Assistant Professor, Department of Pediatrics, School of Medicine, Hamadan University of Medical Sciences, Hamadan, Iran
Abstract
Background: Hypothalamic hamartoma is a rare cause of central precocious puberty in female children. Hamartoma is a malformation composed of ectopic gonadotropin-releasing hormone (GnRh) neurons which secrete pulsatile gonadotropine releasing hormone.Case Report: We present a case of a 1.5-years-old female baby who presented with precocious puberty associated with hypothalamic hamartoma. Serum concentrations of follicle-stimulating hormone (FSH), luteinizing hormone (LH) and estradiol were elevated. She was treated with GnRh agonist and gamma-knife surgery. The size of breast and pubic hair returned to normal after 6 months of therapy.Conclusion: This case report emphasized how this condition should be evaluated and how the girl was managed.
Muram D. Pediatric and adolescent gynecology. In: Pernol ML, Benson R. Current obstetric and gynecology diagnosis and treatment. 6th ed. Englewood Cliffs, NJ: Prentice Hall; 1987. p. 771-2.
Kids Health. Precocious puberty [Online]. [cited 2008 Aug 1]; Available from: URL: http://kidshealth.org/parent/medical/sexual/precocious.html.
Kaplowitz PB, Oberfield SE. Reexamination of the age limit for defining when puberty is precocious in girls in the United States: implications for evaluation and treatment. Drug and Therapeutics and Executive Committees of the Lawson Wilkins Pediatric Endocrine Society. Pediatrics 1999; 104(4 Pt 1): 936-41.
Kaplan SL, Grumbach MM. Pathogenesis of sexual precocity. In Grumbach MM, Sizonenko PC, Aubert ML, editorss. Control of the. onset of puberty. Baltimore, MD: Williams & Wilkins; 1990. P. 620-60.
Mahachoklertwattana P, Kaplan SL, Grumbach MM. The luteinizing hormone-releasing hormone-secreting hypothalamic hamartoma is a congenital malformation: natural history. J Clin Endocrinol Metab 1993; 77(1): 118-24.
Palmert MR, Malin HV, Boepple PA. Unsustained or slowly progressive puberty in young girls: initial presentation and long-term follow-up of 20 untreated patients. J Clin Endocrinol Metab 1999; 84(2): 415-23.
Akhtar H, Guha K, Nahar Z. Precocious puberty: a case report. TAJ 2008; 21(2): 177-9.
Acharya SV, Gopal RA, Menon PS, Bandgar TR, Shah NS. A rare case of central precocious puberty due to hypothalamic hamartoma diagnosed in utero. Endocr Pract 2010; 16(2): 237-40.
Bridges NA, Cooke A, Healy MJ, Hindmarsh PC, Brook CG. Ovaries in sexual precocity. Clin Endocrinol (Oxf) 1995; 42(2): 135-40.
Ivarsson SA, Nilsson KO, Persson PH. Ultrasonography of the pelvic organs in prepubertal and postpubertal girls. Arch Dis Child 1983; 58(5): 352-4.
Garibaldi LR, Aceto T, Jr., Weber C, Pang S. The relationship between luteinizing hormone and estradiol secretion in female precocious puberty: evaluation by sensitive gonadotropin assays and the leuprolide stimulation test. J Clin Endocrinol Metab 1993; 76(4): 851-6.
Sigurjonsdottir TJ, Hayles AB. Precocious puberty. A report of 96 cases. Am J Dis Child 1968; 115(3): 309-21.
Hochman HI, Judge DM, Reichlin S. Precocious puberty and hypothalamic hamartoma. Pediatrics 1981; 67(2): 236-44.
Turjman F, Xavier JL, Froment JC, Tran-Minh VA, David L, Lapras C. Late MR follow-up of hypothalamic hamartomas. Childs Nerv Syst 1996; 12(2): 63-8.
Zuniga OF, Tanner SM, Wild WO, Mosier HD, Jr. Hamartoma of CNS associated with precocious puberty. Am J Dis Child 1983; 137(2): 127-33.
Freeman JL, Zacharin M, Rosenfeld JV, Harvey AS. The endocrinology of hypothalamic hamartoma surgery for intractable epilepsy. Epileptic Disord 2003; 5(4): 239-47.
Lee PA. Medroxyprogesterone therapy for sexual precocity in girls. Am J Dis Child 1981; 135(5): 443-5.
Sorgo W, Kiraly E, Homoki J, Heinze E, Teller WM, Bierich JR, et al. The effects of cyproterone acetate on statural growth in children with precocious puberty. Acta Endocrinol (Copenh) 1987; 115(1): 44-56.
Conn PM, Crowley WF, Jr. Gonadotropin-releasing hormone and its analogs. Annu Rev Med 1994; 45: 391-405.
Partsch CJ, Heger S, Sippell WG. Management and outcome of central precocious puberty. Clin Endocrinol (Oxf) 2002; 56(2): 129-48.
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