Etiology and therapeutic outcomes of children with gonadotropin-independent precocious puberty
2
조회
0
다운로드
0
인용
0
참고문헌
키워드
초록
Purpose: This study was performed to investigate the etiology, clinical features, and outcomes of patients with gonadotropin-independent precocious puberty (GIPP). Methods: The study included 16 patients (14 female and 2 male patients) who manifested secondary sexual characteristics, elevated sex hormones, or adrenal androgens with prepubertal luteinizing hormone levels after gonadotropin releasing hormone stimulation diagnosed between May 1994 and December 2015. Patients with congenital adrenal hyperplasia were excluded. Clinical features, laboratory findings, treatment modalities, and outcomes were retrospectively reviewed. Results: The median age at diagnosis was 2.6 years (range, 0.7–7.9 years) and median follow-up duration was 4.6 years (range, 1 month–9.8 years). Patients with McCune-Albright syndrome (n=5) and functional ovarian cysts (n=4) presented with vaginal bleeding and elevated estradiol levels (23.3±17.5 pg/mL); adrenocortical tumors (n=4) with premature pubarche and elevated dehydroepiandrosterone sulfate levels (87.2–6,530 μg/dL); and human chorionic gonadotropin (hCG)- producing tumor (n=1) with premature pubarche and elevated β-human chorionic gonadotropin levels (47.4 mIU/mL). Two patients were idiopathic. Six patients transited to gonadotropin-dependent precocious puberty median 3.3 years (range, 0.3–5.1 years) after the onset of GIPP. Initial and follow-up height standard deviation scores (0.99±0.84 vs. 1.10±1.10, P=0.44) and bone age advancement (1.49±1.77 years vs. 2.02±1.95 years, P=0.06) were not significantly different. Conclusion: The etiologies of GIPP are heterogeneous, and treatment and prognosis is quite different according to the etiology. Efficacy of treatment with aromatase inhibitors needs to be evaluated after long-term follow-up.
Abstract
Purpose: This study was performed to investigate the etiology, clinical features, and outcomes of patients with gonadotropin-independent precocious puberty (GIPP). Methods: The study included 16 patients (14 female and 2 male patients) who manifested secondary sexual characteristics, elevated sex hormones, or adrenal androgens with prepubertal luteinizing hormone levels after gonadotropin releasing hormone stimulation diagnosed between May 1994 and December 2015. Patients with congenital adrenal hyperplasia were excluded. Clinical features, laboratory findings, treatment modalities, and outcomes were retrospectively reviewed. Results: The median age at diagnosis was 2.6 years (range, 0.7–7.9 years) and median follow-up duration was 4.6 years (range, 1 month–9.8 years). Patients with McCune-Albright syndrome (n=5) and functional ovarian cysts (n=4) presented with vaginal bleeding and elevated estradiol levels (23.3±17.5 pg/mL); adrenocortical tumors (n=4) with premature pubarche and elevated dehydroepiandrosterone sulfate levels (87.2–6,530 μg/dL); and human chorionic gonadotropin (hCG)- producing tumor (n=1) with premature pubarche and elevated β-human chorionic gonadotropin levels (47.4 mIU/mL). Two patients were idiopathic. Six patients transited to gonadotropin-dependent precocious puberty median 3.3 years (range, 0.3–5.1 years) after the onset of GIPP. Initial and follow-up height standard deviation scores (0.99±0.84 vs. 1.10±1.10, P=0.44) and bone age advancement (1.49±1.77 years vs. 2.02±1.95 years, P=0.06) were not significantly different. Conclusion: The etiologies of GIPP are heterogeneous, and treatment and prognosis is quite different according to the etiology. Efficacy of treatment with aromatase inhibitors needs to be evaluated after long-term follow-up.
