Literature DB >> 8194554

Familial testotoxicosis in a Chinese family.

Y J Lim1, L C Low.   

Abstract

We report two cases of 'familial testotoxicosis' in a family of Southern Chinese descent. The proband, an 8-year 4-month-old boy and his 35-year-old father both presented with early sexual development. In both cases the testicular volume was only 6 ml despite fully developed secondary sexual characteristics. Both patients had adult testosterone concentrations but a suppressed gonadotrophin response to gonadotrophin-releasing hormone. The suppressed gonadotrophin response to gonadotrophin-releasing hormone in the father suggests that autonomous gonadal production of sex steroid by the testes can persist well into adult life in some patients with familial testotoxicosis.

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Year:  1994        PMID: 8194554     DOI: 10.1007/bf01954510

Source DB:  PubMed          Journal:  Eur J Pediatr        ISSN: 0340-6199            Impact factor:   3.183


  23 in total

1.  Familial male sexual precocity: report of the eleventh kindred found, with observations on blood group linkage and urinary C19-steroid excretion.

Authors:  F BEAS; R P ZURBUGG; S G LEIBOW; R G PATTON; L I GARDNER
Journal:  J Clin Endocrinol Metab       Date:  1962-11       Impact factor: 5.958

2.  Treatment of familial male precocious puberty with spironolactone and testolactone.

Authors:  L Laue; D Kenigsberg; O H Pescovitz; K D Hench; K M Barnes; D L Loriaux; G B Cutler
Journal:  N Engl J Med       Date:  1989-02-23       Impact factor: 91.245

3.  The role of androgens in the initiation of spermatogenesis in man.

Authors:  E Steinberger; A Root; M Ficher; K D Smith
Journal:  J Clin Endocrinol Metab       Date:  1973-11       Impact factor: 5.958

Review 4.  The use of ketoconazole as an inhibitor of steroid production.

Authors:  N Sonino
Journal:  N Engl J Med       Date:  1987-09-24       Impact factor: 91.245

5.  Male-limited familial precocious puberty in three generations. Apparent Leydig-cell autonomy and elevated glycoprotein hormone alpha subunit.

Authors:  E O Reiter; R S Brown; C Longcope; I Z Beitins
Journal:  N Engl J Med       Date:  1984-08-23       Impact factor: 91.245

6.  Ketoconazole in the management of precocious puberty not responsive to LHRH-analogue therapy.

Authors:  F J Holland; L Fishman; J D Bailey; A T Fazekas
Journal:  N Engl J Med       Date:  1985-04-18       Impact factor: 91.245

7.  Gonadotropin-independent precocious puberty ("testotoxicosis"): influence of maturational status on response to ketoconazole.

Authors:  F J Holland; S E Kirsch; R Selby
Journal:  J Clin Endocrinol Metab       Date:  1987-02       Impact factor: 5.958

Review 8.  Gonadotropin-independent precocious puberty.

Authors:  F J Holland
Journal:  Endocrinol Metab Clin North Am       Date:  1991-03       Impact factor: 4.741

9.  A novel testis-stimulating factor in familial male precocious puberty.

Authors:  P K Manasco; M E Girton; R L Diggs; J L Doppman; P P Feuillan; K M Barnes; G B Cutler; D L Loriaux; B D Albertson
Journal:  N Engl J Med       Date:  1991-01-24       Impact factor: 91.245

10.  Site of action of low dose ketoconazole on androgen biosynthesis in men.

Authors:  R J Santen; H Van den Bossche; J Symoens; J Brugmans; R DeCoster
Journal:  J Clin Endocrinol Metab       Date:  1983-10       Impact factor: 5.958

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