Acute intermittent porphyria and Caesarean delivery

Kantor, Gareth; Rolbin, Stephen H.

doi:10.1007/bf03008790

Cited by 31 publications

(10 citation statements)

References 35 publications

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“…Two alternatives, etomidate and ketamine, are also considered unsafe. These have now been almost entirely superseded by propofol and although animal studies suggest an increased risk, practical experience suggests that it is safe 31–34. Although propofol may also be used for maintenance, the risk of a crisis also relates to the total dose of drug given, and it may therefore be unwise to use it in this way.…”

Section: Autosomal Dominant Acute Porphyriasmentioning

confidence: 99%

Liver transplantation for porphyria: Who, when, and how?

et al. 2007

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Porphyrias are a heterogenous group of diseases that may result in disabling or life threatening neurovisceral symptoms and/or cutaneous photosensitivity. In acute intermittent porphyria, the clinical features, particularly neurological symptoms, may be life-threatening and disabling. Conventional treatment with human hemin, though effective in reducing symptoms, does not reverse neuropathy when structural nerve damage has occurred and may cause intense phlebitis. Liver transplantation (LT) may be considered as treatment for those with repeated life-threatening acute attacks resulting in poor quality of life, requirement of ventilatory support, and progressive loss of venous access due to hemin infusion. Patients with variegate porphyria (VP) present after puberty with neurovisceral symptoms and skin manifestations. LT resolved VP in the 1 patient reported in the literature. Aminolaevulinic acid dehydratase deficient porphyria is a rare autosomal recessive disorder and a child who presented with failure to thrive and required transfusions and parenteral nutrition did not improve with LT. In erythropoietic protoporphyria (EPP), there is excessive production of protoporphyrin in the bone marrow. Protoporphyrin is hepatotoxic and pigment loading of hepatocytes and bile canalicular sludging may result in progressive cholestasis and cirrhosis. LT is beneficial for such patients with end-stage liver disease. Perioperative management includes use of filters on operative lights to prevent skin burns and intestinal perforation. Other concerns include development of neuropathy, biliary complications, and recurrent liver disease. This review addresses the rationale, patient selection, evaluation, management issues, and technique of performing LT in various types of porphyria.

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Section: Autosomal Dominant Acute Porphyriasmentioning

confidence: 99%

Liver transplantation for porphyria: Who, when, and how?

et al. 2007

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“…However, the safe use of regional anesthesia has been reported in porphyria [6,7]. Carp and Clark have suggested that the use of regional anesthesia is not contraindicated as long as the possibility of autonomic dysfunction is considered [15].…”

Section: Discussionmentioning

confidence: 99%

“…Although a variety of drugs have been implicated in precipitating an acute attack [5], it is difficult to predict which drugs will be porphyrogenic. There have been several reports about the anesthetic management of patients with AIP [4,6,7], but the number of case reports about HCP is small [8,9]. We report the anesthetic management of a patient with HCP.…”

Section: Introductionmentioning

confidence: 89%

Safety of isoflurane and epidural anesthesia in a patient with hereditary coproporphyria

et al. 1996

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“…Pregnancy represents a notable risk factor in patients with AIP. The acute attacks associated with AIP have been reported as being more common during pregnancy (24-95%), particularly during the first trimester (4). AIP in pregnant women is also associated with higher rates of spontaneous abortion, hypertension, low birth weight and infant mortality (2-42%) (4,5).…”

Section: Introductionmentioning

confidence: 99%

“…The acute attacks associated with AIP have been reported as being more common during pregnancy (24-95%), particularly during the first trimester (4). AIP in pregnant women is also associated with higher rates of spontaneous abortion, hypertension, low birth weight and infant mortality (2-42%) (4,5). AIP may also trigger posterior reversible encephalopathy syndrome (PRES), a rare acute neurological condition that is characterized by acute neurological symptoms including seizures, visual abnormalities, headache, vomiting, nausea, impaired consciousness and focal neurologic deficits (6).…”

Section: Introductionmentioning

confidence: 99%

Treatment of acute intermittent porphyria during pregnancy and posterior reversible encephalopathy syndrome after delivery: A case report

Zhang

Zheng

et al. 2017

Exp Ther Med

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Abstract. Acute intermittent porphyria (AIP) is a rare inherited disorder of heme metabolism. It has the ability to trigger posterior reversible encephalopathy syndrome (PRES), a rare acute neurologic condition that is characterized by acute neurological symptoms. Pregnancy may induce AIP attacks. The present report describes the case of a pregnant woman with AIP. The patient was treated with heme-arginate during pregnancy and successfully delivered a healthy baby. Following delivery, the patient presented with PRES and experienced generalized seizures. Treatment including arginine hemoglobin, calcium gluconate and sodium chloride was administered. The symptoms of epilepsy did not recur. Magnetic resonance imaging of the head demonstrated that the bilateral occipital lobe lesions were significantly reduced in size following the treatment. In the present report, it was demonstrated that administration of heme-arginate for AIP during pregnancy is safe. Timely administration of arginine hemoglobin, calcium gluconate and sodium chloride may more efficiently improve the clinical status in AIP patients diagnosed with PRES who experience generalized seizures.

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Acute intermittent porphyria and Caesarean delivery

Cited by 31 publications

References 35 publications

Liver transplantation for porphyria: Who, when, and how?

Liver transplantation for porphyria: Who, when, and how?

Safety of isoflurane and epidural anesthesia in a patient with hereditary coproporphyria

Treatment of acute intermittent porphyria during pregnancy and posterior reversible encephalopathy syndrome after delivery: A case report

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