Safe use of propofol in a child with acute intermittent porphyria

Christian, A. S.

doi:10.1111/j.1365-2044.1991.tb09578.x

Cited by 11 publications

(2 citation statements)

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“…For example, the use of two common induction agents, namely, etomidate and thiopentone has been associated with exacerbation of the disease (3, 4). There have been a lot of published data on the safe use of propofol (5–8). However it has been shown that continuous infusion of propofol is associated with elevated urinary porphyrins (9).…”

Section: Discussionmentioning

confidence: 99%

Anesthesia in a child with homozygous porphobilinogen deaminase deficiency: a severe form of acute intermittent porphyria

Sheppard

Dorman

2005

Pediatric Anesthesia

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We report a case history of the anesthetic management of a child with a severe form of acute intermittent porphyria (AIP). AIP is an autosomal dominant condition with incomplete penetrance, caused by deficiency of porphobilinogen deaminase, an enzyme found in the synthetic pathway for heme. Anesthesia and surgery may present many precipitants for a potentially fatal acute porphyric attack. These include fasting, dehydration, stress, infection and drugs. Here, we describe the safe use of sevoflurane in the maintenance of anesthesia. Its relative insolubility and low metabolism suggest that sevoflurane may be a reasonable agent for anesthesia in the porphyric patient.

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Section: Discussionmentioning

confidence: 99%

Anesthesia in a child with homozygous porphobilinogen deaminase deficiency: a severe form of acute intermittent porphyria

Sheppard

Dorman

2005

Pediatric Anesthesia

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“…The literature contains several reports on the use of propofol in patients with acute hepatic porphyrias. With the exception of one small series of 13 patients from South Africa [23], these publications have usually appeared as single case reports [24,25] or as letters to the editor [26][27][28][29][30][31][32]. In general, propofol is considered to be a safe agent in acute porphyria.…”

Section: Discussionmentioning

confidence: 99%

Testing the porphyrinogenicity of propofol in a primed rat model

Böhrer

Schmidt

Martin

et al. 1995

British Journal of Anaesthesia

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We evaluated the porphyrinogenicity of propofol in a rat model. After a pilot study had been conducted to determine an optimal dose, 48 fasting male Sprague-Dawley rats were allocated randomly to six groups. The animals in groups 1-3 received saline i.p. In groups 4-6, the animals were given allylisopropylacetamide (AIA). Twelve hours later, animals in groups 1 and 4 received saline, groups 2 and 5 were given propofol 150 mg kg-1 i.p., followed by 75 mg kg-1 3 h later, and groups 3 and 6 received phenobarbitone 50 mg kg-1 i.p. and 25 mg kg-1 i.p. The animals were anaesthetized and killed 3 h after the second drug bolus and we measured the concentration of cytochrome P450, total porphyrin content and the activity of delta-aminolaevulinic acid synthase (ALAS) in the liver. Urinary delta-aminolaevulinic acid (ALA) and porphobilinogen (PBG) concentrations were measured. Analysis of variance and the t test with Bonferroni's correction were used to compare data. The hepatic cytochrome P450 concentration in the non-primed groups varied from 28.1 to 31.1 nmol g-1; administration of AIA decreased this to 20.1-20.9 nmol g-1. Total hepatic porphyrins were between 0.78 and 1.22 nmol g-1 in the non-primed groups and between 2.71 and 3.54 nmol g-1 in the AIA-primed groups. Hepatic ALAS activity was 29.2 and 35.5 nmol h-1 g-1 in groups 1 and 2. In the primed saline group, ALAS activity was measured at 134.5 nmol h-1 g-1.(ABSTRACT TRUNCATED AT 250 WORDS)

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Vor- und Nachteile der Narkoseeinleitung mit Propofol im Vergleich zu Barbituraten

Gauß

1993

Klinische Anästhesiologie Und Intensivtherapie

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Safe use of propofol in a child with acute intermittent porphyria

Cited by 11 publications

References 0 publications

Anesthesia in a child with homozygous porphobilinogen deaminase deficiency: a severe form of acute intermittent porphyria

Anesthesia in a child with homozygous porphobilinogen deaminase deficiency: a severe form of acute intermittent porphyria

Testing the porphyrinogenicity of propofol in a primed rat model

Vor- und Nachteile der Narkoseeinleitung mit Propofol im Vergleich zu Barbituraten

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