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1: Anaesthesist. 2005 Dec;54(12):1201-8.Click here to read Links

[Patients with ornithine transcarbamylase deficiency. Anaesthesiological and intensive care management]

[Article in German]

Schmidt J, Schroth M, Irouschek A, Birkholz T, Kurzai M, Kröber S, Meisner M, Albrecht S.

Klinik für Anästhesiologie, FAU, Erlangen-Nürnberg, Krankenhausstrasse 12, 91054 Erlangen. joachim.schmidt@gmx.ch

Ornithine transcarbamylase deficiency (OTCD) is the most common inborn urea cycle disorder. Patients with OTCD are at risk of acute metabolic decompensation with hyperammonemia and subsequent encephalopathy, coma and death. Symptoms may be triggered by infections, drugs and stress, evoked by trauma, pain, fear, surgery and anaesthesia or by episodes of protein catabolism, i.e. fasting-induced, post partum or during gastrointestinal bleeding. Several specific considerations must be made for anaesthetic and intensive care management in patients with this disease in order to avoid metabolic decompensation. We report the intensive care management of the first manifestation of late-onset OTCD in a 16-year-old girl and a course of inconspicuous general anaesthesia with midazolam, s-ketamine, fentanyl and isoflurane in a 22-year-old girl with known OTCD.

PMID: 16136341 [PubMed - indexed for MEDLINE]

Patient Drug Information

  • Midazolam (available generically)

    Midazolam is given to children before medical procedures or before anesthesia for surgery to cause drowsiness, relieve anxiety, and prevent any memory of the event. Midazolam is in a class of medications called benzodiaz...

  • Source: AHFS Consumer Medication Information