Precocious Puberty of Cerebral Origin: A Cooperative Study in Japan

SummaryA cooperative study between the 37 centers of the French Hemophilia Study Group was undertaken to establish the prevalence of inhibitor patients in the French hemophilia population. The prevalence reported in the literature varies widely from 3.6% to 17.5%. Some of the studies are dealing with a small number of patients and inhibitor patients are reported either to the total number of hemophiliacs or to the severely affected ones. The French study provided information concerning 3,435 hemophiliacs and showed a prevalence of 6.2% for the overall population. Prevalence of inhibitors was found to be 7% in the population of hemophilia A patients and 12.8% in the population of severely affected ones. The prevalence of inhibitors in the population of hemophilia B patients was 2% and 4% in the population of severely affected hemophilia B patients. The cooperative study also showed that 47.5% of inhibitors are detected before 10 years of age and that 82% of inhibitor patients are high responders. Analysis of inhibitor detection in patients under the age often showed that there was a peak in the population of 2 years old children. Although not comparable to the present study the high incidence of inhibitors with ultrapurified and recombinant FVIII reported in previously untransfused patient may be borne in mind.

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CYPROTERONE ACETATE IN THE TREATMENT OF INFANTILE ADRENOGENITAL SYNDROME WITH PRECOCIOUS PUBERTY

Acta Endocrinologica ◽

10.1530/acta.0.072s169 ◽

1973 ◽

Vol 71 (4_Suppl) ◽

pp. S169 ◽

Cited By ~ 6

Author(s):

J. Beyer ◽

K. Demisch ◽

W. Wiegelmann ◽

J. Happ ◽

F. Kollmann ◽

...

Keyword(s):

Precocious Puberty ◽

Cyproterone Acetate ◽

Adrenogenital Syndrome

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Precocious puberty (PP): effects on growth in patients treated with cyproterone acetate (CYP)

Acta Endocrinologica ◽

10.1530/acta.0.111s001 ◽

1986 ◽

Vol 113 (1_Suppl) ◽

pp. S1

Author(s):

W. SORGO ◽

E. KIRALY ◽

M. HAUPENTHAL ◽

J. HOMOKI ◽

E. HEINZE ◽

...

Keyword(s):

Precocious Puberty ◽

Cyproterone Acetate

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Pharmacodynamics of [D-Ser (t-Bu), Des-Gly]GnRH ethylamide (Buserelin®) in 6 normal volunteers.

Acta Endocrinologica ◽

10.1530/acta.0.1150235 ◽

1987 ◽

Vol 115 (2) ◽

pp. 235-242 ◽

Cited By ~ 3

Author(s):

Y. Reznik ◽

B. P. Winiger ◽

M. L. Aubert ◽

P. C. Sizonenko

Keyword(s):

Urinary Excretion ◽

Precocious Puberty ◽

Plasma Levels ◽

Intranasal Administration ◽

Cross Reaction ◽

Disappearance Rate ◽

Gnrh Analog ◽

Male Adolescents ◽

Normal Volunteers ◽

The Mean

Abstract. The disappearance rate of [D-Ser(t-bu)6,des-Gly10]GnRH ethylamide (Buserelin®, HOE 766) was studied in plasma and urine after intranasal (300 μg) or sc (10 μg/kg) administration. A radioimmunoassay for HOE 766 was developed using 125I[D-Trp6,Des-Gly10]GnRH ethylamide as tracer and an antiserum raised against HOE 766. Cross-reaction with native GnRH was only 1.7%. Sensitivity was 1 pg/tube. In 6 male adolescents, the mean plasma HOE 766 concentration (± sem) was 0.46 ± 0.08, 0.50 ± 0.10, 0.28 ± 0.04, 0.24 ± 0.04, 0.13 ± 0.03, and 0.08 ± 0.02 μg/l 30, 60, 90, 120 and 180 min after the intranasal administration, respectively. Concomitant urinary excretion of HOE 766-like material was 9.43 ± 1.96 μg/4 h. There was a good correlation between integrated plasma levels and urinary excretion (r = 0.92). In the same 6 volunteers, the plasma HOE 766 levels were 21.2 ± 3.0, 25.9 ± 0.8, 21.2 ± 0.9, 17.1 ± 0.7, 12.8 ± 1.1, 8.9 ± 0.4, and 5.9 ± 0.8 μg/l 20, 40, 60, 90, 120, 180 and 240 min after sc injection, respectively. The mean urinary excretion was 543 ± 61 μg/4 h. In two girls with precocious puberty treated during 12 to 15 months with intranasal administration of HOE 766, urinary excretion of HOE 766-like material was shown to correlate well with the degree of inhibition of plasma 17β-E2and of plasma LH and FSH responses to a GnRH challenge. Thus, monitoring of HOE 766 in urine appears to be helpful for evaluating of intranasal therapy with a GnRH analog in precocious puberty.

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