1. Endocrinopathies and DBA
Mandy Whitehead
Children’s Endocrine Specialist Nurse
Leeds General Infirmary

2. Overview The Endocrine System Growth and growth hormone Puberty
Adrenal glands and adrenal crisis

3. The Endocrine System

4. Feedback mechanism in the endocrine system

5. ICP phases of growth Growth Hormone Thyroid Nutrition & Love
Oestrogen & Testosterone
Growth Hormone
Thyroid Nutrition & Love

6. What is normal growth? Growth is a luxury Accurate measurements
Serial measurements
Growth charts
Parental heights are important

7. Growth hormone Secreted by the pituitary gland, pulsatile fashion.
Metabolic hormone
Sugar and fat metabolism
Body composition (lean/fat mass)
Bone health
? Cardiac muscle effect

8. What factors may affect growth in DBA?
Chronic illness
Regular blood transfusions
Steroid treatment
? Other factors

9. Growth hormone deficiency
Small for parents
Falling away from centiles
Cherubic features
Mid facial hypoplasia
Excess adipose tissue

10. Puberty Release of GnRH Pulsatile release of LH and FSH
Stimulates gonads to release sex hormones

11. What is delayed puberty?
Absence of development of secondary sexual characteristics by:
13yrs in girls (breast development)
14yrs in boys (testicular enlargement)
Pubertal arrest
Hypogonadotropic hypogonadism/delayed puberty
Treatment and fertility

12. Adrenal glands Sit on top of each kidney Essential for life
Extremely complex
Produce cortisol
Help to maintain blood sugar and blood pressure
Regulate salt balance
Sex hormones, testosterone and oestradiol

13. Adrenal Insufficiency
AdrenoCorticoTropic Hormone (ACTH) deficiency (pituitary)
Feedback mechanism
High dose steroids
Replacement (hydrocortisone/prednisolone)

14. What is an adrenal crisis?
When the bodies physiological need for cortisol outstrips its ability to produce it
Potentially fatal
Illness
Injury
Surgical procedure

15. How to recognize an adrenal crisis
Hypoglycaemia
Pallor
Sweating
Irritable/confused
Nausea and/or vomiting
Seizures
Hypotension
Lightheaded/dizziness
Headache
Conscious level
Shock

16. How to avoid it / treat it
Extra steroids when unwell (double/treble)
Glucogel
Hydrocortisone injections
Education
Medic alerts

17. Questions?