참고문헌 (25)
- Carel JC, "Consensus statement on the use of gonadotropin-releasing hormone analogs in children", Pediatrics, 123, pp.e752~e762 (2009)
- Carel JC, "Clinical practice: precocious puberty", N Engl J Med, 358, pp.2366~2377 (2008)
- Fuqua JS, "Treatment and outcomes of precocious puberty:an update", J Clin Endocrinol Metab, 98, pp.2198~2207 (2013)
- Eugster EA, "Peripheral precocious puberty : causes and current management", Horm Res, 71(Suppl 1), pp.64~67 (2009)
- Collins MT, "McCune-Albright syndrome and the extraskeletal manifestations of fibrous dysplasia", Orphanet J Rare Dis, 7(Suppl), pp.S1~S4 (2012)
- Eugster EA, "Tamoxifen treatment for precocious puberty in McCune-Albright syndrome: a multicenter trial", J Pediatr, 143, pp.60~66 (2003)
- Kreher NC, "Treatment of familial male-limited precocious puberty with bicalutamide and anastrozole", J Pediatr, 149, pp.416~420 (2006)
- Korea Centers for Disease Control and Prevention, "2007 Korean children and adolescents growth standard (commentary for the development of 2007 growth chart)" (2007), Korea Centers for Disease Control and Prevention, Division of Chronic Disease Surveillance
- Greulich W, "Radiographic atlas of skeletal development of the hand and wrist" (1955), Stanford University Press
- Weinstein LS, "Activating mutations of the stimulatory G protein in the McCune-Albright syndrome", N Engl J Med, 325, pp.1688~1695 (1991)
- Lumbroso S, "Activating Gsalpha mutations: analysis of 113patients with signs of McCune-Albright syndrome: a European Collaborative Study", J Clin Endocrinol Metab, 89, pp.2107~2113 (2004)
- Mieszczak J, "Treatment of precocious puberty in McCune-Albright syndrome", Pediatr Endocrinol Rev, 4(Suppl 4), pp.419~422 (2007)
- Völkl TM, "McCune-Albright syndrome : clinical picture and natural history in children and adolescents", J Pediatr Endocrinol Metab, 19(Suppl 2), pp.551~559 (2006)
- de G Buff Passone C, "Tamoxifen improves final height prediction in girls with McCune-Albright syndrome: a long follow-up", Horm Res Paediatr, 84, pp.184~189 (2015)
- Feuillan P, "Letrozole treatment of precocious puberty in girls with the McCune-Albright syndrome: a pilot study", J Clin Endocrinol Metab, 92, pp.2100~2106 (2007)
- Sims EK, "Fulvestrant treatment of precocious puberty in girls with McCune-Albright syndrome", Int J Pediatr Endocrinol, 2012, pp.26~ (2012)
- Wendt S, "Neoplastic causes of abnormal puberty", Pediatr Blood Cancer, 61, pp.664~671 (2014)
- Sabbaga CC, "Adrenocortical carcinoma in children: clinical aspects and prognosis", J Pediatr Surg, 28, pp.841~843 (1993)
- Ciftci AO, "Adrenocortical tumors in children", J Pediatr Surg, 36, pp.549~554 (2001)
- Michalkiewicz E, "Clinical and outcome characteristics of children with adrenocortical tumors: a report from the International Pediatric Adrenocortical Tumor Registry", J Clin Oncol, 22, pp.838~845 (2004)
- Fassnacht M, "Adrenal tumors: how to establish malignancy?", J Endocrinol Invest, 27, pp.387~399 (2004)
- Wolthers OD, "Androgen secreting adrenocortical tumours", Arch Dis Child, 80, pp.46~50 (1999)
- McAteer JP, "Predictors of survival in pediatric adrenocortical carcinoma: a Surveillance, Epidemiology, and End Results (SEER) program study", J Pediatr Surg, 48, pp.1025~1031 (2013)
- Foster CM, "Variable response to a long-acting agonist of luteinizing hormone-releasing hormone in girls with McCune-Albright syndrome", J Clin Endocrinol Metab, 59, pp.801~805 (1984)
- Pescovitz OH, "True precocious puberty complicating congenital adrenal hyperplasia: treatment with a luteinizing hormone-releasing hormone analog", J Clin Endocrinol Metab, 58, pp.857~861 (1984)