Download Endocrinopathies and DBA - Diamond Blackfan Anaemia

Mandy Whitehead
Children’s Endocrine Specialist Nurse
Leeds General Infirmary
Overview
 The Endocrine System
 Growth and growth hormone
 Puberty
 Adrenal glands and adrenal crisis
The Endocrine System
Feedback mechanism in the
endocrine system
ICP phases of growth
Thyroid
Nutritio
n & Love
Growth
Hormone
Oestrogen &
Testosterone
What is normal growth?
 Growth is a luxury
 Accurate measurements
 Serial measurements
 Growth charts
 Parental heights are
important
Growth hormone
 Secreted by the pituitary gland, pulsatile fashion.
 Growth
 Metabolic hormone
 Sugar and fat metabolism
 Body composition (lean/fat mass)
 Bone health
 ? Cardiac muscle effect
What factors may affect growth in
DBA?
 Chronic illness
 Regular blood transfusions
 Steroid treatment
 ? Other factors
Growth hormone deficiency
 Small for parents
 Falling away from centiles
 Cherubic features
 Mid facial hypoplasia
 Excess adipose tissue
Puberty
 Release of GnRH
 Pulsatile release of LH
and FSH
 Stimulates gonads to
release sex hormones
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
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
Adrenal Insufficiency
 AdrenoCorticoTropic Hormone (ACTH) deficiency
(pituitary)
 Feedback mechanism
 High dose steroids
 Replacement (hydrocortisone/prednisolone)
What is an adrenal crisis?
 When the bodies physiological need for cortisol outstrips
its ability to produce it
 Potentially fatal
 Illness
 Injury
 Surgical procedure
How to recognize an adrenal crisis
 Hypoglycaemia
 Pallor
 Sweating
 Irritable/confused
 Nausea and/or vomiting
 Seizures
 Hypotension
 Lightheaded/dizziness
 Headache
 Conscious level
 Shock
How to avoid it / treat it
 Extra steroids when unwell (double/treble)
 Glucogel
 Hydrocortisone injections
 Education
 Medic alerts
Questions?