急性肾小球肾炎（AGN）

Disorders

ofpubertyDepartment

of

Pediatrics

SoochowUniversity

Affiliated

Children’sHospitalAim

and

claimUnderstanding

the

normal

development

ofchildFamiliar

with

the

causes

of

precociouspubertyGet

hold

of

the

clinical

features

and

diagnosisand

management

of

precociousGeneral

conceptsPuberty

is

defined

as

the

acquisition

of

reproductivecapability.In

clinical

practice

it

is

marked

by

the

acquisition

ofsecondary

sexual

characteristicsBetween

the

age

of

8-18

years

children

acquiresecondary

sexual

characteristic

and

undergo

thebodily

changes

which

we

loosely

call

pubertySome

do

earlier

than

othersSome

go

through

the

changes

in

about

2

years,whileothers

may

take

three

times

as

longGeneral

concepts

it

is

important

to

recognize

the

apparentlyBeneficial

changes

which

take

place

at

pubertysuch

as

rapid

increase

in

size,

strength

andendurance;

the

development

of

gonads

from

theirinfantile

to

mature

state.All

these

changes

and

there

interactionsconstitute

puberty.Normal

pubertySecondary

sex

characteristics

in

girls:Breast

development

at

the

age

abort

8-13

years,thevagina（阴道）

begins

to

increase

in

length,the

uterus（子宫）and

ovarian（卵巢）

follicles（卵泡）increase

in

size,the

pubic

hair（阴毛）appears,menarche（月经）

occurs.Secondary

sex

characteristics

in

boys:the

external

genitalia

begin

to

enlarge

at

the

age

about9-14

years

from

infantile

to

mature,represented

byenlargement

of

the

testes（睾丸）

and

scrotum（阴囊）and

penis（阴茎）,pubic

hair

appears,voicechange,sperm

produce.Features

ofpubertyFemalesbreast

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

afterfemalesThe

stagesof

femaleand

malesexualdevelopment(1-5）乳房发育的分期□Stage

2

-

Breast

bud

withareola

widening□Stage

3

-

Enlargement

ofbreast

and

widening

of

areola.

Noseparation

of

contours□Stage

4

-

Secondary

mounddevelops

with

separation□Stage

5

-

Mature

breast睾丸测量装置Between

early

childhood

and

approximately

8-9

yrof

age

(prepubertal

stage),the

hypothalamic-pituitary-gonadal

axis（下丘脑-垂体-性腺轴）

is

dormant,asreflected

by

undetectable

serum

concentrations

ofluteinizing

hormone

(LH)and

sex

hormones

(estradiolin

girls,

testosterone

in

boys).

In

this

phase,

the

activitof

the

hypothalamus

and

pituitary

may

be

suppressedby

poorly

characterized

neuronal

restraint

pathways.The

feature

of

pubertyAt

about

same

time

as

secondary

sex

characteristicsare

developing,

children

experience

a

dramaticacceleration

of

their

growth—growth

spurt.Change

in

body

proportions(legs

trunk

and

sittingheight)The

skull

forward

growth,

skeletal

maturetion,

lymphoidtissues

developmentGnRH

(from

hypothalamus),FSH,LH

(from

pituitary)have

sure

pulsatile

secretionSex

steroids

modulate

the

adolescent

growth

spurt

andultimately

bring

about

cessation

of

growthDisorder

of

pubertyDuring

normal

puberty,secondary

sexcharacteristics

are

acquired

andreproductive

capacity

is

attained.Puberty

may

be

precocious

or

delayed.Disorder

of

pubertyDefinition

of

precocious

puberty:Precocious

puberty

refers

to

the

developmentof

secondary

sexual

characteristics

beforethe

age

of

8

year

in

girls

or

9

year

in

boys.Cause

of

precocious

pubertyGonadotrophin-dependent/

True

precocious

puberty（促性腺激素依赖性）idiopathic,

familial,CNS

lesions,

e.g.

postirradiation,

surgery,tumours,hydrocephalus.

Gonadotrophin-independent/precociouspseudopuberty

（促性腺激素非依赖性）McCune-Albright

syndrome,(polyostotic

fibrous

dysplasia

of

bone,

café-au-lait

spots

,Excessive

oestrogen

in

girls

or

excess

testorsteronein

boys

isseen

together

with

no

LH

or

FSH

response

to

LHRH)tumours

of

adrenals

or

gonads

,

CAH.□True

precocious

puberty

is

always

isosexual

andstems

from

hypothalamic-pituitary-gonadal

activation.The

gonadotropin-mediated

increase

in

the

size

andactivity

of

the

gonads

leads

to

increasing

sex

hormonesecretion

and

progressive

sexual

maturation.

Inprecocious

pseudopuberty,

some

of

the

secondary

sexcharacteristics

appear,

but

there

is

no

activation

of

thenormal

hypothalamic

pituitary-gonadal

interplay.

