PNAC in Neonate

[Christian French]

I would like to kindly ask your advice regarding a 42 days old baby in NICU who was born preterm 35/52. Direct Bilirubin reached 7.4. The baby is cholestatic which is most likely Parenteral Nutrition Associated Cholestiasis (PNAC). We were not successful in providing fish oil-based IVFE. The baby is still NPO as she is not tolerating feeds. The trace elements are available in combination as traces elements vial, but single elements are not available.
The baby had invasive candidiasis and is on AmBisome and is having Major Omphalocele which is not yet surgically repaired.
We started cycling the soybean based fat emulsion four days a week with three days off Fat emulsion.
We did not start Ursodioxycholic acid as the baby is NPO.

Any recommendations for the trace elements adjustment as Manganese and Copper are hepatotoxic.

[Christian Lancaster]

There are many ways suggested for minimizing or preventing PNAC like:
– Avoid NPO if possible: encourage hypocaloric EF
– Prevent infection of the CVC used for PN
– Cyclic PN
– Specialized Amino acid formula (Parenetral Pediatric amino acid solution containing Taurine)
-Oral Antibiotics: metronidazole , gentamicin
-Oral glutamine
-Phenobarbital
-Ursodeoxycholic acid
– New lipids generation (SMOF)
– Removal or adjusting manganese and copper
– Minimal phytosterol content in IV lipids
-Finally liver + SB transplant
I highly recommend to initiate ursodeoxycholic acid in this case. Also, as you tried lipids emulsions supported with fish oil, you may it effects after 7 to 8 weeks.
Also, you have to pay attention that the dose of lipids has to adjusted (0.5- 1gm/kg/day) even TG level is normal.

Manganese then copper are the most toxic trace elements. Regarding using the trace elements mixture in this case, you may provide full dose of trace elements twice a week. No trace element supplementations, NPO patients in particular, on long PN term, patient may develop anemia on lung run due to the lack of copper supplementation.

[Christian Lancaster]

I don’t have the reference clearly stated the dose in previous reply. The practice is either omitting or adjusting the dose and frequency providing of trace elements are variables among health care providers.
This is one option or you may provide 1/4th of the target dose and adjust the frequency if conjugated bilirubin become more worse..
There are many reports published pointed that bone disease and anemia have been reported in infant receiving a copper-free PN formulation in long term.

[Lillian Parker]

Despite a lack of evidence support omitting or reducing trace element in PNALD

· Check serum Copper levels prior to reducing or omitting trace elements dose to avoid producing a Copper deficiency, even if the direct serum bilirubin level is ≥2.0 mg/dL.

· Maintain an initial dose of 20 mcg/kg/d of copper in PN despite the development of cholestasis (majority of the pediatric trace element available in market contain 20 mcg/ml dose is 1 ml/kg), with monitoring of serum Copper levels .

· Manganese deficiency has not been reported; it may be relatively safe to discontinue Manganese in PN for a short period of time when cholestasis develops, despite a lack of evidence support for this practice.

· Obtain serum Copper and whole-blood Manganese levels at regular intervals (following 2 weeks of PN and monthly thereafter), regardless of bilirubin levels. This practice should decrease a patient’s chance of developing a Copper deficiency or Manganese toxicity while providing more appropriate, individualized parenteral doses.

· Since there is no sufficient data on the proper dose adjustment of trace elements, to decide whether to reduce or omit trace elements is a patient specific. If short PN expected then checking level will increase cost and with no major benefits. Therefore, majority of practitioner will reduce or omit trace elements.

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