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Case Presentation - Precocious Puberty Stephanie Ward

Society for Endocrinology Endocrine Nurse Training Course John MacIntyre Centre University of Edinburgh Tuesday 30th August 2005. Case Presentation - Precocious Puberty Stephanie Ward Paediatric Endocrine Clinical Nurse Specialist Great Ormond Street Hospital for Children NHS Trust. Details.

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Case Presentation - Precocious Puberty Stephanie Ward

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  1. Society for EndocrinologyEndocrine Nurse Training CourseJohn MacIntyre CentreUniversity of EdinburghTuesday 30th August 2005 Case Presentation - Precocious Puberty Stephanie Ward Paediatric Endocrine Clinical Nurse Specialist Great Ormond Street Hospital for Children NHS Trust

  2. Details • M.M. • 11 ½ months • Female • White, Caucasian

  3. Presentation • From birth noticed to have breast development • Marked progression of breast development from 6 months • 6 months - fine blond pubic hair, becoming darker by 7 months • 6 ½ months - vaginal bleed. Streak of fresh blood in every nappy with occasional mucous. Duration 4 days and no further bleeding

  4. Past Medical and Family History • No antenatal problems • Full term SVD - BW kgs • Uneventful neonatal period • Well baby • Fully immunized • Developmentally normal for age • Mother diagnosed with hypothyroidism when baby was 5 months old • Maternal aunt and grandmother have hyperthyroidism • No other significant family history

  5. Examination and Investigation • Tanner Pubertal Staging (aged 0.62 yrs/7½ mnths) - B2 P2 A1 M1 • Tests performed - Pelvic Ultrasound - LHRH test - PRL/Oestradiol - AFP/Beta – HCG - TFTs - Brain MRI

  6. Investigation Results • Pelvic Ultrasound - large bulky uterus, measuring approximately 5.3 x 1.5 x 1.8 cms. Thin endometrial stripe. Ovaries could not be visualized. No adrenal or adnexal masses identified. • LHRH test -

  7. Investigation Results (continued) • PRL 247 mU/L • Oestradiol 605 pmol/L • Alpha-Fetoprotein (AFP) 30 (0 –10 kU/L) • Beta-HCG < 1 (0 – 4 IU/L) • TFTs - TSH 3.6 (< 6 mU/L) - FT4 24.1 (14 – 23 pmol/L)

  8. Investigation Results (continued) • Brain MRI - There is a pedunculated non-enhancing mass extending inferiorly from the floor of the third ventricle on the left. The anterior and posterior pituitary glands and infundibulum are normal. Features are those of a hypothalamic hamartoma.

  9. Plan of Care • Commencement of GnRH analogue therapy - Gonapeptyl 1.875mgs s.c/i.m. at 0,14 and 28 days, thereafter every four weeks. Initial 6 weeks of Cyproterone Acetate 50mgs/m2/day in 2 divided doses. • OPA 2 months after commencement of therapy, then at 4-6 monthly intervals, to assess pubertal staging. • Auxological assessment at OP review to assess growth velocity • Repeat pelvic ultrasonography if any doubt over suppression, and pituitary MRI • Therapy to continue for minimum of 9-10 years

  10. Outcome • Treatment commenced at 0.64 yrs age (7 ½ mths) • OPA - aged 0.89yrs (10 ½ months) One more vaginal bleed 5 weeks after commencing therapy, but nothing since then. Breast development has regressed Good response to GnRH therapy. F/UP in 6 months with auxology data.

  11. Future Implications • Continuity/regularity of four weekly injections of GnRH analogue and potential of breakthrough of symptoms • Psychological support of family and child given altered physical development and nature of tumour • Side effect of medication - locally - site irritation/pain - generally - weight gain • Potential of PCOS later in life • Impact on target height • When to stop GnRH therapy

  12. References • Feuillan, P.P. et al (1999) Reproductive Axis after discontinuation of Gonadotropin-Releasing Hormone Analog treatment of Girls with Precocious Puberty: Long Term Follow-Up Comparing Girls with Hypothalamic Hamartoma to Those with Idiopathic Precocious Puberty. J Clin End Met. Vol 84 No 1 p44-49. • Gonapeptyl Data Sheet (2003) - Ferring Pharmaceuticals. • GOSH (2004) - Shared Care Guidelines – The Management Of Patients with Central Precocious Puberty (CPP) using Gonadotrophin Releasing Hormone Analogues (GnRH) .

  13. References (continued) • Heger S et al (1999) Long-Term Outcome after Depot Gonadotrophin-Releasing Hormone Agonist Treatment of Central Precocious Puberty: Final Height, Body Proportions, body Composition, bone Mineral Density, and reproductive Function. J Clin End Met. Vol 84 No 12 p4583-4590. • Jung, H. et al (2003) Association of Morphological Characteristics with Precocious Puberty and/or Gelastic Seizures in Hypothalamic Hamartoma. J Clin End Met. Vol 88 No 10 p4590-4595. • Partsch C-J et al (1998) Efficacy of the subcutaneous reformulated triptorelin depot in children with central precocious puberty. Acta Paediatr 87: 1240-4.

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