Paeds - Endocrine Flashcards
Early onset Type 2 diabetes
< 40 y/o
Has more complications
MODY
Typically affects every generation (single gene autosomal dominant)
Doesn’t necessarily need insulin
Diagnosis of T1DM
Point of care FINGER PRICK test if suspect
- if indicative -> SAME DAY REFERAL
Don’t wait for OGTT or HbA1c - kids can decompensate very fast and typically tend to present acutely
T2DM RFx in kids
- FEMALE
- Non-white ethnicity
- Obesity
- Deprivation
T2DM presentation in kids
More insidious
Many have acanthosis nigricans (outward sign of insulin resistance)
can present with DKA
T2DM Dx
Usually OGTT
- HbA1c
T2DM common comorb
HTN
Kidney disease
OBESITY
Insulin management in T1DM
- Commence on 0.5-0.8 Units/kg/day
- 50% long acting; 50% rapid acting for meals (10% breakfast, 20% lunch, 20% tea)
- Pen therapy usually initial
- pump therapy if indicated /appropriate
- Fixed doses to start with and then carb counted meals with a set insulin:carbohydrate ratio (ICR)
- Insulin sensitivity factor for correction doses (ISF), based on total daily dose of insulin and 100 rule
How much should blood glucose be tested + which other times
At least 5 times a day
pre-meals, pre bedtime, exercise, feeling unwell and post-prandial
Diff ways to test blood glucose
- Finger prick test
- Continuous Glucose Monitoring System
- Flash Glucose Monitoring System
Advantages of finger prick test
accurate + no time lag
Advantages of Flash Glucose Monitoring System
Glucose trends, alarms for highs and lows, ‘follow’ facility for carers and teachers, can communicate with pump delivery systems, less trauma to fingers
DKA severity
- MILD – pH < 7.3 or plasma bicarbonate <15 mmol/l
- MODERATE – pH <7.2 or plasma bicarbonate < 10 mmol/l
- SEVERE – pH < 7.1 or plasma bicarbonate < 5 mmol/l
One to one nursing or HDU if under 2 years or severe DKA
DKA Mx
- FLuids
- Insluin
- Hourly glucose monitoring
- 2-4 hourly Electrolytes (K+ and ketones)
- Hourly strict fluid balance
- Hourly neuro obs
Complications of DKA
Cerebral oedema
shock
hypokaelaemia
aspiration
thrombus (viscous)
Calculating total fluid requirement
- If clinically dehydrated but not in shock: give IV 10ml/kg saline over 30 mins
- SUBTRACT this bolus from total fluid deficit
- If shocked give 20 ml/kg
- DON’T SUBTRACT from fluid deficit
T2DM Tx
DIET + LIFESTYLE (10% weight loss in pre-pubertal child can reverse diabetes)
Metformin +/- insulin
Consider GLP-1 agonists:
- Liraglutide >10 years
- Semaglutide >18 years
Glucose targets for T2DM
Pre-meal: 4-7 mmol/l
Pre-bedtime: 5-8
Post-prandial: <10
Average: <7
HbA1c <48 mmol/mol
Calculating how much insulin to give
- 1 unit per 15g of carbs
- 1 unit brings blood glucose down by 8 (correction factor)
Options for insulin therapy
- MDI
- Simple insulin pump
- Hybrid closed loop (pump + continuous glucose monitor)
- automatically suspends if going hypo
- Closed loop (artificial pancreas)
Complications of DM (further than the usual)
- Lipohypertrophy
- Diabetes burnout
- Biabulimia
- Self harm with insulin
- High carb loads + insulin therapy -> insulin resitance + high BMI
- increase prevelance of overweight + obesity
Hypo management
Mild/management:
- glucose tablets, gells, or very fast acting food/drink e.g. lucozade
- only take prescribed dose
- Check glucose in 15 mins
- Follow up with long acting carbs
Severe:
Glucose injection (IM)
Neonatal diabetes
Rare. If transient, oft reoccurs later in life
Very sensitive to insulin
Frequent small milk feeds
Practical problems of testing and injections
Risks of hypoglycaemia vs risk of longstanding diabetes
Difficulties of DM in toddlers
- Food – tend to graze, small meals little and often
- Behavioural issues – eating, injections, tests
- Hypos – may be difficut to recognise
- Hypos may affect neurodevelopment
Challenges of DM Tx in kids
- Food – making own choices, school dinners
- Effect of exercise – PE, playground, after school activity, clubs, and team sport
- Behaviour and compliance
- Sleepovers
- School residentials and trips
Challenges of glycaemic control in adolescence
Poorest glycaemic control due to non-compliacne + risk-taking behaviour
weight manipulation
sex / drugs / alcohol
Insulin resistance
Diabetes care package
- Admit + therapy + education
- Home + school visits
- Remote reviews via cloud
- 3 monthly outpatient appointment
- Annual review; more frequent if needed
- coeliac and thyroid screening, injection sites for all. From 12 years, bp, ACR, lipids, retinopathy, psychological screening, dietetic review, foot examination
- age appropriate education
- admit to stabalise as required
Congenital adrenal hyperplasia
Congenital deficiency of 21-hydroxylase enzyme -> underproduction of cortisol + aldosterone and OVERPRODUCTION OF ANDROGENS from birth
- AUTOSOMAL RECESSIVE INHERITANCE
(sometimes caused by 11-beta-hydroxylase def)
Congenital adrenal hyperplasia pathophys
-
21-hydroxylase converts Progesterone into Aldosterone + Cortisol
- Progesterone can convert to testosterone WITHOUT 21-hydroylase - When 21-hydroxylase is defective -> Progesterone can’t be converted so:
- LOW aldoseterone + cortisol
- High excess progesterone which converts to EXCESS TESTOSTERONE
Congenital adrenal hyperplasia Px
Severe - present from birth:
- Virilised ‘ambigous’ genetalia + enlarged clitoris
-
Hyponatraemic hypokalaemia
+ Hypoglycaemia (because of low aldosterone + cortisol)
- Poor feeding
- Vomiting
- Dehydration
- Arrhythmias
Mild - present in childhood/puberty:
- Female:
- Tall for age
- Facial hair
- Absent periods
- Deep voice
- Early puberty
- Male:
- Tall for age; Deep voice
- Early puberty
- Large penis; small testicles
Can also get skin hyperpigmentation due to increased melanocyte stimulating hormone due to increased ACTH production to compensate for low cortisol
Congenital adrenal hyperplasia Mx
Specialist endocrinologists + monitor growth/development
- Cortisol replacement
- HYDROCORTISONE
- Aldosterone replacement
- FLUDROCORTISONE
- female patients with virilised genitalia may require corrective surgery
Androgen insenitivity
46 XY - X-LINKED RECESSIVE with normal chromosomal number
Androgen insenitivity pathophys
- Defect in ANDROGEN RECEPTOR
- END-ORGAN RESISTANCE to testosterone
- Male genotype but female phenotype
Androgen insensitivity Px
Range from complete to mild (female genetalia - normal male genitalia)
- Undermasculinization of external genitalia at birth
- Presence of rudimentary vagina / testes
- Elevated testosterone, oestrogen + LH on testing
Impaired virilization at puberty
- may have some FHx
Can also get some reduced virilization during puberty in genotypical females (carriers?)
Androgen insensitivity Mx
- Sex correction as newborn
- Oft gonadectomy after puberty if complete / mostly female phenotype
- Survaillence for complications (osteoporosis, gynacomastia)
- sometimes genetalia may develop differently post-nataly
- Genetic counselling / genetic testing
- Consider gender counselling / INFORM child at some point in a controlled environment
