Transcript Paediatric Endocrine Emergencies Gavin Burgess thanks Jonathan

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
• 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 (1020ml/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?
• Pitfalls:
Case 1 cont.
• 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