Propionic Acidaemia with Negative Antenatal Testing - Clinical Protocol for Post-Natal Testing for Babies at Risk of
|Publication: 21/05/2013 --|
|Last review: 08/08/2019|
|Next review: 08/08/2021|
|Approved By: Trust Clinical Guidelines Group|
|Copyright© Leeds Teaching Hospitals NHS Trust 2019|
This Clinical Protocol is intended for use by healthcare professionals within Leeds unless otherwise stated.
Clinical Protocol for Post-Natal Testing for Babies at Risk of Propionic Acidaemia with Negative Antenatal Testing
In families with members affected by propionic acidaemia, antenatal testing should be routinely performed. This is usually in the way of chorionic villous sampling. There is a small chance a ‘negative’ result may be wrong, therefore post-natal examination and investigations are essential prior to the baby being discharged home.
All babies must remain in hospital for at least 48 hours.
1. Day of delivery
- Complete examination by a paediatrician (ST4 or above) & inform consultant on-call.
- If the baby is lethargic, jittery or tachypnoeic an urgent venous ammonia should be sent.
2. Between 24 and 48 hours of age
- Urine sample to biochemistry for organic acids
- Guthrie blood spot for acylcarnitine.
- (This is because there is a very small chance the CVS testing may be incorrect.)
3. After 48 hours of age if
- the baby remains clinically well,
- has been seen by an experienced paediatrician (ST4 or above) and
- the relevant urine and blood samples have been sent and results chased
- they may go home with appropriate advice & information leaflet (below). If at any point the baby becomes unwell, they need urgent review and management by a senior paediatrician.
- Ensure community midwife and GP are aware of baby’s discharge.
To provide a clear protocol for the management of babies born with a family history of propionic acidaemia and negative antenatal testing.
Inborn errors of metabolism
|Target patient group:||Babies who fulfil the above criteria|
|Target professional group(s):||Secondary Care Doctors
Secondary Care Nurses
C. Expert consensus.
Trust Clinical Guidelines Group
LHP version 1.0
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