PUBERTATEA

PUBERTATEA
Definitie
 Perioada in care se desfasoara maturizarea sexuala, care implica:

 Dezvoltarea caracterelor sexuale secundare

 Puseul de crestere pubertara

 Dezvoltarea functiei de reproducere

 Modificari psihologice
 PUBERTATEA
Determinism

 Teoria « gonadostatului » (Hohlewg 1931, Grumbach, 1974)

 Scaderea sensibilitatii gonadotropilor la hormonii gonadici (f-b negativ

la doze f mici de sexoizi)

 Teoria « supresiei intrinseci »
 Existenta unui sistem inhibitor depasit, la pubertate, de factorii

stimulatori
 Teoria « desincronizarii »
 Desincronizarea secretiei GnRH
 PUBERTATEA
Inainte de pubertate
 Adrenarha
  eficientei mecanismului inhibitor
intrinsec SNC
  pragului sensibilitatii
gonadostatului la sexoizi
  pulsuri GnRH (amplitudine si
frecventa) – pred nocturn
  sensbilitate hfz la GnRH
  secretia FSH/LH
  sensibilitatea gonadica la
gonadotropi
  secretia de steroizi gonadici
Odata cu progresia pubertatii
 Reducerea continua a eficientei
mecansimului inhibitor intrinsec
 Pulsatii GnRH de tip adult urmate de
pulsuir de LH
 Dezvoltare progresiva car sex
secundare
 Debut spermatogeneza la baieti
 Aparitia f-b + la fete
 Declansarea ovulatiei la fete
 TERMENI UTILIZATI
 Adrenarha
 crestrea secretiei androgenice la nivelul zonei reticulate
adrenale
 Gonadarha
 activarea secretiei de sexoizi la nivelul gonadelor
 Pubarha
 dezvoltarea pilozitatii sexuale
 Telarha
 dezvoltarea sinilor
 Menarha
 prima menstra
 DEBUT PUBERTAR
> 8 ani(fete) >10 ani (baieti)
 Scaderea dramatica a debutului pubertar in ultimul secol
(tendinta “seculara”)
 cresterea calitatii hranei

 starea de sanatate

 diferente etnice
Tendinta « seculara » de scadere a
virstei de debut al pubertatii
Varsta de aparitie a semnelor pubertare

Semne Virsta de aparitie

pubertare - 2 DS media + 2 DS
Fete
B2 8,5 10,9 13,3
P2 9 11,2 13,4
Menarha 10,8 12,7 14,6
B5 11,5 14 16,5
Baieti
G2 9,5 11,5 13,5
P2 9,9 12 14,1
G5 11,5 14 16,5
Stadiile pubertare
Proeminarea mamelonului

Mugure mamar. Reliefarea

si cresterea diametrului
areolei
Cresterea in continuare a
mugurelui mamar.
Pigmentarea areolei
Proiectarea inainte a areolei si
a mamelonului care formeaza
un al doilea relief, in « sticla
de ceas »
Stadiu adult. Areola se
confunda cu conturul
general al snului
Stadiile pubertare

Fire rare, lungi, fine, putin pigmentate, la

nivelul labiilor mari

Fire mai dese / groase / ondulate /

pigmentate, deasupra simfizei pubiene

Par de tip adult, fara extensie catre

radacina coapsei

Par de tip adult, dispozitie in triunghi

Stadiile pubertare testiculi pilozitate pubiana
Acelasi aspect ca in Absenta
copilarie

 moderata scrotumului si Citeva fire rare, fine,

a testiculilor (4-6 ml) lungi, putin pigmentate,
Plicaturarea tegumentelor drepte, la radacina
scrotale penisului

Testiculi > (6-12 ml) Fire mai dense/groase/

ondulate, deasupra
simfizei pubiene

Testiculi > (12-16 ml) Aspect asemanator

Pielea scrotului plicaturata pilozitatii adulte, dar
extensie triunghiulara

Volum si configuratie de tip Aspect adult cu dispozitie

adult (testiculi > 16 ml) rombica
 Pubertatea intarziata
 Absenta maturarii sexuale la fete si baieti la o varsta cronologica
> + 2,5 DS peste varsta instalarii pubertatii
 baieti:

 absenta cresterii volumuli testicular la 14 ani

 fete:

 absenta telarhei la 13 ani

 absenta menarhei la 15 ani

 Pubertatea intarziata - cauze
Hipogonadism hipergonadotrop

 Congenital
 Disgenezie gonadica
 Defecte de sinteza sau receptor androgen
 Anorhidie sau critporhidie

