PAEDIATRIC ENDOCRINOLOGY
ADDISON
T1DM DKA
(adrenal insufficiency)
Autoimmune destruction of pancreatic B cells leading Insulin deficiency – unable to utilise Adrenal glands do not produce sufficient steroid hormones (esp.
to insulin deficiency glucose switching to anaerobic cortisol and aldosterone)
Ø 25-50% of new T1DM present in DKA metabolism and fatty acid oxidation Autoimmune Addison
Primary
for ketones (most common
PP inadequate ACTH secondary to congenital
Secondary
hypoplasia of pituitary
inadequate CRH release due to long-term
steroid usage > 3 wks
Tertiary
® sudden withdrawal of steroids means
endogenous steroids not produced
• FHx of autoimmune diseases • T1DM Ø FHx of autoimmune disease
RF • Genetics • Infection Ø Long-term steroid usage (tertiary)
• Viral trigger – Coxsackie B and enterovirus
• Polyuria • Polyuria + polydipsia ® UWL Babies + Children Children specific
• Polydipsia • N/V + abdo pain Ø Lethargy, Ø Abdo pain
vomiting, Ø Muscle weakness and cramps
• FTT - UWL (due to severe dehydration) • Kussmaul’s breathing
Ø FTT - poor feeding, Ø Bronze hyperpigmentation
Sx • Secondary enuresis (bedwetting in previously • Sweet smelling acetone breath poor wt gain (elevated ACTH and MSH
dry child)
• Syncope – altered LOC Ø Hypoglycaemia stimulate melanocytes)
Ø Jaundice Ø Developmental delay + poor
academic performance
Ø Short-term Ø Ketoacidosis Addisonian (adrenal) crisis
o Hyperglycemia (DKA) Ø Severe dehydration Ø HyperK
o Hypoglycemia (XS exogenous insulin) – SA Ø Hyperkalaemia - arrythmias Ø HypoNa
Ø Hypoglycemia
+LA CHO ® IV 10% dextrose 2mg/kg Ø Cerebral oedema (rapid IV Ø HypoTN
bolus or IM glucagon bolus) – headache, altered
Comp.
o Nocturnal Hypoglycaemia – sweaty child behaviour, bradycardia
overnight ® alter insulin regime o Slow IV fluids
Ø Long-term – monitor microvascular (eye, o Mannitol
neuro, nephron) and macrovascular
complications. (PVD, IHD, CAD, Stroke, HTN) o IV 3% hypertonic
saline
Ø FBC, EUC • EUC- hyperkalaemia • FBC
Ø BSL • BSL – high > 11mM • EUC – check potassium
Ø Blood cultures (if fever present) • ABG – metabolic acidosis – pH < • BSL
7.3
• Aldosterone : renin ratio
Autoimmune screen • Blood ketones > 3mM
• Short-synacthen test (measure blood cortisol at baseline,
Ø C-peptide, proinsulin • Urine dipstick - ketones 30 and 60 mins)
Ix Ø Anti-GAD, Zn8 transporter, Islet cell antibodies o Failure of cortisol to rise (> 2x baseline) = Addison
Ø TFT - anti-TPO (autoimmune) • Cortisol + ACTH levels (measure before steroid
administration)
Ø Anti-TTG - coeliac
Cortisol ACTH Aldo Renin
Addison Low High Low High
2nd Low Low Normal Normal
Ø MDT approach – education + reassurance – Ø Refer to paediatricians ® ABCD Acute Mx of Addisonian crisis:
paediatric endocrinologist, diabetic nurse Ø ICU monitoring
Ø IV access
educator, paediatrician, dietician, Ø IV hydrocortisone
ophthalmologist, podiatrist o IVF – 0.9% NS infusion Ø IVF
over 48 hrs (NOT bolus) Ø Correct hypoBSL ® IV 10% dextrose
Ø Insulin dependent: (titrate accordingly)
to avoid cerebral oedema Ø Monitor and correct fluid and electrolyte
o SA – Actrapid
o IV insulin fixed rate
o LA – lantus Replacement steroids:
o IV dextrose if BSL < 14mM
o Insulin pump (dexcom) (prevent relative) Ø Fludrocortisone – mineralocorticoid (for aldo)
hypoglycaemia) Ø Hydrocortisone - glucocorticoid (for cortisol)
Ø Monitor CHO intake
Mx o IV K+ (if hypokalemia)
Ø Monitor BSL control Long-term
o HbA1C (every 3-6/12) Ø ID tag – steroid card
o Freestyle Libre (PBS) Ø Treat underlying cause Ø Educate “sick day rules” – increase steroid dosage during
acute illnesses to match normal steroid response
o ABx for sepsis
Ø Paediatric endocrinologist – monitor
o 3% hypertonic saline o Growth and development
for cerebral oedema o BP
o EUC
o BSL
o Bone profile
o Vitamin D
Other causes of hypoglycaemia
Ø Hypothyroidism
Ø Glycogen storage disorder
Ø GH deficiency
Ø Liver cirrhosis
Ø Alcohol
Ø Fatty acid oxidation defects (e.g. MCADD)
ADDISON
T1DM DKA
(adrenal insufficiency)
Autoimmune destruction of pancreatic B cells leading Insulin deficiency – unable to utilise Adrenal glands do not produce sufficient steroid hormones (esp.
