Paeds endocrine Flashcards
what viruses may trigger T1DM
Coxsackie B virus and enterovirus
Ideal blood sugar range
4.4 and 6.1 mmol/l
New diagnosis of T1DM blood tests?
FBC
U&E
Formal laboratory glucose
Blood cultures if ?infection
HbA1c
Thyroid function tests and thyroid peroxidase antibodies (TPO) to test for associated autoimmune thyroid disease
Tissue transglutaminase (anti-TTG) antibodies for associated coeliac disease
Insulin antibodies, anti-GAD antibodies and islet cell antibodies to test for antibodies associated with destruction of the pancreas and the development of type 1 diabetes
Management hypo in T1diabetes
- Rapid acting glucose such as lucozade
+ slower acting carbohydrates such as biscuits or toast to maintain the blood sugar level when the rapid acting glucose is used up
If severe:
IV dextrose and intramuscular glucagon
Long term complications of hyperglycaemia
- macrovascular: CAD, peripheral ischaemia, stroke, HTN
- microvascualar: Peripheral neuropathy, Retinopathy, Kidney disease, particularly glomerulosclerosis
- infection related : cadidiasis, UTI, pneumonia, skin adn soft tissue infections
Features DKA
Polyuria
Polydipsia
Dehydration
Nausea and vomiting
Weight loss
Acetone smell to their breath
Altered consciousness
Sepsis (may be underlying trigger)
Potassium imbalance –> arrythmias
Diagnosing DKA
Hyperglycaemia (i.e. blood glucose > 11 mmol/l)
Ketosis (i.e. blood ketones > 3 mmol/l)
Acidosis (i.e. pH < 7.3)
DKA management
- Correct dehydration over 48 hours (too quickly –> cerebral oedema)
- Prevent hypoglycaemia with IV dextrose once blood glucose falls below 14mmol/l.
- Add potassium to IV fluids and monitor serum potassium closely.
- Monitor glucose, ketones and pH to assess their progress and determine when to switch to subcutaneous insulin.
choice:
- “Use 0.9% sodium chloride with 20 mmol potassium chloride in 500 ml (40 mmol per litre) until
blood glucose levels are less than 14 mmol/l “
- You would also want to administer IV insulin, but only start this 1-2 hours after administering IV therapy. SC insulin (0.1units/kg/hr)
Reduced consciousness
Hypotension
Hypoglycaemia, hyponatraemia and hyperkalaemia
adrenal crisis (addisonian crisis)
Types of adrenal insufficiency
Primary: autoimmune destruction of adrenal glands –> global depletion in stuff produced by adrenal glands
Secondary: problem with pituitary gland not producing enough ACTH -> reduced stimulation of adrenal gland to produce cortisol
Tertiary: usually caused by long term steroid use (endogenous doesn’t kick in enough)
Pathophysiology addisons
Autoimmune destruction of adrenal gland –> low cortisol and low aldosterone
High ACTH
Low cortisol
Low aldosterone
High renin
Pathophysiology secondary adrenal insufficiency
Pituitary gland not producing adequate ACTH –> reduced stimualtion of adrenal glands to produce cortisol –> low cortisol
Low ACTH
Low cortisol
Normal aldosterone
Normal renin
Investigation addisons? how does it work
Short synacthen test (ACTH stimualtion test)
synthetic ACTH –> stimulate adrenal glands –> if it < doubles then priamry adrenal insufficiency
features of adrenal insufficiency
Lethargy
Vomiting
Poor feeding
Hypoglycaemia
Jaundice
Failure to thrive
Nausea and vomiting
Poor weight gain or weight loss
Reduced appetite (anorexia)
Abdominal pain
Muscle weakness or cramps
Developmental delay or poor academic performance
Bronze hyperpigmentation to skin in Addison’s caused by high ACTH level
Investigations adrenal insufficiency
U&Es (hyponatraemia and hyperkalaemia)
blood glucose (hypoglycaemia)
Short synctacten if ?addisons
Management adrenal insufficiency
Hydrocortisone is a glucocorticoid hormone used to replace cortisol.
Fludrocortisone is a mineralocorticoid hormone used to replace aldosterone if aldosterone is also insufficient.