 Sindroame
 Klinefelter
 Turner
 Noonan

 Secundar
 Chirurgie
 Radioterapie
 Traumatism
Pubertatea intirziata constitutionala
 Mai frecvent baieti
 Antecedente familiale
 Antecedente personale evocatoare
 carente nutritionale
 boli cronice
 maladia celiaca
 intirziere mai importanta a virstei
osoase
 < 11 ani la fete
 < 13 ani la baieti
 Virsta osoasa
Metacarpian I – 1 ½ ani
Osul cu cirlig - 1/2 ani
Osul mare - ¼ ani
Trapez - 4 ½ ani
Pisiform 8-10 ani
Trapezoid - 4 ½ ani
Piramidal – 2 ½ ani
Scafoid - 4 ½ ani
Semilunar – 3 ½ ani
Extremitatea distala a
Extremitatea distala a radiusului ¾ ani
cubitusului – 4 ani

 Varsta osoasa la nivelul oaselor carpului

 Virsta osoasa
 10 ani  18 ani
 Pubertatea intirziata constitutionala
 Testele hormonale inutile
 Diagnostic retrospectiv: urmarire la 4 -6 luni a
cresterii si a aparitiei semnelor pubertare
 Primele semne pubertare trebuie sa apara
 fete: inainte de 16 ani
 baieti; inainte de 18 ani

 Tratamentul nu e necesar
 Se pot administra la baieti mici doze de testosteron
retard
Pubertatea intirziata -Anorexia nervosa
 Pierdere deliberata si sustinuta in greutate
 determinata de teama de imaginea deformata a propriului
corp
 Nu trebuie confundata cu anorexia ca simptom
 caracterizata prin pierderea apetitului sau pierderea
interesului pentru mincare
Pubertatea intirziata -Anorexia nervosa
 1% adolescente din lumea
industrializata
 Greutatea , 85% din greutatea
normala sau BMI  17,5 kg/m2
 Frica obsesiva de crestere in
greutate
 Tulburare a imaginii corporale
 Amenoree
 Primara
 Secundara - oprire a evolutiei

pubertare
 Etape ale anorexiei
1. Dieta din motive cosmetice
2. Dieta din cauza fixatiei nevrotice asupra ingestiei de
alimente si a greutatii
3. Reactie anorectica
4. Anorexia nervoasa adevarata

Freis, Psychiatry, 1974

 Pubertatea intirziata - cauze
Hipogonadism hipogonadotrop
 Idiopatic

 Sindroame
 Prader-Willi
 Lawrence-Moon si Bardet-Biedl

 Deficitul de Gn RH asociat cu anosmie

 Kalmann de Morsier
 Hipopituitarism
 Deficit izolat de LH si FSH
 panhipopituitarism
Prader - Willi
 1/10000-25000
 Anomalie q11-13 crs 15
 Hipotonie neonatala
 Facies caracteristic: fata ingusta, ochi
migdalati, gura mica...
 Obezitate – legata (si) de hiperfagie
 BC: exces de masa grasa
 Hipogonadism hipogonadotrop
 Probleme de comportament + retard
intelectual
 intirziere in crestere
 Kalmann de Morsier
 1/7,500 barbati, 1/50,000 femei
 Familial sau sporadic
 X-linked KAL1 – Xp22.3
 autosomal dominant (KAL-2)
 autosomal recessif (KAL-3)
 Status eunucoidal
 Testiculi mici, frecvent critporhizi
 Anosmie
 Malformatii asociate
 faciale
 SNC
 Schelet
 renal

14 ani
Sindrom Turner
 1/2500
 45,X0, mozaicism

 Malformatii somatice

 Malformatii viscerale

 Cord sting
 Rinichi

 IQ normal (dar deficit spatio-

temporal)
 Tratament – GH, E-P
Sindrom Klinefelter
 1/1000
 47,XXY
 Testiculi mici (< 3,5 cm
lungime)
 Spermatogeneza deficitara
 Status eunucoidal
 Ginecomastie pubertara
 IQ low normal
 Pubertatea precoce
 Dezvoltarea
caracterelor sexuale
secundare la o virsta < -
2 DS fata de medie
 inainte de 8 ani la fete
 inainte de 9 ani la baieti

Riscuri
Compromiterea taliei finale
Perturbari emotionale
 PUBERTATEA PRECOCE

 Frecventa este de 8 ori mai mare la fete decit la baieti

 cele mai frecvente forme sunt idiopatice

 Pot apare unul sau mai multe semne de sexualizare

 pina la dezvoltarea unei pubertati complete

 Sexualizarea precoce se poate face

 in acelasi sens cu sexul genetic si gonadic (pubertate precoce

izosexuala)
 discordant (pubertate precoce heterosexuala).
 Pubertate
precoce isosexuala

E sau T crescuti

dozare FSH/LH/hCG

FSH, LH FSH LH 

FH LH, hCG 
raspuns N la GnRH hCG crescut

Pubertate precoce T secretante hCG Baieti

Hepatoame,Coriocarcinoame Testotoxicoza, CAH
adevarata T testiculare Neo gonadice, iatrogena

Fete
imagistica McCune Albright, Chist/
neo ovar, iatrogena
 CLASIFICARE
 Pubertate precoce adevarata
 activarea prematura a axului hipotalamo-hipofizo-gonadal

