IJCCR -2015v5n49 - page 9

International Journal of Clinical Case Reports 2015, Vol.5, No. 49, 1-5
2
to Tanner staging, advance in bone age (higher of
two years than the chronological age) and paraclinical
results: organic causes and hormonal results.
Once the diagnosis (positive and etiologic) is
posed and suppressive or/and specific treatment is
started, all the children were followed with clinical
reassessments, biological and radiological ones.
Results
The PP is twice more common in girls than in boys
with a sex ratio (Girl / Boy) of 2.3. The mean age at
diagnosis was 4.2 ±1.4 years (1-8.5) in girls and 5.6 ±
1.4 years (17 months - 9 years) in boys.
The average delay of endocrinology consultation
compared to the age detection of signs of early
puberty was 2.5 years in boys (6 months - 6 years) and
3.2 years in girls (6 months - 4 years). The PP was
evolutive in all the boys (100%) and only half of girls
(52.5%) (Table I).
The average of plasma sex steroids was 84.6 pmol / L
(60 to 80.4) for estradiol in girls and 4.8 nmol / l (1.4
to 4) for testosterone in boys.
The PP is of central origin (CPP) in 11.6% of girls, whose
are of idiopathic causes in 70% of them (Table II).
In boys, the PP is of central origin in 28.5% and
of organic causes in all of them. The peripheral origin
is noted in 72% of cases and it’s related to a
congenital adrenal hyperplasia (Table III).
Table I Scalability of the PP
Scalability of the PP
Girls (%)
Boys (%)
Evolutive PP
52,5
100
Regressive PP
30,5
/
Nonevolutionary PP
17
/
Table II Etiologies of PP in girls
PP
Etiologies
Number (%)
CPP Idiopathic
Organic
Hamar toma
Hydrocephalus
15 (71,4)
6 (11,6)
2
4
PP
Organic
Ovarian Tumor
Granulosa tumor
Mac Cune Albright Syndrom
Congenital adrenal hyperplasia
6 (17)
5
4
1
1
Table III Etiologies of PP in boys
PP
Etiologies
Number (%)
CPP Organic
Hamartoma
Hydrocephalus
2 (22,2)
01
01
PPP Congenital adrenal hyperplasia
7 (72)
Congenital adrenal hyperplasia related to a deficiency
of the 21-hydroxylase enzyme is the cause of
peripheral PP for all the cases in boys (Table III).
The initiation of suppressive treatment of PP by
LHRH analogues in the case of central precocious
puberty allowed seeing a regression of sexual
characters and a stabilization of bone maturation in all
cases.
At the beginning of treatment, pubertal development
was at the stage S3 (S2- S4), with a size at +2.1 ±0.8
DS / TC) and a bone age of 10.5 ±0.8 years.
Under treatment, breast development decreased and
bone maturation decelerated on the first year. The
average of bone age lead after two years of treatment
was of 1.4 years (6 months- 2 years) vs. 2.5 ±1 year at
the start of treatment. The mean final height was 158.2
±6.6 cm (-1,5 ±0.1 DS / TS).
A patient with hypothalamic hamartoma presented
neurological complications such as gelastic attacks
and generalized tonico-clonic epilepsy indicating an
urgent neurosurgical care.
The surgical treatment of ovarian tumors has led
to the regression of precoce pubertal signs;
however the histological study was in favor of
malignancy and a complementary therapy wi th
chemotherapy was indicated.
In the case of congenital adrenal hyperplasia, the
alternative suppressive therapy with glucocorticoids
failed to control the PP. The evolution was marked by
the transition to PPC training which required the
addition of LHRH analogues. A regression of sexual
characters with stabilizing of bone age was recorded.
Discussion
The PP is an important reason for consultation in
Endocrinology and Pediatrics. Its frequency seems to
be increasing in all countries in those 15 to 20 years.
Several extrinsic and intrinsic factors are implicated in
the hatching of this disease.
1,2,3,4,5,6,7,8 10,11,12,13,14
Powered by FlippingBook