scholarly journals Adult height following a combined treatment of ketoconazole - cyproterone acetate - leuprolide depot in a boy with atypical McCune-Albright syndrome

HORMONES ◽  
2014 ◽  
Author(s):  
Maria Francesca Messina ◽  
Tommaso Aversa ◽  
Luisa de Sanctis ◽  
Malgorzata Wasniewska ◽  
Mariella Valenzise ◽  
...  
Keyword(s):  
Cyproterone Acetate ◽  
Adult Height ◽  
Combined Treatment ◽  
Mccune Albright Syndrome ◽  
Albright Syndrome ◽  
Mccune Albright

scholarly journals Combined treatment with bicalutamide and anastrozole in a young boy with peripheral precocious puberty due to McCune-Albright Syndrome

2012 ◽  
Vol 59 (2) ◽  
pp. 111-117 ◽  
Author(s):  
Daniele Tessaris ◽  
Patrizia Matarazzo ◽  
Alessandro Mussa ◽  
Gerdi Tuli ◽  
Francesca Verna ◽  
...  
Keyword(s):  
Precocious Puberty ◽  
Combined Treatment ◽  
Mccune Albright Syndrome ◽  
Albright Syndrome ◽  
Peripheral Precocious Puberty ◽  
Mccune Albright

scholarly journals SUN-061 Anthropometric and Reproductive Outcomes of Patients with Gonadotropin-Independent Precocious Puberty Due to McCune-Albright Syndrome After Treatment with Distinct Therapeutic Agents

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Aline Guimarães de Faria ◽  
Priscila Sales Barroso ◽  
Carolina Ramos ◽  
Ana Pinheiro Machado Canton ◽  
Marina Cunha Silva ◽  
...  
Keyword(s):  
Aromatase Inhibitors ◽  
Precocious Puberty ◽  
Vaginal Bleeding ◽  
Adult Height ◽  
Therapeutic Agents ◽  
Chronological Age ◽  
Mccune Albright Syndrome ◽  
First Choice ◽  
Albright Syndrome ◽  
Mccune Albright

Abstract Ovarian estrogen-secreting cysts leading to peripheral precocious puberty (PPP) are some of the major clinical manifestations of the McCune-Albright syndrome (MAS). Therapeutic options for PPP of MAS include tamoxifen, progestational agents, aromatase inhibitors (AI) and anti-androgens that aiming to block sex steroid synthesis or action. Here, we described the anthropometric and reproductive follow-up of patients with PPP of MAS treated with distinct therapeutic agents. Thirteen unrelated girls with MAS were studied. They had PPP combined with café-au-lait spots or/and fibrous dysplasia. All patients were treated with one or more of the following agents: tamoxifen, medroxyprogesterone acetate, aromatase inhibitors (anastrozole or letrozole) and anti-androgens (cyproterone), and, in cases with secondary gonadotropic axis activation, depot GnRHa was used. Patients were evaluated every three months, when height, weight, and Tanner pubertal stage were determined. Vaginal bleeding or other adverse effects were also reviewed. The chronological age (CA) at the diagnosis of PPP was 5.9 ± 2.35 (2.4 to 10.2 years). Thelarche and vaginal bleeding were the first manifestations in 76.9% and 53%, respectively. The first choice of treatment was tamoxifen in 30.7% of the patients, followed by aromatase inhibitors (23%) and medroxyprogesterone acetate in 23% of them. Tamoxifen plus medroxyprogesterone, or cyproterone, or leuprorelin were used (each one) as the first choice in 1 patient (7.6%). Eight patients (61%) presented secondary central precocious puberty and were treated with depot GnRHa. Vaginal bleeding was recurrent in 70% of patients, during treatment. Progression of breast Tanner stage during treatment occurred in 78% of the patients. The great majority (80%) of girls presented bone age (BA) advancement at the diagnosis of PPP (mean Δ BA - CA of 3.2±1.3 yr), which was normalized for chronological age in all except one patient. The mean duration of treatment was 5.8 ± 3.4 yr (ranging from 1 to 12 yr). Three patients are still under medical treatment. Hypertrichosis and uterine enlargement were the main side effects of tamoxifen in 3 and 5 patients, respectively. One patient treated with letrozole presented laboratory hyperandrogenism. Ten patients reached their adult height (149.9 ± 7.9 cm), 60% of them were below their target height. Menarche occurred at a median age of 11.8 yr (10.4 to 14 y), and all but one patient presented regular menstrual cycles. One patient spontaneously became pregnant. Despite a reasonable number of treatment options for peripheral PP in MAS, none of them showed proven effective results in stopping vaginal bleeding, reduce pubertal progression and preserving potential genetic adult height. Therefore, due to the extremely heterogeneous nature of PPP of MAS, the clinical treatment remains a challenge.