 Pubertate precoce falsa (pseudo-pubertate precoce)

 nu implica axul hipotalamo-hipofizo-gonadal

 sexoizi extragonadali

 activare independenta a gonadei

 Pubertatea precoce
Adevarata (completa izosexuala)
 Idiopatica

 Tumori hipotalamice (hamartom)

 Traumatisme, maladii inflamatorii

 Secundara tratamentului tardiv al sindromului adreno-genital

 Pubertatea precoce
Falsa (« pseudo ») - izosexuala

baieti fete
 Tumori hCG - secretante  Chist ovarian
 SNC – germinom, teratom  Neoplazie adrenala sau
 extraSNC – hepatom, ovariana secretanta de
coriocarcinom estrogeni
 Secretie androgenica crescuta
 Testiculara
 Testotoxicoza
 Adenom cel Leydig
 Adrenala
 CAH
 Neoplasm virilizant
 Pubertatea precoce
Falsa (« pseudo ») - izosexuala
Ambele sexe
 Sd. McCune-Albright

 Iatrogena sau exogena

 Hipotiroidia severa

Variante ale dezvoltarii pubertare

 Telarha precoce
 Menarha precoce izolata
 Adrenarha precoce
 Ginecomastia pubertara
 Macro-orhidism
 Premature Thelarche
 Premature unilateral or bilateral development of the breast
tissue in girls between the age of 12 to 24 months.
 There are no other associated pubertal changes.
 Bone age, growth velocity, and biochemical testing are
normal.
 It is usually a diagnosis of exclusion.
 Frequent clinical follow up to monitor growth, and pubertal
progression is required.
 Premature Adrenarche
 The early production of adrenal androgens characterizes this benign
condition.

 It presents with pubic or axillary hair, body odor, or acne before the age of
8 years. 

 There is no breast development in females and no testicular enlargement in

males.
 Bone age is usually not advanced.
 It is essential to rule out exposure to androgen sources such as creams or
gels, adrenal tumors, and late-onset CAH.
 Premature Menarche
 Isolated premature menarche is the onset of vaginal bleeding
in girls less than 7 years of age.
 They may present with either a single episode or few cycles
(less than 3) of bleeding and have normal progression to
puberty.
 Recent studies have suggested no effect on adult height.
 Sexual abuse, vaginal foreign body, and infections of the vulva
and vagina need to be ruled out.
 Pubertatea precoce
Hetero-sexuala

Feminizare la baieti Masculinizarea la fetite

 CAH – deficit de 21 si 11

 Neoplazie OH
adrenala  Tumora SR (Cushing)

 Corioepiteliom  Tumora ovariana

 Exogen (arrhenoblastom)
 Deficit de aromataza

 iatrogen
 Tratamentul pubertatii precoce
 Detectarea si tratarea leziunilor responsabile
 Oprirea maturizarii

 Sexuale

 Scheletale

 Prevenirea
 Tulburarilor emotionale
 Riscului de abuz sexual
 Tratamentul pubertatii precoce
centrale
  The decision to treat depends on the age of the child and the progression of puberty.
 If the child has rapidly progressing symptoms or if bone age is significantly advanced, consider
treatment.
 The main goals of treatment are to preserve the adult height and to alleviate the associated
psychosocial stress.
 GnRH agonists are the standard of care.
 Many different formulations (intranasal, intramuscular and subcutaneous) of long and short-
acting GnRH agonists exist. The choice of the formulation depends on the patient and clinician
preference.
  In the Romania, leuprolide acetate is the most common. It is a depot injection administered
every 3 months.
 GnRH agonist therapy is generally considered safe, with no reported significant adverse events.
The most common adverse events include local skin reactions (intramuscular pain, sterile
abscesses) and post-menopausal symptoms (hot flushes).
 While on treatment, periodic monitoring of pubertal progression, growth velocity, and skeletal
maturation are necessary.
 Tratamentul pubertatii precoce-
periferice
 Treatment is directed towards eliminating the source of sex steroids.
 Surgery is indicated in gonadal and adrenal tumors. 
 If exogenous sources of sex steroids are identified, they should be
eliminated.
 Classic congenital CAH is treated with glucocorticoids.
 In McCune-Albright syndrome, some benefit occurs with blocking the
estrogen synthesis using aromatase inhibitors (anastrozole, letrozole) and
selective estrogen selective receptor modulator (tamoxifen).  
 The optimal treatment for familial male-limited precocious puberty is not
well established, but the preferred treatment is a combination of an
androgen antagonist (spironolactone) and an aromatase inhibitor
(anastrozole, testolactone)
 PROGNOSTIC
 Early onset of treatment is usually associated with greater success in
preserving final adult height. 
 The outcomes depend on factors; such as advancement of bone change,
age at which precocious puberty initiated, the timing of initiation, and
duration of treatment.
   The HPG axis returns to normal after the cessation of the therapy, and
these children usually have a normal progression of puberty after stopping
treatment.
 There is very little information on the long term endocrine, metabolic,
reproductive, and psychological consequences.
  The prognosis of PPP varies depending on the cause.