breast; pubic hair may appear simultaneously but more often appears later .Maturation of the external genitalia, the appearance of axillary hair, and the onset of menstruation follow. The ear

31、ly menstrual cycles may be more irregular than they are with normal puberty. The initial cycles are usually anovulatory, but pregnancy has been reported as early as 5.5yr of age.,Clinical manifestation,In boys, enlargeme

32、nt of the testes is followed by enlargement of the penis, apperance of pubic hair,and acne. Erections are common, and nocturnal emissions may occur. The voice deepens, and linear growth is accelerated, testicular biopsie

33、s have shown stimulation of all elements of the testes, and spermatogenesis has been observed as early as 5-6yr of age. In affected girls and boys, height, weight, and osseous maturatron are advanced. The increased rate

34、of bone maturation results in early closure of the epiphyses, and the ultimate stature is less than it would have been otherwise.,without treatment, approximately 1/3 of girls and an even larger percentage of boys achiev

35、e a height less than the 5th percentile as adults. Mental development is usually compatible with chronological age. Emotional behavior and mood swings are common, but serious psychologic problems are rare.,男孩，8歲外生殖器發(fā)育

36、陰毛發(fā)育身高躥長——中樞性性早熟,,CPP,Children with suspected CPP should be investigated in order to answer two question: To confirm the dependence of the pubertal maturation on hypothalamo-pituitary function. To exclude an i

37、ntracranial tumour.,CPP,An assessment of physiological Gn secretion is the most helpful investigation.Patients have a nomal pubertal LH and FSH response after an LHRH test, The bone age is usually advanced.Pelvic ultra

38、sound assessment is useful in girls. CT or MRI scanning of the brain is advisable.,Disorder of puberty,Precocious puberty is more common in girls.It is usually due to early onset of normal puberty.(80%)In boys, It is

39、usually due to an intracranial tumour(40%),Disorder of puberty,Precocious puberty should be differentiated from:Premature thelarche: isolated breast development in a very young girl.a non-progressive,benign condition.P

40、remature adrenarche: isolated early appearance of pubic hair in either sex. a benign self-limiting condition due to early maturation of adrenal androgen secretion, but an adrenal tumour may need to be excluded,CPP,The

41、problems associated with Icpp are in three areas:Premature sexual development, especially menarche at primary school.Psychological disturbance, especially masturbation in boysImpaired growth prognosis,Treatment of ch

42、ildren with cpp,Treatment depends on cause. It may be necessary to attempt to reduce the rate of skeletal maturation to avoid early cessation of growth and a reduction in adult height.GnRH analogues are useful in treatm

43、ent with cpp and have no known side effects.GnRHa are effective at suppressing the secondary sexual characteristics of puberty and the first few years of use are to improve height prognosis,參考書目,《Paediatrics》 (mosby’s

44、crash course) 科學(xué)出版社《Paediatrics》北京大學(xué)醫(yī)學(xué)部出版社《 Nelson Paediatrics》,思考題,Which type of precocious puberty can not be treated?Answer : Premature thelarche ,Premature adrenarche and Premature menarche can not be treat