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Precocious Pseudopuberty With Testicular Enlargement - medIND

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Clinical Brief<br />

<strong>Precocious</strong> <strong>Pseudopuberty</strong> <strong>With</strong> <strong>Testicular</strong> <strong>Enlargement</strong><br />

R. Rajput, A. Bhansali, R. Bhat and R. Sialy<br />

Department of Endocrinology, Postgraduate Institute of Medical Education and Research, Chandigarh, India<br />

Abstract. Gonadotropins independent precocious puberty (GIPP) in male is characterized by early appearance of sexual hairs<br />

and phallic growth but without testicular enlargement. We report a case of GIPP with testicular enlargement who was diagnosed<br />

to have testotoxicosis and successfully managed with spironolactone. [Indian J Pediatr 2006; 73 (4) : 356-358]<br />

E-mail : anilbhansali_endocrine@rediffmail.com<br />

Key words : <strong>Precocious</strong> pseudopuberty; Testotoxicosis<br />

Puberty is the process of physical maturation manifested<br />

by an increase in growth rate and appearance of<br />

secondary sexual characteristics. Puberty is said to be<br />

precocious if it occurs in boys younger than 9 years of age,<br />

while in girls, less than 7 years of age, and in African<br />

American girls younger than 6 years of age. 1 <strong>Precocious</strong><br />

puberty can be divided into two categories. The first<br />

category is gonadotropins dependent precocious puberty<br />

(GDPP) or true precocious puberty, which involves the<br />

premature activation of hypothalamic-pituitary-gonadal<br />

axis and characterized by appearance of axillary and<br />

pubic hairs along with testicular enlargement. The second<br />

category is gonadotropins independent precocious<br />

puberty (GIPP) or precocious pseudopuberty in which the<br />

increase in sex steroids is independent of pituitary<br />

gonadotropins release, and axillary and pubic hairs<br />

appear along with phallic enlargement but without<br />

associated testicular enlargement.<br />

We describe a case of a five and a half year old boy<br />

who presented with precocious pseudopuberty with<br />

testicular enlargement.<br />

Correspondence and Reprint requests : Dr. Anil Bhansali,<br />

Department of Endocrinology, Postgraduate Institute of Medical,<br />

Education and Research, Chandigarh- 160012, India. Fax : 91-172-<br />

744401, 745078<br />

1, 2<br />

CASE REPORT<br />

A five and a half-year-old boy presented with progressive<br />

phallic enlargement, development of axillary and pubic<br />

hairs, aggressive behavior and rapid increase in height<br />

over the last two years. The child was born of a nonconsanguineous<br />

marriage and had no family history of<br />

precocious puberty. He had no history of central nervous<br />

system infections, head injury, neoplasm or prior<br />

radiation and exposure to exogenous sex steroids. On<br />

examination, his height was 128 cm (90 th percentile) and<br />

weight was 23.4 kg, with target height of 165.3cm. He had<br />

no café-au-lait spots and bony swellings. Systemic<br />

examination was unremarkable. Genital examination<br />

revealed a stretched penile length (SPL) of 6 cm and<br />

testicular volume of 6 ml bilaterally (Prader’s orchidometer).<br />

Both testes were firm, symmetrical and nontender<br />

(Fig 1). Axillary and pubic hairs were Tanner stage<br />

2 and had no gynecomastia. Investigations revealed a<br />

Fig. 1. Showing clinical profile of the patient.<br />

356 Indian Journal of Pediatrics, Volume 73—April, 2006<br />

102


103<br />

bone age of 10 years. Basal serum lentiaisling hormone<br />

(LH) was


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3. Low LC, Wang Q: Gonadotropin indepaendent precocious<br />

puberty. J Pediatr Endocrinol Metab 1998: 497-507.<br />

4. Melvin M Grumbach, Dennis M Styne. Puberty: Ontogeny,<br />

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Kronenberg, Metmed, polonsky, eds. Williams’s textbook of<br />

Endocrinology. Phialdelphia; WB Saunders, 2003; 1219-1221.<br />

5. Egli CA, Rosenthal SM, Grumbach MM et al. Pituitiary<br />

gonadotropin-independent male-limited autosomal domonant<br />

sexual precocity in nine generations: familial testotoxicosis.<br />

R. Rajput et al<br />

Arch Pathol Lab Med 1985; 109 : 990-995.<br />

6. Shenker A, Laue L, kosugi S et al. A constitutively activating<br />

mutation of the luteinizing hormone receptor in familial male<br />

precocious puberty. Nature 1993; 365: 652-654.<br />

7. Laue L, Kenigsberg D, Pescovitz OH. Treatment of familial<br />

male precocious puberty with spironolactone and testolactone.<br />

N Engl J Med 1989; 320 : 498-502.<br />

8. Leschek EW, Jones J, Barnes KM et al. Six year results of<br />

spironolactone ans testolactone treatment of familial malelimited<br />

precocious puberty with addition of deslorelin after<br />

central puberty onset. J Clin Endocrinol Metab 1999; 84(1) : 175-<br />

178.<br />

358 Indian Journal of Pediatrics, Volume 73—April, 2006<br />

104

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