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Paediatric Endocrine Emergencies

Paediatric Endocrine Emergencies. Gavin Burgess thanks Jonathan Dawrant. Case 1. 7 y girl with vague flu-like illness for last week, low grade fever Some weight loss (clothes are looser), but mother has put family on “detox” program for 1 month

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Paediatric Endocrine Emergencies

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  1. Paediatric Endocrine Emergencies • Gavin Burgess • thanks Jonathan Dawrant

  2. Case 1 • 7 y girl with vague flu-like illness for last week, low grade fever • Some weight loss (clothes are looser), but mother has put family on “detox” program for 1 month • The girl is on the track team, trying out for nationals

  3. Case 1 cont. • Nausea, abdominal pain, fatigue • Looks thin, as does whole family • No family history of significance

  4. Case 1 cont. • P 120, BP 110/70, R30, sats 96% • Moderately dehydrated • Normal LOC

  5. Case 1 cont. • What labs do you want?

  6. Case 1 cont. • CBC: Hb 140, plt 400, WCC 14, L shift • Lytes: Na 137, K 4.5, Cl 100, BUN 7, Creat 50, glc 30 • Gas: 7.29/40/50/12/-10 • UA ketones 3+, clear

  7. Case 1 cont. • Definition of DKA

  8. Case 1 cont. • pH <7.25 • HCO3 <15

  9. Case 1 cont. • Management • replace with NS, if hypovolaemic (10-20ml/kg). Trend towards no routine bolus @ ACH • No evidence for NS vs 0.45NS as fluid thereafter • replace losses no more than 2x maintenance over next 48h

  10. Case 1 cont. • Management cont. • Add 40 mEq/l KCl+KPO4 (50:50) • insulin infusion: 25U in 250ml, run @ weight, remember to deduct this volume from the total maintenance fluid

  11. Case 1 cont. • Management cont.: • when glucose reaches 15mmol/l, start to add glucose (5%) to the maintenance, increasing the concentration. Do not adjust insulin rate

  12. Case 1 cont. • Monitoring: • alternating cap gas and lytes, for results q2h

  13. Case 1 cont. • Pitfalls: • using subcutaneous insulin to treat DKA • cerebral oedema - risk factors?

  14. Case 1 cont. • Pitfalls: • cerebral oedema • Elevated BUN • low PCO2 • Bicarb treatment • Na fails to rise as GLC normalises • <3y • New diagnosis

  15. Case 1 cont. • Signs of cerebral oedema.... start mannitol or 3% saline. • cerebral oedema has 60-80% mortality rate • accounts for >50% of hospital and 30% of home deaths

  16. Case 1 cont. • Pitfalls: • fasciitis - cases associated with new presentation • Attributing excercise/eating disorder to the cause of the symptoms

  17. Case 1 cont. • turn down insulin to 0.05u/kg/h when bicarb 15mmol/l • PO intake from around 17-18mmol/l

  18. Diabetics with lows - • may be on a pump! • always check the TYPE of insulin (lentis vs R) • OFTEN obtunded - don’t need CT scans

  19. Case 2 • hours old male brought in as PHN thought he was jittery

  20. Case 2 cont. • mother had borderline GDM • birthweight 4.1kg

  21. Case 2 cont. • Critical labs: • insulin • cortisol • growth hormone • repeat glucose, lactate • urine ketones - poor man’s 17OH butyrate • plasma AA, urine OA • SCM order sheet

  22. Case 2 cont. • What glc level would prompt you to draw critical labs? • Is there an ideal time to draw the labs?

  23. Case 2 cont. • Glucose solutions and doses: • infant: D10W 2-4ml/kg • 1-8: D25W 2-4ml/kg • older: D50W 1 ampule

  24. Case 3 • red hair and peripheral eosinophilia?

  25. Case 3

  26. Case 3 • 2y male, son of paramedic, found unconscious at home • rushed to ACH • “dirty” hands

  27. Case 3 cont. • Labs: • glc 2 • Na 129 • K 5.5

  28. Case 3 cont. • hydrocortisone 50-100mg iv (subsequent 50mg/m2) • fluid resuscitation • look for endocrine neon pink sheet

  29. Case 3 cont. • pigment with adrenal failure (vs central) • stress dosing - don’t need mineralocorticoid replacement

  30. Case 3 cont. • what’s the commonest cause of adrenal failure?

  31. Case 3 cont. • iatrogenic esp. rheumatological conditions

  32. Case 4 • 2 week male, lethargy, poor feeding, vomiting

  33. Case 4 cont. • always check genitalia

  34. Case 4 cont. • 21 hydroxylase deficiency, AR, 90% of cases • “shunt” of hormone down androgen pathway • salt wasting starts at birth • Enzyme levels take weeks to come back - but on Alberta screen • lack of aldosterone and cortisol

  35. Case 4 cont. • where’s the block?

  36. Case 4 cont. • girls have abnormal (but variable) external genitalia, normal internal genitalia • boys may have penile enlargement, but normal sized testes • boys often missed

  37. Case 4 cont. • labs show low Na, high K, glc frequently normal, mild acidosis • fluid resuscitation • mineralo (not acutely) + glucocorticoid replacement

  38. Case 5

  39. Case 5 • Joseph Heller

  40. Case 5 • 2d girl with jittery spells, exaggerated startle, some posturing

  41. Case 5 cont • Elongated face, almond-shaped eyes, long but wide nose, small nostrils, small and low-set ears, dark red rings under the eyes, open-mouthed expression, reduced movement and low muscle tone, small jaw, flat cheekbones

  42. Case 5 cont. • Catch 22 • congenital heart disease (conotruncal) • abnormal face • thymic hypoplasia • cleft palate • hypocalcaemia • microdeletion of 22

  43. Case 5 cont. • Treatment • 1ml/kg Ca gluconate • cardiac monitor • always check Mg, replace first • no more than 50mg/min: 10ml of 10% Ca glu = 90mg Ca • then add to iv 100mg/kg/24h. or PO

  44. Case 5 cont • admit all tetany, seizures and cases of laryngospasm for work up

  45. Case 6 • moans, groans, stones

  46. Case 6 cont • Orthopaedics call: • fracture follow-up, 8yo girl Ca ionised 1.3 • “What should I do?”

  47. Case 6 cont. • investigations?

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