Download Re-feeding in Juvenile Dermatomyositis

Case Presentation
Presenting history
• AC, 12 year old female, referred to Outpatients
• Joint pain for last 18 months
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Ankles, knees, front of legs
Worse on exertion
swelling disappearing at the end of the day
• Early morning stiffness
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Hands and feet
Spreading over last few weeks to include ankles and knees
• Worsening handwriting
Further history
Rash on back of hands and knuckles for 4 months
Short of breath on exertion
Hair loss for 2 weeks
Weight loss (possibly 3-4 kg) and difficultly with
physical activity for 4 months
Examination Findings
• Pallor
• Scaly rash on nose, elbows and knees and dorsum of great
toes, Ulcerating in some area
• Papules on DIPs, PIPs, MCPs
• Painful restriction of movement in ankles, internal rotation of
hips, MCPs, DIPs
• Painful flexion of spine
• 3/6 power lower limb, 4/6 in shoulders
Differential diagnosis
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Probable Juvenile Dermatomyositis (JDM)
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Ulcerative rash
With possible lung and GI involvement
Delayed presentation-over 18 months
Exclude underlying malignancy / infection
Investigations
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CK normal
ALT 40
AST 77 ↑↑ (normal <50), LDH 1072↑
Phosphate 1.36, Ca 2.20
ESR- 54, CRP <1
dsDNA Normal, RF negative
ANA positive 1 in 100
IgG, IgA, IgM normal, Anti RO / LA Normal
C3 1.08, C4 0.17 (normal)
Virology all normal
Further investigations
 Bone marrow normal
 Echo / ECG normal
 Bronchoscopy normal
 HRCT chest –possible some interstitial lung disease
Management
• 2 groups of 3 IV methylprednisolone pulses
• Maintenance steroids given IV also
• Plan 6 monthly doses cyclophosphamide
• Immediate improvement in condition
• Appetite improved, as well as eating
But….
• Returned with ALT 167 , Phos 0.69 
• ?cause low Phosphate – Refeeding syndrome
• Started phosphate supplements
• Urgent dietetic review arranged
Dietary review
• Weight noted 2nd centile
• Height 50th centile
• BMI 13.3
• Weight for Height 74%
• Regular electrolytes suggested
• Slowly titrate up polymeric diet against results
The refeeding
syndrome
Background
• First described in the 1st century
• After World War II, Schnitker documented that
following liberation, 21% of chronically starved
Japanese prisoners died despite the provision of
‘‘adequate diet’’ that included vitamin
supplementation tarvati
• Starting to eat again after prolonged starvation
seemed to precipitate heart failure
• Pathophysiology better described by Keys in 1950
(study participants were starved for 6 months and
then fed!)
Pathophysiology
• In prolonged starvation (weeks to months), glycogen stores
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are expended while proteins are conserved
Switch to Ketone bodies from fatty acids as main energy
source
This results in an intracellular loss of electrolytes, in particular
phosphate (despite possibly normal serum concentrations)
Insulin is suppressed , glucagon is increased
When they start to feed a sudden shift from fat to
carbohydrate metabolism occurs and secretion of insulin
increases.
This stimulates cellular uptake of electrolytes which are
already depleted
Increase in basal metabolic rate
Clinical features
• Importantly, the early clinical features of refeeding
syndrome(RFS) are non-specific and may go
unrecognised.
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rhabdomyolysis,
leucocyte dysfunction,
respiratory failure,
cardiac failure,
hypotension,
arrhythmias,
seizures,
coma,
sudden death
• It has been reported to occur with many conditions,
but never with JDM.
Dealing with RFS
• Understanding amongst general physicians and surgeons is
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limited.
Many patients at risk of refeeding syndrome are not treated
on specialist nutrition units.
Measurement of electrolytes may not be done and when
done, a trend may not be noticed, but simply absolute values
noted.
The other barrier is a lack of consensus on treatment.
Despite many single cases reports and discussion papers on
refeeding syndrome, general paucity of guidance, particularly
in paediatrics
An RCT is currently being started at GOS looking at RFS in
children, hypothesising that the speed of introduction of
nutrition will cause adverse events (Graeme O’connor)
Identifying those at risk
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Rapid weight loss
Number of days without nutrition
Weight for height <75% of ideal body weight (4SD)
Extreme risk wt for ht <70%
Dehydration
Bradycardia <45bpm
QTc Prolongation
Management
• Start feeds at 5-25kcal/kg per day
• Monitor
• QTc, BP / temp / pulse, PO4 (1st to drop), Mg2+, K+,
Glucose, Wt gain
• Oral thiamine 200mg bd – to facilitate carbohydrate metabolism
Progress in AC
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ALT improved
2 more episodes of dropping phosphate over next
week, as well as 1 episode of low potassium
Normal diet within 10 days
Did well with cyclophosphamide
Recurrence of symptoms
Calcinosis in neck
On infliximab infusions
Take home message
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Refeeding syndrome is possible in any child
who has had nutritional depletion, regardless
of the cause.
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Initial bloods can be completely normal and so
vigilance is required, as well as appropriate
screening and action to protect high risk
patients