OBJECTIVES. Rebecca McEachern, MD. Puberty: Too early, Too Late or Just Right? Special Acknowledgements. Maryann Johnson M.Ed.

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1 1 Puberty: Too early, Too Late or Just Right? Maryann Johnson M.Ed., BSN, RN Special Acknowledgements Rebecca McEachern, MD OBJECTIVES Illustrate basic endocrine system and hormonal pathways Define the evaluation process and treatment of endocrine disorders Describe nursing implications and care for children with endocrine disorders and their families 2 1

2 Slide 1 1 I added a break between puberty and you questiosn about it. REBECCA MCEACHERN, 2/20/2013 Slide 3 2 I changed the verbs in the objectives to match those I had used when you sent in your objectives. The objectives are the listener's objectives not the speaker's objectives so you are telling them what they should be able to do by the end of your talk. REBECCA MCEACHERN, 2/20/2013

Conflict of Interest Disclosure None Endocrine System Interacts with all body systems Controls growth and development Responds to stress Has various roles in reproduction, birth and lactation

3 Conflict of Interest Disclosure None Endocrine System Interacts with all body systems Controls growth and development Responds to stress Has various roles in reproduction, birth and lactation Maintains homeostasis 3 Is critical for energy production, storage and utilization Master gland Location Structure Disorders MRI> Empty Sella (flattened pitutary) Interupted pit stalk>level of disruption > hormone affected ADH Oxytocin GH FSH/LH ACTH TSH Prolactin 2

4 Slide 5 3 I only made 2 changes - make all first words verbs so they are consistent REBECCA MCEACHERN, 2/20/2013

Growth Hormone Secretion GNRH LH/FSH + - Testosterone LH Leydig cells Testosterone FSH

5 Normal Puberty Gonadarche: gonadal stimulation Adrenarche: androgen production Thelarche: breast development Normal variations: Premature thelarche Benign premature adrenarche Growth Hormone Secretion GNRH LH/FSH + - Testosterone LH Leydig cells Testosterone FSH Sertoli cells Spermatogenesis Androgens (DHEA-S) Adrenarche: Pubic hair Acne Axillary sweat/odor 3

6 Boys Pubertal Change testicular volume Pubic hair Maximum growth rate Adult distribution of hair Average age Growth Hormone Secretion GNRH LH/FSH + - Estradiol Androgens (DHEA-S) Pubic hair Acne Axillary sweat/odor 4

7 Girls Pubertal change Breast bud Pubic hair Max. growth spurt Adult pubic hair Menarche Adult breasts Average age Abnormal Puberty Precocious puberty <8 in girls <9 in boys Central axis on early Peripheral peripheral sources of testosterone or estradiol Combined Delayed puberty Non-benign adrenarche Virilization 5

8 Central Precocious Puberty Girls Thelarche < 8 years of age Idiopathic (80%) Hypothalamic harmatoma Congenital brain defects Tumors, Cysts Infection, Inflammation Radiation, Chemotherapy Central Precocious Puberty Boys Testicular enlargement < 9 years of age Idiopathic (30%) Hypothalamic harmatoma Congenital brain defects Tumors, Cysts Infection, Inflammation Radiation, Chemotherapy Peripheral Precocious Puberty in Boys and Girls Severe hypothyroidism TSH FSH Receptor PPP Tumors Estrogen secreting Androgen secreting Ovarian Cyst Exogenous Hormones CAH 6

9 Clinical Evaluation Growth Assessment most important History Family history (pubertal timing, hirsutism, infertility) Symptoms as described above Physical Thyroid exam Fundoscopic exam Skin exam (café au lait spots, axillary freckling) Breasts/Gonads plus genitalia Axillary sweat, acne, pubic hair Precocious Puberty Investigation Gonadotropins to determine origin Estradiol/testosterone Bone age If central MRI Evaluate remainder of pituitary Genetic evaluation as required Precocious Puberty Investigation If peripheral TSH/PTH to evaluate intrinsic receptor activation Abdominal and if needed testicular CT etc (tumors) 7

10 Precocious Puberty Investigation If premature adrenarche 17 OH P to rule out CAH Testosterone and DHEAS as measures of activation of axis and to rule out tumours 6 Treatment of Central Precocious Puberty Treat underlying cause if possible Treat underlying endocrine dysfunction LT4 for Hypothyroidism Cortisol replacement for CAH Surgery Remove source 7 Treatment of Central Precocious Puberty Consider a decision to stop puberty based on: Rate of progression Degree of final height compromise (early growth spurt>short adult stature) Degree of advancement of bone age Predicted adult height Psychological Implications Family decision 8

11 Slide 23 6 I found this slide hard to follow so I reworked it a little. REBECCA MCEACHERN, 2/20/2013 Slide 24 7 I found this slide hard to follow so I reworked it a little. REBECCA MCEACHERN, 2/20/2013

12 8 Treatment of Central Precocious Puberty Stop activation of axis or effect on bone age*: GnRH agonist Steroidgenesis inhibitor* Antiandrogen* Testolactone* Aromatase inhibitor* Delayed Puberty History/Physical/Lab/Imaging Normal Constitutional Delay Abormal Hypogonadism Primary Secondary GNRH LH/FSH + - Estradiol Testosterone 9