Sick day rules adrenal insufficiency
may need to increase steroids (hydrocortisone)
Management adrenal crisis
Parenteral steroids (i.e. IV hydrocortisone)
IV fluid resuscitation
Correct hypoglycaemia
Careful monitoring of electrolytes and fluid balance
puberty stages girls
Boobs
Pubes
Grow
Flow
puberty stages boys
Grapes → penis
Drapes
Grow and deepening of voice
Blow
Normal age range for puberty girls and boys
8 – 14 in girls and 9 – 15 in boys
Define precocious puberty
‘development of secondary sexual characteristics before 8 years in females and 9 years in males’
thelarche
the first stage of breast development
adrenarche
onset of androgen-dependent body changes such as growth of axillary and pubic hair, body odor, and acne
Classifications of precocious puberty
- Gonadotrophin dependent (‘central’, ‘true’)
due to premature activation of the hypothalamic-pituitary-gonadal axis
FSH & LH raised
- CNS tumour
- neurofibromatosis type 1 - Gonadotrophin independent (‘pseudo’, ‘false’)
due to excess sex hormones
FSH & LH low
- adrenal tumour/hyperplasia
- McCune albright syndrome
pubarche
first pubic hair
What is McCune-Albright syndrome? features?
McCune-Albright syndrome is not inherited, it is due to a random, somatic mutation in the GNAS gene.
Features
precocious puberty
cafe-au-lait spots
polyostotic fibrous dysplasia
short stature
Causes of delayed puberty?
Hypogonadotrophic
- Damage to pituitary/hypothalamus
- Growth hormone deficiency
- Hypothyroidism
- Hyperprolactinaemia
- serious chronic conditions
- excessive exercise
- Kallman syndrome
Hypogonadism
- Previous damage to the gonads
- Congenital absence of the testes or ovaries
- Kleinfelter’s Syndrome (XXY)
- Turner’s Syndrome (XO)
What is kallman syndrome
Kallman syndrome is a inherited genetic condition causing hypogonadotrophic hypogonadism, resulting in failure to start puberty. It is associated with a reduced or absent sense of smell (anosmia)
threshold for initiating investigations for delayed puberty
No evidence of pubertal changes in a girl aged 13 or a boy aged 14
or girl 15 with secondary but no period
Presentation growth hormone deficiency
Neonates:
Micropenis
Hypoglycaemia
Severe jaundice
Older children
Poor growth, usually stopping or severely slowing from age 2-3
Short stature
Slow development of movement and strength
Delayed puberty
pharmacological name growth hormone
somatropin
What is the growth hormone stimualtion test
Measuring effect of medications that normally stimulate the release of growth hormone. Examples of these medications include glucagon, insulin, arginine and clonidine.
Growth hormone levels are monitored regularly for 2-4 hours after administering the medication to assess the hormonal response. In growth hormone deficiency there will be a poor response to stimulation.
Presentation congenital hypothyroid
tested for on blood spot
Prolonged neonatal jaundice
Poor feeding
Constipation
Increased sleeping
Reduced activity
Slow growth and development
Types of diabetes insipidus and pathophysiology
Cranial
Nephrogenic
Water deprivation test
- avoid fluid for 8 hours
- measure urine osmolarity
- give synthetic ADH
- 8 hours later measure osmolarity again
after dep after ADH
Cranial low high
Nephrogenic low low
Priamry high high
Management cranial DI
desmopressin
Management nephrogenic DI
thiazides, low salt/protein diet
Invetsigation DI
water deprivation test
Bicarbonate level in DKA?
low as ketones are using it up
Fluid specifics DKA
IV fluids (0.9% NaCl 10ml/kg) + SC insulin (0.1units/kg/hr)
creatinine level DKA
mildly raised as dehydrated
How is fluid resus different if shocked or not shocked?
If shocked: do NOT subtract bolus from rest
If not shocked: subtract bolus later
DKA severity
pH < 7.1 severe DKA (10% dehydration)
pH <7.2 moderate DKA (5% dehydration)
pH<7.3 mild DKA not dehydrated
normal range bicarbonate
22-29 mEq/L
normal blood pH
7.35 to 7.45, with the average at 7.40
What to do if you suspect T1 diabetes but not DKA
suspect if random blood glucose > 11 mmol
refer for immediate same day assessment
Management cerebral oedema DKA
- Hypertonic saline (2.7% or 3% saline. 2.5-5ml/kg over 10-15min)
or
- Mannitol (20% 0.5-1g/kg over 10-15min)
what is conns syndrome?
hyperaldosteronism (high aldosterone)
either due to primary or secondary
