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Endocrinopathies and DBA
Mandy WhiteheadChildren’s Endocrine Specialist Nurse
Leeds General Infirmary
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OverviewThe Endocrine System
Growth and growth hormone
Puberty
Adrenal glands and adrenal crisis
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The Endocrine System
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Feedback mechanism in the endocrine system
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ICP phases of growth
Thyroid Nutrition & Love
Oestrogen & Testosterone
Growth Hormone
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What is normal growth?
Growth is a luxury Accurate
measurementsSerial
measurementsGrowth chartsParental heights are
important
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Growth hormoneSecreted by the pituitary gland, pulsatile
fashion.GrowthMetabolic hormoneSugar and fat metabolismBody composition (lean/fat mass)Bone health? Cardiac muscle effect
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What factors may affect growth in DBA?Chronic illness
Regular blood transfusions
Steroid treatment
? Other factors
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Growth hormone deficiencySmall for parents
Falling away from centiles
Cherubic features
Mid facial hypoplasia
Excess adipose tissue
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PubertyRelease of GnRH
Pulsatile release of LH and FSH
Stimulates gonads to release sex hormones
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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
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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
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Adrenal InsufficiencyAdrenoCorticoTropic Hormone (ACTH)
deficiency (pituitary)
Feedback mechanism
High dose steroids
Replacement (hydrocortisone/prednisolone)
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What is an adrenal crisis?When the bodies physiological need for cortisol
outstrips its ability to produce it
Potentially fatal
Illness
Injury
Surgical procedure
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How to recognize an adrenal crisisHypoglycaemia
PallorSweatingIrritable/confusedNausea and/or vomitingSeizures
HypotensionLightheaded/dizzinessHeadacheConscious levelShock
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How to avoid it / treat itExtra steroids when unwell (double/treble)
Glucogel
Hydrocortisone injections
Education
Medic alerts
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Questions?