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?