PUBERTY DISORDER

OLEH
RAHMAD RAMADHANI

SUPERVISOR
dr. Gladys Gunawan, Sp.A(K)
dr. Astarini Hidayah, Sp.A
PUBERTY

 Puberty is the developmental stage during which a child becomes a young adult, characterized by the
maturation of gametogenesis, secretion of gonadal hormones, and development of secondary sexual
characteristics and reproductive functions
NORMAL PUBERTY DEVELOPMENT

Gonadal steroid hormone (estrogen or testosterone) levels promote the development and
growth of the secondary sexual characteristics and the changes in body composition
associated with pubertal development.
NORMAL PUBERTY DEVELOPMENT
PHYSICAL DEVELOPMENT DURING PUBERTY

 One of the hallmarks of puberty is the rapid growth spurt that takes place at this stage of development.
 There are sex-specific patterns in terms of the timing of the growth spurt as well as age at skeletal
maturity. On females growth spurt happens in early stages of puberty with average age 12,1 years. On
males growth spurt happens in later stages of puberty with average age 14,1 years.
 Tanner staging process  assess pubertal stage
 The Tanner staging process describes the pattern of genital and pubic hair development in boys as the
testes enlarge and the pattern of breast and pubic hair development in girls as the ovaries enlarge.
GROWTH VELOCITY DURING
PUBERTY
TANNER STAGE
DISORDER OF PUBERTY

 Precocious puberty is diagnosed when secondary sexual characteristics are identified (tanner stage 2)
in girls younger than 8 years and boys younger than 9 years.
 Puberty is considered delayed when there are no signs of breast development by 13 years of age in
girls or testicular enlargement by 14 years of age in boys
PRECOCIOUS PUBERTY:
ETIOLOGY

 Central precocious puberty (CPP) caused by early activation

of HPG axis
 Peripeheral precocious puberty (PPP) caused by stimulation
of steroid sex hormone without influence of Gonadotropin
hormones
PRECOCIOS PUBERTY:
DIAGNOSIS

 Anamnesis : onset of the signs, progression rate, and growth tempo in the last 6-12 months, presence
of secondary sex characteristics (acne, oily skin, erection, night ejaculation and vaginal bleeding) in
addition to the presence of pubertal signs. History of PP in family supports the diagnosis of familial
forms
 Physical examination: Pubertal staging should be performed according to Tanner-Marshall method on
physical examination, and anthropometric evaluations should be defined by measurement of weight,
height and body proportions. All old and new data should be marked on growth chart. Growth velocity
per year must be calculated
 Laboratory evaluation : measurement of gonadotropins (LH, FSH) and related sex steroids
 Imaging : Bone age, pelvic ultrasonography in girls to evaluate uterine and ovarian size, Cranial and
pituitary magnetic resonance imaging (MRI) should be performed to rule out organic CPP
PRECOCIOS PUBERTY:
DIAGNOSTIC APPROACH IN GIRL
PRECOCIOS PUBERTY:
DIAGNOSTIC APPROACH IN BOY
CENTRAL PRECOCIOS PUBERTY:
TREATMENT

 The main goal of treatment in children with CPP is the preservation of height potential.
 Patient with CPP indicated to get Gonadotropin releasing hormone analog (GnRHa) therapy. GnRHa plays
important role in CPP therapy.
 Leuproreline was one of GnRHa that widely used for CPP treatment. Leuproreline acetat was given with
inisial dose 100 µg/Kg/month (IM or SC) and maintenance dose 80-100 µg/Kg/month
 During therapy with GnRHa the patient must be evaluated every 3 month for
1. Growth velocity
2. Secondary sex sign with tanner stage
3. LH, testosterone estrogen level
4. Bone age
PERIPHERAL PRECOCIOS PUBERTY:
TREATMENT

 The main goal of therapy is to reduce the production of seroid sex hormone
 Medicamentosa used for PPP :
1. Medroxy progesterone acetat
2. Siproterone acetat
3. Ketoconazole
4. Testolactone
DELAYED PUBERTY

 Delayed puberty is the absence of breast development by 13 years of age in girls or the absence of
testicular growth to at least 4 mL in volume or 2.5 cm in length by 14 years of age in boys.
 Constitutional delay of growth and puberty is the most common cause of delayed puberty in boys
(60%) and girls (30%).
 It represents an extreme of the normal spectrum of pubertal timing and is a diagnosis of exclusion. For
more than 75% of patients with constitutional delay of growth and puberty, family history may reveal
parental pubertal delay
GROWTH IMPAIRMENT IN PRECOCIOUS PUBERTY

 Impaired adult height is the main long-term somatic consequence of untreated CPP
 The adult height in untreated men is about −2.5/−3.0 standard deviation scores (SDS)
below the normal mean
 Adult height in men results in at least –1 SDS below the mean adult height of females
with untreated CPP
 Other concerns related to CPP are altered body proportions in adulthood, with an
upper: lower ratio >1
PSYCHOLOGICAL CONSEQUENCES OF PRECOCIOUS PUBERTY
PSYCHOLOGICAL CONSEQUENCES OF PRECOCIOUS PUBERTY
PSYCHOLOGICAL CONSEQUENCES OF PRECOCIOUS PUBERTY
DELAYED PUBERTY:
ETIOLOGY

 Causes of delayed puberty are broadly divided into two categories, based on where the hypothalamic-pituitary-
gonadal (HPG) axis failure has occurred.
1. Hypergonadotropic hypogonadism is characterized by an appropriate activation of the hypothalamic-pituitary
component, but failure of gonadal sex steroid production.
2. Hypogonadotropic hypogonadism (HH) is secondary to delay or failure of the hypothalamic/ pituitary portion
of the HPG axis.
 The most common cause of pubertal delay is CDGP
DELAYED PUBERTY:
ETIOLOGY
DELAYED PUBERTY:
DIAGNOSTIC APPROACH
DELAYED PUBERTY:
DIAGNOSTIC APPROACH
DELAYED PUBERTY:
THERAPY

 Therapy for delayed puberty aimed for achieve sign of puberty, growth velocity acceleration, achievement of libido, sexual
activity, and fertility
 Constitutional delay of growth and puberty
 Without any therapy
 Weak androgenic anabolic for several months, such as oxandrolone, fluoximesterone with dosage 0.04-0.1 mg / kg / day orally
 Hypergonadotropic hypogonade
 Boy : bepotestosterone enantate 100-200 mg intramuscular every 3-4 weeks or equivalent
 Girl : Ethynil estradiol initial 0.02-0.05 mg/day orally or equibalent for 3 weeks/ month + medroxyprogesterone acetate 5
mg/day x 5 day
 Hypogonadotropic hypogonade
 Boy : -
 Girl : Ethynil estradiol, Clomyphen sitrat 50-100 mg/day for 5 day
GROWTH IMPAIRMENT IN DELAYED PUBERTY

 The natural history in boys who have delayed puberty is for the growth spurt to start
sometime between the ages of 15 and 17 years,
 Because they have a longer period of time to grow, the delayed growth spurt usually
results in an adult height within the lower half of the normal range.
 Review of the growth chart usually shows linear growth slightly below but parallel to
the third percentile for many years.
GROWTH IMPAIRMENT IN DELAYED PUBERTY
PSYCHOLOGICAL CONSEQUENCES OF PRECOCIOUS PUBERTY
THANK YOU