scholarly journals Management of precocious puberty in girls with McCune–Albright syndrome using letrozole

2018 ◽  
Vol 7 (12) ◽  
pp. 1424-1431 ◽  
Author(s):  
Xi Wang ◽  
Qi Yu
Keyword(s):  
Precocious Puberty ◽  
Growth Velocity ◽  
Adult Height ◽  
Skeletal Maturation ◽  
College Hospital ◽  
Cross Sectional ◽  
Mccune Albright Syndrome ◽  
The Mean ◽  
Albright Syndrome ◽  
Mccune Albright

Objective To evaluate the safety and efficacy of letrozole in girls with progressive precocious puberty (PP) associated with McCune–Albright syndrome (MAS). Design Monocentric retrospective cross-sectional and longitudinal study of consecutive patients. Patients Ten MAS patients treated at Peking Union Medical College Hospital between September 1999 and December 2017 were retrospectively reviewed; those with complications due to PP were followed. Results The mean age at letrozole initiation was 4.5 ± 2.6 years, while the mean duration of treatment was 3.3 ± 2.4 years. Letrozole was highly effective at decreasing the rate of skeletal maturation, with a significant decrease in the bone age-to-chronological age (BA/CA) ratio from 1.9 ± 1.1 pre-treatment to 1.5 ± 1.2 on letrozole treatment (P = 0.016). Moreover, growth velocity Z-scores declined from 0.41 ± 0.5 to −0.2 ± 0.31 with treatment (P < 0.001). Predicted adult height Z-scores increased significantly from −2.03 ± 2.33 at baseline to 1.13 ± 0.84 following treatment initiation (P = 0.029). Moreover, vaginal bleeding declined significantly on letrozole. Conclusions Our findings suggest that letrozole may be an effective therapy in some girls with MAS, as treatment results in improved BA/CA ratio, growth velocity and predicted adult height. Possible adverse effects include nettle rash.

Abstract #39: McCune-Albright Syndrome in Conjunction with Acromegaly and Subclinical Hyperthyroidism

2003 ◽  
Vol 9 ◽  
pp. 15-16
Author(s):  
Manju Chandran ◽  
Eric N. Gold ◽  
Roopa Sathyaprakash ◽  
Leonard J. Deftos
Keyword(s):  
Subclinical Hyperthyroidism ◽  
Mccune Albright Syndrome ◽  
Albright Syndrome ◽  
Mccune Albright

Atypical Femoral Fracture in McCune-Albright Syndrome

2020 ◽  
Vol 7 (3) ◽  
pp. 147-152
Author(s):  
Salman Ghaffari ◽  
◽  
Mehran Razavipour ◽  
Parastoo Mohammad Amini ◽  
◽  
...  
Keyword(s):  
Femoral Fracture ◽  
Dynamic Compression ◽  
Plate Fixation ◽  
Medullary Canal ◽  
Mccune Albright Syndrome ◽  
Café Au Lait Spots ◽  
History Of ◽  
Albright Syndrome ◽  
Mccune Albright

McCune-Albright Syndrome (MAS) is characterized by endocrinopathies, café-au-lait spots, and fibrous dysplasia. Bisphosphonates are the most prescribed treatment for reducing the pain but their long-term use has been associated with atypical fractures of cortical bones like femur in patients. We present a 23-year-old girl diagnosed with MAS. She had an atypical mid-shaft left femoral fracture that happened during simple walking. She also had a history of long-term use of alendronate. Because of the narrow medullary canal, we used 14 holes hybrid locking plate for the lateral aspect of the thigh to fix the fracture and 5 holes dynamic compression plate (instead of the intramedullary nail) in the anterior surface to double fix it, reducing the probability of device failure. With double plate fixation and discontinuation of alendronate, the complete union was achieved five months after surgery

Sign in / Sign up

Export Citation Format

Share Document