Paeds - Endocrine

Question 1

Early onset Type 2 diabetes

Answer 1

< 40 y/o

Has more complications

Question 2

MODY

Answer 2

Typically affects every generation (single gene autosomal dominant)

Doesn’t necessarily need insulin

Question 3

Diagnosis of T1DM

Answer 3

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

Question 4

T2DM RFx in kids

Answer 4

• FEMALE
• Non-white ethnicity
• Obesity
• Deprivation

Question 5

T2DM presentation in kids

Answer 5

More insidious

Many have acanthosis nigricans (outward sign of insulin resistance)

can present with DKA

Question 6

T2DM Dx

Answer 6

Usually OGTT
- HbA1c

Question 7

T2DM common comorb

Answer 7

HTN
Kidney disease
OBESITY

Question 8

Insulin management in T1DM

Answer 8

• 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

Question 9

How much should blood glucose be tested + which other times

Answer 9

At least 5 times a day

pre-meals, pre bedtime, exercise, feeling unwell and post-prandial

Question 10

Diff ways to test blood glucose

Answer 10

• Finger prick test
• Continuous Glucose Monitoring System
• Flash Glucose Monitoring System

Question 11

Advantages of finger prick test

Answer 11

accurate + no time lag

Question 12

Advantages of Flash Glucose Monitoring System

Answer 12

Glucose trends, alarms for highs and lows, ‘follow’ facility for carers and teachers, can communicate with pump delivery systems, less trauma to fingers

Question 13

DKA severity

Answer 13

• 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

Question 14

DKA Mx

Answer 14

• FLuids
• Insluin
• Hourly glucose monitoring
• 2-4 hourly Electrolytes (K+ and ketones)
• Hourly strict fluid balance
• Hourly neuro obs

Question 15

Complications of DKA

Answer 15

Cerebral oedema
shock
hypokaelaemia
aspiration
thrombus (viscous)

Question 16

Calculating total fluid requirement

Answer 16

• 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

Question 17

T2DM Tx

Answer 17

DIET + LIFESTYLE (10% weight loss in pre-pubertal child can reverse diabetes)

Metformin +/- insulin

Consider GLP-1 agonists:
- Liraglutide >10 years
- Semaglutide >18 years

Question 18

Glucose targets for T2DM

Answer 18

Pre-meal: 4-7 mmol/l
Pre-bedtime: 5-8
Post-prandial: <10
Average: <7

HbA1c <48 mmol/mol

Question 19

Calculating how much insulin to give

Answer 19

• 1 unit per 15g of carbs
• 1 unit brings blood glucose down by 8 (correction factor)

Question 20

Options for insulin therapy

Answer 20

• MDI
• Simple insulin pump
• Hybrid closed loop (pump + continuous glucose monitor)
  
  • automatically suspends if going hypo
• Closed loop (artificial pancreas)

Question 21

Complications of DM (further than the usual)

Answer 21

• Lipohypertrophy
• Diabetes burnout
• Biabulimia
• Self harm with insulin
• High carb loads + insulin therapy -> insulin resitance + high BMI
• increase prevelance of overweight + obesity

Question 22

Hypo management

Answer 22

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)

Question 23

Neonatal diabetes

Answer 23

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

Question 24

Difficulties of DM in toddlers

Answer 24

• Food – tend to graze, small meals little and often
• Behavioural issues – eating, injections, tests
• Hypos – may be difficut to recognise
• Hypos may affect neurodevelopment

Question 25

Challenges of DM Tx in kids

Answer 25

- 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

Question 26

Challenges of glycaemic control in adolescence

Answer 26

Poorest glycaemic control due to non-compliacne + risk-taking behaviour weight manipulation sex / drugs / alcohol Insulin resistance

Question 27

Diabetes care package

Answer 27

- 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

Question 28

Congenital adrenal hyperplasia

Answer 28

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)

Question 29

Congenital adrenal hyperplasia pathophys

Answer 29

- **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

Question 30

Congenital adrenal hyperplasia Px

Answer 30

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

Question 31

Congenital adrenal hyperplasia Mx

Answer 31

Specialist endocrinologists + monitor growth/development - Cortisol replacement - HYDROCORTISONE - Aldosterone replacement - FLUDROCORTISONE - female patients with virilised genitalia may require corrective surgery

Question 32

Androgen insenitivity

Answer 32

46 XY - X-LINKED RECESSIVE with normal chromosomal number

Question 33

Androgen insenitivity pathophys

Answer 33

- Defect in ANDROGEN RECEPTOR - END-ORGAN RESISTANCE to testosterone - Male genotype but female phenotype

Question 34

Androgen insensitivity Px

Answer 34

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?)

Question 35

Androgen insensitivity Mx

Answer 35

- 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