In

thislatter

group,

the

sex

characteristics

may

be

isosexual（同性）

or

heterosexual（异性）(“contrasexual”)Conditions

causing

precocious

puberty:GONADOTROPIN-DEPENDENT

PUBERTY

(TRUEPRECOCIOUS

PUBERTY)□Idiopathic□Organic

brain

lesions□Hyoothalamic

hamartoma□Brain

tumors,

hydrothephalus,

severe

head

trauma,myelomeningocele□Hypothyroidism,

prolonged

and

untreated□COMBINED

GONADOTROPIN-DEPENDENT

ANDGONADOTROPIN-INDEPENDENT

PUBERTY□Treated

congenital

adrenal

hyperplasia□McCune-Albright

syndrome,

late□Familial

male

precocious

puberty,

lateGONADOTROPIN-INDEPENDENTPUBERTY(PRECOCIOUS

PSEUDOPUBERTY)□Females□isosexuol

(feminizing)

conditions□McCune-Albright

syndrome□Autonomous

ovarian

cysts□0varian

tumors□Granulosa-theca

cell

tumor

associated

with

0llier

disease□Teratoma,

chorionepitheliomaSex-cord

tumor

with

annular

tubules

(SCTAT)associated

with

Peutz-Jeghers

syndromeFeminizing

adrenocortiral

tumorExogenous

estrogensHeterosexuol

(masculinizing)

conditionscongenital

adrenal

hyperplasiaAdrenal

tumorsOvarian

tumorsGlucocorticoid

receptor

defectExogenous

androgensMales□isosexuol

(norulinizing)

conditions□Congenital

adrenal

hyperplasia□Adrenocortical

tumor□Leydig

cell

tumor□Familial

male

precocious

puberty□isolated□Assoriated

with

pseudohypoparathyroidism□HCG-secreting

tumors□Central

nervous

systemHepatoblastomaMedjastinal

tumor

associated

with

KlinefeltersyndromeTeratomaGlucocorticoid

receptor

defectExogenous

androgenHeterosexual

(feminizing)

conditionsFeminizing

adrenocortical

tumorSCTAT

associated

with

Peutz-leghers

syndromeExogenous

estrogens□INCOMPLETE

(PARTIAI)

PREOCIOUS

PUBERTYPremature

thelarche（单纯乳房发育）Premature

adrenarche（单纯肾上腺初现）Premature

menarche（单纯月经来潮）Central

precocious

puberty(CPP)²

Precocious

puberty

is

termed

‘central’when

it

is

caused

by

the

premature

activationof

the

hypothalamo-pituitary-

gonadal

axis.Itis

also

to

describe

this

condition

as

idiopathicprecocious

puberty(also

not

all

causes

ofcentral

precocious

puberty

are

idiopathic).Clinical

manifestationSexual

development

may

begin

at

any

age

andgenerally

follows

the

sequence

observed

in

normalpuberty.

In

girls,

the

first

sign

is

development

of

thebreast;

pubic

hair

may

appear

simultaneously

butmore

often

appears

later

.Maturation

of

the

externalgenitalia,

the

appearance

of

axillary

hair,

and

theonset

of

menstruation

follow.

The

early

menstrualcycles

may

be

more

irregular

than

they

are

withnormal

puberty.

The

initial

cycles

are

usuallyanovulatory,

but

pregnancy

has

been

reported

as

earlyas

5.5yr

of

age.Clinical

manifestationIn

boys,

enlargement

of

the

testes

is

followed

byenlargement

of

the

penis,

apperance

of

pubic

hair,andacne.

Erections

are

common,

and

nocturnal

emissionsmay

occur.

The

voice

deepens,

and

linear

growth

isaccelerated,

testicular

biopsies

have

shown

stimulation

ofall

elements

of

the

testes,

and

spermatogenesis

has

beenobserved

as

early

as

5-6yr

of

age.

In

affected

girls

andboys,

height,

weight,

and

osseous

maturatron

are

advanced.

The

increased

rate

of

bone

maturation

resultsin

early

closure

of

the

epiphyses,

and

the

ultimate

statureis

less

than

it

would

have

been

otherwise.without

treatment,

approximately

1/3

ofgirls

and

an

even

larger

percentage

of

boysachievea

height

less

than

the

5th

percentile

as

adults.Mental

development

is

usually

compatiblewith

chronological

age.

Emotional

behaviorand

mood

swings

are

common,

but

seriouspsychologic

problems

are

rare.男孩，8岁

外生殖器发育阴毛发育身高蹿长——中枢性性早熟CPPChildren

with

suspected

CPP

should

beinvestigated

in

order

to

answer

two

question:To

confirm

the

dependence

of

the

pubertalmaturation

on

hypothalamo-pituitary

function.To

exclude

an

intracranial

tumour.CPPAn

assessment

of

physiological

Gn

secretion

is

themost

helpful

investigation.Patients

have

a

nomal

pubertal

LH

and

FSH

responseafter

an

LHRH

test,

The

bone

age

is

usually

advanced.Pelvic

ultrasound

assessment

is

useful

in

girls.CT

or

MRI

scanning

of

the

brain

is

advisable.Disorder

of

pubertyPrecocious

puberty

is

more

common

ingirls.It

is

usually

due

to

early

onset

of

normalpuberty.(80%)In

boys,

It

is

usually

due

to

an

intracranialtumour(40%)Disorder

of

pubertyPrecocious

puberty

should

be

differentiated

from:Premature

thelarche:

isolated

breast

development

in

avery

young

girl.a

non-progressive,benign

condition.Premature

adrenarche:

isolated

early

appearance

ofpubic

hair

in

either

sex.

a

benign

self-limitingcondition

due

to

early

maturation

of

adrenal

androgensecretion,

but

an

adrenal

tumour

may

need

to

beexcludedCPPThe

problems

associated

with

Icpp

are

in

threeareas:Premature

sexual