13 Slide 25 8 I found this slide hard to follow so I reworked it a little. REBECCA MCEACHERN, 2/20/2013

14 Secondary Hypogonadism (Hypogonadotropic Hypogonadism) Hypopituitarism Hypothyroidism (delays bone age) Kallman Syndrome Radiation Head trauma Functional gonadotropin deficiency CF Anorexia Chronic diseases Kallman Syndrome GNRH Agenesis of olfactory bulbs 50% have midline defect Incidence Males 1/10,000 Females 1/50,000 GNRH LH/FSH - + Estradiol Testosterone 10

15 Primary Hypogonadism (Hypergonadotropic Hypogonadism) (Gonadal Failure) Turner s Syndrome Gonadal dysgenesis Radiation / Chemotherapy Klinefelter Syndrome Vanishing Testes (congenital anorchia) Viral orchitis Clinical Evaluation Growth Assessment most important History Family history (pubertal onset, anosmia) Symptoms as described above esp.anosmia Review to evaluate hypopit symptoms Physical Thyroid exam Dysmorphic features (eg. Turner) Pubertal staging Fundoscopic examination Biochemical Evaluation Pubertal hormones LH/FSH Testosterone/Estradiol DHEAS TSH Evaluation for chronic illness Pituitary function 11

Bone age MRI as needed Imaging GnRH Stimulation testing Can help differentiate between hypogonadism and constitutional delay Treatment of Hypogonadism Replacement Estrogen Add Provera Birth

16 Bone age MRI as needed Imaging GnRH Stimulation testing Can help differentiate between hypogonadism and constitutional delay Treatment of Hypogonadism Replacement Estrogen Add Provera Birth control Testosterone Injections Patches Gels Teaching Psychosocial Compliance Follow up 8 months old - Vaginal bleeding - Tanner 3 breasts - LH = 10 miu/ml - FSH = 4 miu/ml Central Precocious Puberty 12

17 - MRI = hypothalamic - hamartoma Barrow Neurological Institute Lupron started age 7 months and Histrelin implant placed age 4 8/12 Central Precocious Puberty JB 13

18 Hypogonadotropic Hypogonadism SB Constitutionally Early Puberty DJ PAH = 158 cm (5f2) Normal puberty CG 14

Endocrine Nursing Evaluation Education Process Disease Compliance Follow up Psychosocial issues Sick Day Management Makes A Difference References/Resources Bhatia, S., Neely, E., & Wilson, D.

19 Endocrine Nursing Evaluation Education Process Disease Compliance Follow up Psychosocial issues Sick Day Management Makes A Difference References/Resources Bhatia, S., Neely, E., & Wilson, D. (2002) Serum Lutiening Hormone Rises Within Minutes After Depot Leuprolide Injection: Implications for Monitoring Therapy. Pediatrics, 109 (2), e 30. Courtney, Jan. Clinical Handbook of Pediatric Endocrinology. Quality Medical Publishing Garibaldi, L., Aceto, T., Weber, C., & Pang, S. (1993) The Relationship between Luteinizing Hormone and Estradiol Secretion in Female Precocious Puberty: Evaluation by Sensitive Gonadotropin Assays and the Leuprolide Stimulation Test. Journal of Clinical Endocrinology and Metabolism, 76(4), Guyton, Arthur. Textbook of Medical Physiology. WB Saunders Co Lavin, Norman. Manual of Endocrinology and Metabolism. Lippincott Williams & Wilkins, 2002 Lifshitz, Fima. Pediatric Endocrinology. Marcel Dekker, Inc Moshang, T. Pediatric Endocrinology, The Requisites in Pediatrics, Elsevier Mosby, 2004 Pediatric Endocrine Nursing Society. Partners in Education Rieser, Patricia. Overview of the Endocrine System. Pediatric Endocrinology Nursing Society Resource Manual Styne, Dennis. Pediatric Endocrinology. Lippincott, Williams, & Wilkins Weber, C, Behm, K; Pediatric Endocrine Provocative Testing, Pediatric Endocrinology Nursing Society Resource Manual, References/Resources cont. Shulman, Dorothy, et al. Adrenal Insufficiency: Still a Cause of Morbidity & Death in Children. Pediatrics Styne, Dennis. Pediatric Endocrinology. Lippincott, Williams, & Wilkins Weber, C, Behm, K; Pediatric Endocrine Provocative Testing, Pediatric Endocrinology Nursing Society Resource Manual, Weber, C, Delaune, J., Endocrine 101, Workshop Pediatric Endocrine Nursing Society Conference, April 18-21,

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Laura Stewart, MD, FRCPC Clinical Associate Professor Division of Pediatric Endocrinology University of British Columbia

Precocious Puberty Laura Stewart, MD, FRCPC Clinical Associate Professor Division of Pediatric Endocrinology University of British Columbia Faculty Disclosure Faculty: Laura Stewart No relationships with