Transient 5-oxoprolinuria: unusually high anion gap acidosis in an infant.

Transient 5-oxoprolinuria is a phenomenon that is well recognised in adults. We illustrate an unusual paediatric case of transient 5-oxoprolinuria presenting during an episode of severe sepsis with concomitant paracetamol use. The 15-month-old patient had an extremely high anion gap metabolic acidosis. Adequate resuscitation failed to correct the biochemical disturbance, and high levels of 5-oxoproline were identified. A combination of haemofiltration, replenishment of glutathione stores with N-acetylcysteine and cessation of paracetamol administration resulted in the resolution of the acidosis. Subsequent testing following treatment of the sepsis revealed no ongoing 5-oxoprolinuria. CONCLUSION: Transient 5-oxoprolinuria has been previously reported in the adult population during episodes of severe sepsis and various pharmaceutical interventions. This case illustrates that it is a phenomenon that should be considered in paediatric patients where a very high anion gap metabolic acidosis exists that cannot be explained by the biochemical indices. WHAT IS KNOWN: • 5-oxoprolinuria in the paediatric population is usually secondary to an inborn error of metabolism. • Transient 5-oxoprolinuria is well recognised in adults during episodes of severe glutathione depletion. WHAT IS NEW: • Transient 5-oxoprolinuria is a phenomenon rarely reported in the paediatric population. • It highlights the importance of investigating a high anion gap such that unusual diagnoses are not missed.

Pediatric DNAR orders in the perioperative period.

Do not attempt resuscitation (DNAR) orders are a formal expression of the intention to refrain from resuscitation. Since their inception in 1974, such orders have become widely accepted within the hospital setting. However, their acceptance in theatres where anesthesia may cause cardiovascular instability, outcomes from cardiac arrest are improved and when there is a cross-over of techniques between anesthetic practice and resuscitation, has been more problematic. In order to ascertain the opinions on DNAR orders in the perioperative period, a questionnaire was sent to all consultant members of the Association of Paediatric Anaesthetists of Great Britain and Ireland, which asked about DNAR orders, resuscitation under anesthesia and included a number of case studies. Of the 280 respondents, 160 (57.1%) agreed that an anesthetist could alter the order in the perioperative period with 41 (14.6%) stating that they would always suspend such an order. Most anesthetists agreed that they would discuss DNAR orders during their preoperative assessment but could not agree as to which interventions constituted normal anesthetic practice as opposed to resuscitation. At present, there is variation in practice between pediatric anesthetists over suspension of DNAR orders in the perioperative period and no specific guidelines to refer to. We suggest that guidelines be produced and that these should take into account the work that has already taken place and guidelines published by other anesthetic communities.