PERIPHERAL PRECOCIOUS PUBERTY SECONDARY TO MCCUNE-AL BRIGHT SYNDROME

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1 CASE REPORT PERIPHERAL PRECOCIOUS PUBERTY SECONDARY TO MCCUNE-AL BRIGHT SYNDROME Suhaimi H 1, Fatimah Harun 2, Mohd Yazid Jalaluddin 2 1. Department of Paediatrics, School of Medical Sciences, Universiti Sains Malaysia, Kubang Kerian, Kelantan, Malaysia 2. Department of Paediatrics, University Malaya Medical Centre, Kuala Lumpur, Malaysia Abstract We report a rare case of McCune-Albright syndrome in an 8-month-old girl who presented with early breast development and cyclical vaginal bleeds. She was confirmed to have a peripheral type of precocious puberty based on her hormonal studies. We also highlighted the use of tamoxifen in the management of peripheral type of precocious puberty. Keywords: Peripheral Precocious Puberty, Mccune-al Bright Syndrome Corresponding Author: Dr. Suhaimi Hussain, Department of Paediatrics, School of Medical Sciences, Universiti Sains Malaysia, Kubang Kerian, Kelantan, Malaysia Tel: hsuhaimi@kb.usm.my Case Report NE was an 8-month-old girl, the first child in the family, ex-premature 24 weeks with birth weight of 700 g. She had a stormy neonatal period with respiratory distress syndrome (RDS), intraventricular haemorrhage (IVH) grade 2, retinopathy of prematurity (ROP) grade 1 and was admitted for three months. She presented again at the age of six months with progressive breast development associated with per vaginal bleeding. The per vaginal bleed was followed by brownish discharge for 4-5 days that occurred monthly at six months and seven months of age. There was no similar problem in the family. Parents are non-consanguineous. The infant was on exclusive breast feeding and there was no exposure to any drugs or herbs that might lead to premature onset of puberty. Clinically, she was not dysmorphic. Both breasts were at Tanner stage 3 (Figure 1). Her vaginal mucosa was oestrogenised. She had a typical café-au-lait skin lesion at her right thigh that measured 4 x 5 cm with ragged edges (Figure 2). Her abdomen was soft and she had no other signs of precocious puberty.

2 Figure 1. Breast Tanner stage 3 Figure 2. Café-au-lait with coast of Maine

3 Hormonal study showed a peripheral type of precocious puberty with predominant follicle-stimulating hormone (FSH) rise compared to luteinizing hormone (LH) (Table 1). Her oestradiol level was very high and ultrasound pelvis revealed multiple ovarian cysts (Figure 3). Figure 3. Multiple ovarian cysts Table 1. There should be a title / description / legend of this table for e.g. hormonal profile of patient Hormonal study LH ( m IU/ml) 1.3 < FSH ( m IU/ml) Oestradiol ( pmol/l) Testosterone ( nmol/l) hcg (< 5 ml U/ml (0-10) - FT4 (10-24 pmol/l)/ / TSH ( m IU/L) Cortisol (nmol/l) DHEA-S (nmol/l) - < She was then started on tamoxifen 10 mg daily to block the effect of oestrogen on the breast and endometrium. After a few months of starting the treatment, her breast size regressed and there was no more per vaginal bleeding. Discussions This girl had a peripheral type of precocious puberty as she had prepubertal level of gonadotropins but with a pubertal level of oestradiol. Precocious puberty is the

4 appearance of secondary sexual characteristics before the age of 8 years in a girl and 9 years in a boy respectively. For central type or gonadotropin dependent precocious puberty, both LH and FSH are high and it should show predominantly LH rise compared to FSH. In a girl, LH binds to LH receptors on theca cells of ovaries and results in androgen production. The androgen is transferred to granulosa cells to produce oestrogen. FSH leads to follicular maturation. Under the influence of oestrogen, the endometrium of uterus thickens but without fertilization the endometrium is shed in the form of per vaginal bleeds. The source of high oestrogen in this girl was from the ovaries as ultrasound study showed multiple follicular cysts measuring cm (Figure 3). She had no other lesions in the ovaries and adrenal. McCune-Albright (MAS) syndrome is a rare condition in children and for a clinical diagnosis, a patient should have two out of three criterion. This girl had a typical caféau-lait and a peripheral type of precocious puberty or endocrine hyperfunction [1]. MAS is due to mutation in the alpha subunit of G protein. G protein is responsible for intracellular signaling. When a ligand/hormone binds to surface receptor, G protein becomes activated and in turn activates adenyl cyclase and increase camp. In MAS, the activated G protein remains activated and unable to be de-activated leading to endocrine hyperfunction [2,3]. Confirmation of diagnosis requires tissue biopsy to look for G protein mutation and it is in fact the best yield compared to leucocytes and skin biopsy [4,5]. The confirmation of the diagnosis based on the tissue biopsy was not done as we could not get any consent from the parents. The girl was treated with tamoxifen 10 mg daily and after 2-3 months she had no more cyclical per vaginal bleeds and her breast enlargement regressed to Tanner stage 1. Biochemically her oestradiol level was normalized to pre-pubertal level. Tamoxifen is an oestrogen blocker and it has been used for the treatment of breast cancer in adults. However its usage for the treatment of precocious puberty in children has not been as much since peripheral type of precocious puberty is such a rare condition. There were some reported cases of its usage worldwide [4,5]. Antioestrogen, blocks the effects of oestrogen on tissue, by binding and blocking oestrogen receptor on the cell surface. The precise mechanism is unknown. It does not interfere at all in the underlying process. It only blocks the effects of oestrogen on the tissues and its main effect is to stop per vaginal bleeding and rate of bone age advancement better than aromatase inhibitor (testolactone) and in fact it is relatively safe [4,5]. Even though, she was successfully treated with peripheral blocker/tamoxifen, she needs to have a long term endocrine follow up with annual screening to look for other endocrine hyperfunctions. References [1] Shenker A, Weinstein LS, Moran A, et al. Severe endocrine and nonendocrine manifestations of the McCune-Albright syndrome associated with activating mutations of stimulatory G protein GS. J Pediatr. Oct 1993;123(4):

5 [2] Weinstein LS, Shenker A, Gejman PV, et al. Activating mutations of the stimulatory G protein in the McCune-Albright syndrome. N Engl J Med. Dec ;325(24): [3] Spiegel AM. The molecular basis of disorders caused by defects in G proteins. Horm Res. 1997;47(3): [4] Eugster EA, Rubin SD, Reiter EO, et al. Tamoxifen treatment for precocious puberty in McCune- Albright syndrome: a multicenter trial. J Pediatr. 2003;143(1):60-6. [5] Eugster EA, Shankar R, Freezle LK, et al. Tamoxifen treatment of progressive precocious puberty in a patient with McCune-Albright syndrome. J Pediatr Endocrinol. 1999;12(5):681-6.

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