Society for Endocrinology Endocrine Nurse Training Course John MacIntyre Centre University of...

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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
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Transcript of Society for Endocrinology Endocrine Nurse Training Course John MacIntyre Centre University of...

Society for EndocrinologyEndocrine Nurse Training Course

John MacIntyre CentreUniversity 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

• M.M.

• 11 ½ months

• Female

• White, Caucasian

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

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

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

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 -

Time 0 minutes 20 minutes 60 minutes

LH (IU/L) 2.5 46.6 25.6

FSH (IU/L) 3.9 14.5 13.8

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)

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.

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

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.

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

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) .

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.