Paediatric Endocrine Emergencies

Gavin Burgessthanks 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

• The girl is on the track team, trying out for nationals

Case 1 cont.

• Nausea, abdominal pain, fatigue

• Looks thin, as does whole family

• No family history of significance

Case 1 cont.

• P 120, BP 110/70, R30, sats 96%

• Moderately dehydrated

• Normal LOC

Case 1 cont.

• What labs do you want?

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

Case 1 cont.

• Definition of DKA

Case 1 cont.

• pH <7.25

• HCO3 <15

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

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

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

Case 1 cont.

• Monitoring:

• alternating cap gas and lytes, for results q2h

Case 1 cont.

• Pitfalls:

• using subcutaneous insulin to treat DKA

• cerebral oedema - risk factors?

Case 1 cont.• Pitfalls:

• cerebral oedema

• Elevated BUN

• low PCO2

• Bicarb treatment

• Na fails to rise as GLC normalises

• <3y

• New diagnosis

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

Case 1 cont.

• Pitfalls:

• fasciitis - cases associated with new presentation

• Attributing excercise/eating disorder to the cause of the symptoms

Case 1 cont.

• turn down insulin to 0.05u/kg/h when bicarb 15mmol/l

• PO intake from around 17-18mmol/l

• Diabetics with lows -

• may be on a pump!

• always check the TYPE of insulin (lentis vs R)

• OFTEN obtunded - don’t need CT scans

Case 2

• hours old male brought in as PHN thought he was jittery

Case 2 cont.

• mother had borderline GDM

• birthweight 4.1kg

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

Case 2 cont.

• What glc level would prompt you to draw critical labs?

• Is there an ideal time to draw the labs?

Case 2 cont.

• Glucose solutions and doses:

• infant: D10W 2-4ml/kg

• 1-8: D25W 2-4ml/kg

• older: D50W 1 ampule

Case 3

• red hair and peripheral eosinophilia?

Case 3

Case 3

• 2y male, son of paramedic, found unconscious at home

• rushed to ACH

• “dirty” hands

Case 3 cont.

• Labs:

• glc 2

• Na 129

• K 5.5

Case 3 cont.

• hydrocortisone 50-100mg iv (subsequent 50mg/m2)

• fluid resuscitation

• look for endocrine neon pink sheet

Case 3 cont.

• pigment with adrenal failure (vs central)

• stress dosing - don’t need mineralocorticoid replacement

Case 3 cont.

• what’s the commonest cause of adrenal failure?

Case 3 cont.

• iatrogenic esp. rheumatological conditions

Case 4

• 2 week male, lethargy, poor feeding, vomiting

Case 4 cont.

• always check genitalia

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

Case 4 cont.

• where’s the block?

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

Case 4 cont.

• labs show low Na, high K, glc frequently normal, mild acidosis

• fluid resuscitation

• mineralo (not acutely) + glucocorticoid replacement

Case 5

Case 5

• Joseph Heller

Case 5

• 2d girl with jittery spells, exaggerated startle, some posturing

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

Case 5 cont.• Catch 22

• congenital heart disease (conotruncal)

• abnormal face

• thymic hypoplasia

• cleft palate

• hypocalcaemia

• microdeletion of 22

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

Case 5 cont

• admit all tetany, seizures and cases of laryngospasm for work up

Case 6

• moans, groans, stones

Case 6 cont

• Orthopaedics call:

• fracture follow-up, 8yo girl Ca ionised 1.3

• “What should I do?”

Case 6 cont.

• investigations?

Case 6 cont.

• Ca ionised and total, ALP, albumin

• renal function

• UA, Ca:creatinine spot

• ECG - shortening of QT interval

Case 6 cont.

• malignancy

• renal

• immobilisation

• Vit D and A

Case 6 cont.

• ICU

• NS at 2x maintenance

• lasix

• bisphosphonates

Case 6 cont.

• EXTREMELY rare in paediatrics, arguably not an emergency as correction over hours

• hypervitaminosis D

• mild BP, mild Ca elevation, constipation

Case 6 cont.

• most frequently present with irritability, poor feeding, constipation

Case 7

Case 7 cont.

• 13 yo F headache, palpitations, sweating

Case 7 cont.

• the rule of 10.....

Case 7 cont.

• ∝-blockade

• same as for malignant hypertension

• UA for?

For completeness sake...

• Thyroid coma

• Thyroid storm

• no case reports

• DI/SIADH - more fluid/lytes problem