Induction of anaesthesia with ketamine during an acute crisis of hereditary coproporphyria

Capouet, Vincent; Dernovoi, Boris; Azagra, Juan Santiago

doi:10.1007/bf03010140

Cited by 12 publications

(3 citation statements)

References 12 publications

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“…Although there are some reports showing ketamine's potentiality in triggering AIP crisis,[ 24 ] it might be safe because it has been used safely in quiescent AIP. [ 24 29 30 ] Succinylcholine has been used for many years and has been proven to be safe, as has tubocurarine. [ 31 ] Though both pancuronium and alcuronium have been classified as unsafe by some authors, they have been widely used by many anesthetists without harm.…”

Section: Discussionmentioning

confidence: 99%

Anesthetic implication of tricuspid valve replacement in a patient with acute intermittent porphyria

et al. 2016

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Facing a patient with acute intermittent porphyria (AIP), there is narrow safety margin which circumscribe all the therapeutic actions including choice of drugs. This would become even more complicated when it comes to a stressful and drug-dependent process like a cardiopulmonary bypass. According to author's researches, no specific AIP case of tricuspid valve (TV) replacement is reported recently. Furthermore, fast-track anesthesia was safely used in this 37-year-old male known the case of AIP, who was a candidate for TV replacement and removing the port catheter. The patient was extubated subsequently, only 3 h after entering the Intensive Care Unit.

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Section: Discussionmentioning

confidence: 99%

Anesthetic implication of tricuspid valve replacement in a patient with acute intermittent porphyria

et al. 2016

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“…Thiopental and etomidate are very powerful inductors of the ALA synthetase activity and should be avoided, even though some authors mention its use in latent porphyria 17. Ketamine is not porphyrogenic in experimental models, and its use seems permissible in asymptomatic porphyria 18. Sufentanil, as well as all opioids, can be administered without any problem in porphyric patients.…”

Section: Discussionmentioning

confidence: 99%

Recovery from a variegate porphyria by a liver transplantation

et al. 2004

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The porphyrias are a group of inherited or acquired enzymatic defects of heme biosynthesis. Each type of porphyria has a characteristic pattern of overproduction and accumulation of heme precursors based on the location of dysfunctional enzyme in the heme synthetic pathway. Variegate porphyria, one of the acute hepatic porphyrias, is characterized by a partial reduction in protoporphyrinogen oxidase, the seventh enzyme of the heme biosynthetic pathway. A case of liver transplantation is described with a recovery from a variegate porphyria. Acute porphyria is commonly worsened by a wide variety of medications. We describe a step-by-step perioperative management protocol. (Liver Transpl 2004;10:935-938.) P orphyrias ( Fig. 1) are a group of genetic diseases usually with autosomic dominant transmission. They are characterized by a disorder of the heme synthesis owing to a defect of an enzyme involved in its production and leading to an accumulation of heme's precursors, the porphyrins, in some tissues and in the excreted matter (urine and feces). 1 Eighty percent of the heme synthesis takes place in the bone marrow, whereas 20% is hepatic and is involved in the P 450 cytochrome synthesis. Enzymatic defects are always incomplete; an absolute deficiency would be incompatible with life. Two groups of porphyrias are described-hepatic and erythropoietic-depending on the tissue in which the metabolic disorder predominates. All hepatic porphyrias, except porphyria cutanea tarda, produce acute neuro-visceral manifestations such as abdominal pain, and neurologic and psychiatric disorders. 2 The disease results from an overproduction of porphyrins in the liver. The accumulation of some porphyrins, in porphyria cutanea tarda and protoporphyria, is then responsible for the chronic hepatic disease. We report our experience with a recovery from a variegate porphyria (VP) by a liver transplantation. Case PresentationA 46-year-old man underwent a liver transplantation for a Child-Turcotte-Pugh B alcohol induced cirrhosis. His history included chronic alcohol abuse, with no alcohol intake for 1 year, with 1 episode of decompensated ascites and 2 episodes of spontaneous bacterial infections of the ascites fluid. When the patient was eligible for liver transplantation, blood tests were as follows: prothrombin time, 37 %; total serum bilirubin; 206m/L; platelets; 100,000/mL; albumin; 33g/L. He also experienced a VP progressing for 20 years that was responsible for abdominal pain and cutaneous manifestations (bullous skin eruption upon exposure to sunlight during a trip to Spain). Following an acute attack two years ago, the laboratory tests showed an increase in porphobilinogen in the urine to 13.2 m/L (normal, Յ5 m/L); an increase in total porphyrin in the feces, dry weight 230 nm/g (normal, Յ 200nm/g), mainly involving the protoporphyrins without involving coproporphyrin and uroporphyrin; and a total porphyrin concentration in the plasma (spectrofluorometric dosage) reaching the upper normal limit. An evaluation of the lymphocyte pr...

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“…Therefore, propofol is considered safe in patients with porphyrias, during the asymptomatic and symptomatic periods. Etomidate and ketamine showed certain impact on porphyria crisis onset in animal models, however, in clinical practice, they are considered safe in therapeutic doses [45][46][47]. Still, one should be careful with repeated etomidate usage, since Harrison at al.…”

Section: Anesthetic Managementmentioning

confidence: 99%

Anesthetic implications in porphyrias

Randjelović¹,

Stojanović²,

Radeka³

et al. 2023

Front. Anesthesiol.

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The porphyrias are a group of disorders related to deficient heme biosynthesis, caused by malfunction of certain enzymes in the synthesis pathway. Erythropoietic porphyrias present with cutaneous symptoms and do not affect the nervous system. Hepatic porphyrias develop acute attacks with mild to severe neurovisceral symptoms, dramatic course, and rare, but possibly lethal outcomes. Anesthetic management of patients suffering from hepatic porphyria is challenging regarding the possibility of triggering or worsening the acute attack with medications that induce or maintain anesthesia. The medications are labeled as safe or unsafe according to laboratory experiments, clinical studies, case reports and experience. In this paper, we discuss underlying pathophysiology, presentation, therapy recommendations and anesthetic implications related to porphyrias.

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Induction of anaesthesia with ketamine during an acute crisis of hereditary coproporphyria

Cited by 12 publications

References 12 publications

Anesthetic implication of tricuspid valve replacement in a patient with acute intermittent porphyria

Anesthetic implication of tricuspid valve replacement in a patient with acute intermittent porphyria

Recovery from a variegate porphyria by a liver transplantation

Anesthetic implications in porphyrias

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