Endocrinopathies and DBA
Mandy WhiteheadChildren’s Endocrine Specialist Nurse
Leeds General Infirmary
OverviewThe 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 Nutrition & Love
Oestrogen & Testosterone
Growth Hormone
What is normal growth?
Growth is a luxury Accurate
measurementsSerial
measurementsGrowth chartsParental heights are
important
Growth hormoneSecreted by the pituitary gland, pulsatile
fashion.GrowthMetabolic hormoneSugar and fat metabolismBody 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 deficiencySmall for parents
Falling away from centiles
Cherubic features
Mid facial hypoplasia
Excess adipose tissue
PubertyRelease 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 glandsSit on top of each kidneyEssential for lifeExtremely complexProduce cortisol Help to maintain blood
sugar and blood pressure
Regulate salt balanceSex hormones,
testosterone and oestradiol
Adrenal InsufficiencyAdrenoCorticoTropic 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 crisisHypoglycaemia
PallorSweatingIrritable/confusedNausea and/or vomitingSeizures
HypotensionLightheaded/dizzinessHeadacheConscious levelShock
How to avoid it / treat itExtra steroids when unwell (double/treble)
Glucogel
Hydrocortisone injections
Education
Medic alerts
Questions?