to insulin deficiency glucose switching to anaerobic cortisol and aldosterone)
Ø 25-50% of new T1DM present in DKA metabolism and fatty acid oxidation Autoimmune Addison
Primary
for ketones (most common
PP inadequate ACTH secondary to congenital
Secondary
hypoplasia of pituitary
inadequate CRH release due to long-term
steroid usage > 3 wks
Tertiary
® sudden withdrawal of steroids means
endogenous steroids not produced
• FHx of autoimmune diseases • T1DM Ø FHx of autoimmune disease
RF • Genetics • Infection Ø Long-term steroid usage (tertiary)
• Viral trigger – Coxsackie B and enterovirus
• Polyuria • Polyuria + polydipsia ® UWL Babies + Children Children specific
• Polydipsia • N/V + abdo pain Ø Lethargy, Ø Abdo pain
vomiting, Ø Muscle weakness and cramps
• FTT - UWL (due to severe dehydration) • Kussmaul’s breathing
Ø FTT - poor feeding, Ø Bronze hyperpigmentation
Sx • Secondary enuresis (bedwetting in previously • Sweet smelling acetone breath poor wt gain (elevated ACTH and MSH
dry child)
• Syncope – altered LOC Ø Hypoglycaemia stimulate melanocytes)
Ø Jaundice Ø Developmental delay + poor
academic performance
Ø Short-term Ø Ketoacidosis Addisonian (adrenal) crisis
o Hyperglycemia (DKA) Ø Severe dehydration Ø HyperK
o Hypoglycemia (XS exogenous insulin) – SA Ø Hyperkalaemia - arrythmias Ø HypoNa
Ø Hypoglycemia
+LA CHO ® IV 10% dextrose 2mg/kg Ø Cerebral oedema (rapid IV Ø HypoTN
bolus or IM glucagon bolus) – headache, altered
Comp.
o Nocturnal Hypoglycaemia – sweaty child behaviour, bradycardia
overnight ® alter insulin regime o Slow IV fluids
Ø Long-term – monitor microvascular (eye, o Mannitol
neuro, nephron) and macrovascular
complications. (PVD, IHD, CAD, Stroke, HTN) o IV 3% hypertonic
saline
Ø FBC, EUC • EUC- hyperkalaemia • FBC
Ø BSL • BSL – high > 11mM • EUC – check potassium
Ø Blood cultures (if fever present) • ABG – metabolic acidosis – pH < • BSL
7.3
• Aldosterone : renin ratio
Autoimmune screen • Blood ketones > 3mM
• Short-synacthen test (measure blood cortisol at baseline,
Ø C-peptide, proinsulin • Urine dipstick - ketones 30 and 60 mins)
Ix Ø Anti-GAD, Zn8 transporter, Islet cell antibodies o Failure of cortisol to rise (> 2x baseline) = Addison
Ø TFT - anti-TPO (autoimmune) • Cortisol + ACTH levels (measure before steroid
administration)
Ø Anti-TTG - coeliac
Cortisol ACTH Aldo Renin
Addison Low High Low High
2nd Low Low Normal Normal
Ø MDT approach – education + reassurance – Ø Refer to paediatricians ® ABCD Acute Mx of Addisonian crisis:
paediatric endocrinologist, diabetic nurse Ø ICU monitoring
Ø IV access
educator, paediatrician, dietician, Ø IV hydrocortisone
ophthalmologist, podiatrist o IVF – 0.9% NS infusion Ø IVF
over 48 hrs (NOT bolus) Ø Correct hypoBSL ® IV 10% dextrose
Ø Insulin dependent: (titrate accordingly)
to avoid cerebral oedema Ø Monitor and correct fluid and electrolyte
o SA – Actrapid
o IV insulin fixed rate
o LA – lantus Replacement steroids:
o IV dextrose if BSL < 14mM
o Insulin pump (dexcom) (prevent relative) Ø Fludrocortisone – mineralocorticoid (for aldo)
hypoglycaemia) Ø Hydrocortisone - glucocorticoid (for cortisol)
Ø Monitor CHO intake
Mx o IV K+ (if hypokalemia)
Ø Monitor BSL control Long-term
o HbA1C (every 3-6/12) Ø ID tag – steroid card
o Freestyle Libre (PBS) Ø Treat underlying cause Ø Educate “sick day rules” – increase steroid dosage during
acute illnesses to match normal steroid response
o ABx for sepsis
Ø Paediatric endocrinologist – monitor
o 3% hypertonic saline o Growth and development
for cerebral oedema o BP
o EUC
o BSL
o Bone profile
o Vitamin D
Other causes of hypoglycaemia
Ø Hypothyroidism
Ø Glycogen storage disorder
Ø GH deficiency
Ø Liver cirrhosis
Ø Alcohol
Ø Fatty acid oxidation defects (e.g. MCADD)
