Paeds endo/Reproductive Flashcards
What is insulin and where is it produced ?
-Anabolic hormone produced by the beta cells in the islets of Langerhans. inthe pancreas
In what 2 ways does insulin reduced blood sugar ?
- Causes cells to absorb glucose
- Causes muscle and liver cells to absorb glucose and stored as glycogen
What is glucagon and where is it produced ?
-Catabolic hormone that increasedsblood glucose and is produced by alpha cells in the islets of langerhans of the pancreas
How does glucagon increase blood glucose ?
- Glycogenolysis : tells the liver to break glycogen to glucose
- Gluconeogenesis : tells the kiver to convert proteins and fats to glucose
What is ketogenesis and when does it occur
- The liver converts fatty acids to ketones
- Occurs when there is insufficient supply of glucose and glycogen stores are exhausted
How do children present with T1DM?
-DKA
OR
- Polyuria
- Polydipsia
- Weight loss
What are the two methods of insulin injection for T1DM
-Insulin pump
OR
-Long acting inuslin + short acting injected 30 mins before carb intake -> Basal-Bolus
How is severe hypoglycaemia treated ?
-IV dextrose + intramuscular glucagon
Give 4 macrovascular complications of hyperglycaemia
- Coronary artery disease
- Peripheral ischaemia
- Stoke
- HTN
Give 3 microvascular complications of hyperglycaemia
- Peripheral neuropathy
- Retinopathy
- Kidney disease, particularly glomerulosclerosis
What 3 problems arise in DKA ?
- Ketoacidosis
- Dehydation -> polyuria, polydipsi
- Potassium imbalance -> low total body potassium
How is DKA diagnosed ?
- Hyperglycaemia
- Ketosis
- Acidosis
- Raised creatinine kinase
Why should someone in DKA have their GCS closely monitored ?
- Risk of cerebral oedema
- The rapid correction of dehydration and hyperglycaemia causes water to shift from extracellular to intracellular space cause the brain to swell
- Tx : slow IV fluids, IV mannitol and IV hypertonic saline
How is DKA treated?
- Correct dehydration evenly over 48 hrs -> IV fluids (0/9% NaCl 10ml/kg)
- Get a fixed rate of insulin infusion : 0.1 units/kg/hr
- Prevent hypoglycaemia with IV dextrose once blood glucose is below 14mmol/l
- Add potassium and monitor
What is hypospadias?
- > Congenital abnormality : ventral urethral meatus, hooded prepuce and chordee (curvature) in more severe forms
- > management with surgery at 12 mnths, cicrumcision is CI
What is a cause of primary adrenal insufficiency ?
-Addison’s disease -> most commonly autoimmune and is damage to the adrenal glands resulting in reduced cortisol and aldosterone
What blood results are seen in primary adrenal failure ?
- Low cortisol
- Low aldosterone
- High ACTH
- High renin
What is an addisonian crisis ?
- Acute presentation of severe addisons
- Reduced consciousness, hypotension, hypoglycaemia, hyponatraemia and hyperkalaemia
How is an addisonian crisis managed ?
- IV hydrocortisone (100mg)
- IV fluid resus (1 litre saline over 30-60min)
- Correct hypoglycaemia (dextrose in fluid)
How is addisons disease managed ?
- Hydrocortisone to replace cortisol
- Fludrocortisone to replace aldosterone
- Sick day rules : increase steroid, monitor blood sugar and IM steroid if D&V
Explain the short synacthen test
- Synthetic ACTH is given
- In healthy adrenal glands, cortisol will increase
- Less than double the baseline rise in cortisol suggests addisons
Congenital adrenal hyperplasia : what, inheritance
- Congenital deficiency in 21-hydroxylase enzyme leading to underproduction of cortisol and aldosterone and overproduction of androgens from birth
- Autosomal recessive
What are the blood results in someone with CAH
- Low cortisol
- Low aldosterone
- High testosterone
- Raised ACTH
->21-hydroxylase is used to convert progesterone to cortisol and aldosterone, without it, the excess is converted to testosterone
How does severe CAH present ?
- At birth with ‘ambiguous genitalia’ and enlarged clitorus
- Shortly after birth : Hyponatraemia, hyperkalaemia and hypoglycaemia + metabolic acidosis = poor feeding, vomiting, dehydration and arrhythmias
How does milder CAH present in women ?
- Tall for age
- Facial hair
- Absent periods
- Deep voice
- Early puberty
How does milder CAH present in males ?
- Tall for age
- Deep voice
- Large penis
- Small testicles
- Early puberty
How does growth hormone deficiency present?
- Neonates : micropenis, hypoglycaemia, severe jaundice
- Older infants : poor growth, short stature, slow development of movement and strength and delayed puberty
How is growth hormone deficiency managed ?
-Daily subcutaneous injections of GH (somatropin)
If not picked up at birth, how can congenital hypothyroidism present ? (7)
- Prolonged neonatal jaundice
- Poor feeding
- Constipation
- Increased sleeping
- Reduced activity
- Slow growth and development
- Puffy face, macroglossia
What is the most common cause of hypothyroidism in children
- Autoimmune thyroiditis (Hashimoto’s)
- Anti-TPO antibodies
Give 4 complications of undescended testis
- Infertility
- Torsion
- Testicular cancer
- Psychological
when will a baby with undescended testis be referred and when with orchidopexy be carried out
- Unilateral : referral from 3-6 mnths, surgery at 1 yr
- Bilateral : review by paediatrician within 24 hrs
- Sudden onset severe testicular pain
- Referred to lower abdo
- N&V
- Swollen, tender testis
Testicular torsion
what are 3 signs of testicular torsion
- Testis retracted upwards
- Cremasteric reflex lost
- Elevation of testis doesn’t ease pain (Prehn’s sign)
what antibodies are seen in autoimmune thyroiditis
Antithyroid peroxidase (anti-TPO)
Antithyroglobulin
give 5 causes of obesity in children
- GH deficiency
- Hypothyroid
- Down’s
- Cushing’s
- Prader-Willi syndrome
what are the features of DKA
- Abdo pain
- Polyuria, polydipsia, dehydration
- Kussmal respiration
- Acetone smelling breath
what 3 abx are seen in T1DM
- IAA (90% of young children - insulin autoantibodies )
- Anti-GAD (glutamic acid antibodies)
- ICA (islet cell Antibodies)
when is T1DM diagnosed
- Symptoms +
- Fasting glucose >7.0mmol/l
- Random 11.1 or greater
What are the features of addison’s
- Lethargy, weaknes, anorexia, N&V, weight loss, ‘salt craving’
- Hyperpigmentation
- Loss of pubic hair in women
what bloods are seen in addisons
- Hypoglycaemia
- Hyponatraemia
- Hyperkalaemia
- Metabolic acidosis