PRECOCIOUS PUBERTY

Ask name of patient: comment on voice

High pitched childs voice normal for age

Mature female(estrogen effect)

Deep(androgenized)

Look for facial hair, male body habitus, muscular growth.

Anthromemetry and segments.

SHORT…long standing precocious,russel silver,cushings,NF 1,hypothyroidism, SGA

TALL….mc cune Albright,CAH(recent onset)

Arm span greater than ht…growth spurt

Asymmetry….russel silver

Wt …increase in cushings,hypothyroid

OFC…hydrocephalus,,.IC tumour

B.P …..11 hydroxylase def, IC tumor

Abdomen…hepatoblastoma,ovarian and adrenal mass

Tanner staging(request for orchoidometer)

Back scoliosis,screen skin for café au lai spots

Long tract signs in lower limbs plus GAIT

EYE: nerve palsies due to brain tumor

My pt is a 6 yrs old male child who is conscious & cooperative with normal facies, facial hair
growth & deep voice . His Ht is 120cm,wt is 17kg,ofc 50cm.His HR is 94/min, BP of 140/90 >85th
centile. Tanner staging is stage 3. Abdominal & spinal examination is unremarkable. Screening of
skin did not reveal any stigmata.All the accessible cranial nerves are intact, there is no focal deficit

INVESTIGATIONS:

FSH: LH ratio is low prepubertal,

GnRH stimulation ….. LH shud be < 5IU/L

Bone age… advanced in central precocious puberty

Estradiol/TESTOSTERONE

DHEA/17 OH progesterone
SKELETAL SURVEY: polyostotic fibrous dysplasia

IMAGING: USG + CT abdomen….. CT/MRI brain

INDICATIONS OF IMAGING:….. In all males

Females < 6 yrs, rapid breast enlargement, >30pg/ml of estradiol.

T/M : Leuprolide acetate 0.25-0.3 mg/kg (min 7.5 mg) I/M every 4 weeks.

Tanner atging of breast II-III can regress but not 3-5,amount of glandular tissue decreases, menses
cease, pubic hair remain stable but progression slows, dec in uterine n ovarian size.

In boys testicular size regress, variable regression in pubic hair & dec in freq of erections

If T/M is effective testosterone shud decrease to prepubertal levels < 10-20ng/dl &

estrogen <5-10pg/ml, LH < 1 IU/dl

Menarche & ovulatory cycles start abt an avg of 18 mths (16-24 mths) after cessation of therapy

GH along with GnRH analog can be used to address short stature

PRECOCIOUS PUBERTY

Before 8 yrs in girls & before 9 yrs in boys.

90% girls have idiopathic, 75% boys have CNS cause.

Female sequence….. BPM…. Breast development, pubic hair, menarche

Male sequence……testicular growth, pubic hair, axillary hair

In pseudoprecocious puberty testicular size is normal

CENTRAL PERIPHERAL

IDIOPATHIC GIRLS: ISO

CNS CAUSES: Brain tumor, hydrocephalus, Mc cune Albright
hypothalamic hamartoma, Ovarian tumor/cysts
meningeomyelocele, severe head trauma, Granulosa theca cell tumor
Adrenal tumor feminizing
Prolonged & untreated hypothyroidism Exogenous estrogens
HETERO GIRLS:
BOTH CENTRAL & PRECOCIOUS CAH
Treated CAH Adrenal/ovarian tumor
MC cune Albright Glucocorticoid receptor defect
Familial male precoious puberty Exogenous androgens
 BOYS: ISO
CAH
Leydig cell tumor/adrenal tumor
Hcg sec tumor
CNS
Hepatoblastoma
Mediastinal tumor in klinefelter
Teratoma
Glucocorticoid receptor defect
HETERO:
Adrenal tumor feminizing
Exogenous estrogens

GIRLS < 8 yrs

All 3 in sequence Not all 3 in sequence

TRUE PSEUDO

Screen skin, CNS symptoms 1) MASS ABDOMEN… ovarian cyst/tumor

Other hormonal changes of mccune ↑↑↑ estradiol/no responst to GnRH

2)ADRENARCHE …… skin pigmentation…. CAH

-ve +ve Adrenal mass

Idiopathic CT /MRI

FSH/LH

ACTH/TFTs

MCcune Albright