Treatment of Acute Intermittent Porphyria With Large Doses of Propranolol

Douer, Dan; Weinberger, Abraham; Pinkhas, J; Atsmon, A

doi:10.1001/jama.1978.03290080056028

Cited by 25 publications

(5 citation statements)

References 9 publications

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“…In our case, the very high doses and plasma levels used without the occurrence of hypoten sion, bradycardia or heart failure [as reported in 1 case of porphyria (3)] imply an extreme overstimulation of the adrenergic system during the attack. The decrease in the excretion of urinary porphyrins observed in our patient concomi tantly with propranolol administration may be related to its repression of ALA synthetase and is in accordance with previous clinical and experimental reports (6,9,16).…”

Section: Discussionsupporting

confidence: 93%

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Hematin and Propranolol in Acute Intermittent Porphyria

Brezis¹,

Ghanem²,

Weiler-Ravell³

et al. 1979

Eur Neurol

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The authors present a case of acute intermittent porphyria (AIP) in an almost fatal relapse with quadriplegia, bulbar paralysis and coma. Intravenous hematin produced an immediate arousal from coma and allowed a gradual resumption of bulbar and autonomic functions. Persistent tachycardia and hypertension necessitated huge doses of intravenous propranolol. Both hematin and propranolol administrations were followed by a remarkable decrease in urinary amino-levulinic acid and porphobilinogen excretion. Nevertheless, after the acute stage, the patient was left with a severe generalized muscle wasting. After 7 months of intensive physical therapy, complete recovery of all neuromuscular functions was achieved. The modern aspects of the management of AIP are presented; the efficacy and the limits of hematin and propranolol therapy are discussed.

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Section: Discussionsupporting

confidence: 93%

“…Propranolol has been shown to have benefi cial effects in a few attacks of porphyria variegata (1, 2, 6) and in AIP (9). It has been suggested that the mechanism may be through its partial repression of ALA synthetase induc tion as well as by its effect on membranes (16).…”

Section: Discussionmentioning

confidence: 99%

Hematin and Propranolol in Acute Intermittent Porphyria

Brezis¹,

Ghanem²,

Weiler-Ravell³

et al. 1979

Eur Neurol

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“…Propranolol (3 × 20 mg/day) is another possibility to treat symptoms of adrenergic hyperactivity in AIP, because it seems to decrease the induction of ALAS (6,15). Since attacks of AIP can be precipitated by hormonal factors, devastating the lives of young women suffering monthly premenstrual porphyric crisis, many treatment regimens were tried.…”

Section: European Journal Of Endocrinology (1999) 141mentioning

confidence: 99%

“…glucose administration (300-500 g/day) and/or the administration of haematin or haem arginate (165 mg/day) (4,5). Propranolol (3 × 20 mg/day) can be used to treat symptoms of adrenergic hyperactivity associated with AIP (6).…”

Section: Introductionmentioning

confidence: 99%

Premenstrual attacks of acute intermittent porphyria: hormonal and metabolic aspects - a case report

Block

Leeuw

Gaal

1999

European Journal of Endocrinology

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We report the case of a 38-year-old woman with acute intermittent porphyria (AIP). Following the observation of an acute AIP attack in the patient's father, the diagnosis was established after genetic and biochemical examinations. At the age of 29, eight months after delivery of her first and only child, the patient was hospitalized due to a first proven attack of AIP. In the following years she suffered several premenstrual AIP attacks, with clinical symptoms ranging from abdominal pain to paralysis. One attack was accompanied by an increased urinary catecholamine output, strongly indicating adrenergic hyperactivity. The precipitation of acute episodes by secretion of gonadotrophins and a severe hyponatraemia due to a syndrome of inappropriate anti-diuretic hormone secretion indicated hypothalamic involvement in the pathogenesis of AIP. This patient has experienced an evolution of treatment regimens. At first, acute attacks were treated by i.v. hypertonic glucose. Afterwards propranolol was instituted as a maintenance therapy. Later on, i.v. injections of haem arginate were very successful in resolving acute AIP episodes. However, until therapy with an LHRH analogue was started, the patient continued to suffer premenstrual AIP attacks. These LHRH analogues cause hypothalamic inhibition of gonadotrophin secretion, with stabilization of endogenous ovarian steroid production at a low level, and therefore may be effective in preventing acute exacerbations of this disease. Since this patient went on a fixed regimen of an LHRH analogue combined with the lowest dose oestrogen patch her quality of life has improved substantially and she has not required hospitalization, now for over 3 years.

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“…propranolol. Propranolol in high doses (284 mg intravenously during a period of 18 hours and after that 300 to 950 mg/day orally) has been reported to effect a clinical remission associated with a decline in urinary excretion of porphyrin precursors in an acute attack of intermittent porphyria (Douer et al 1978).…”

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confidence: 99%

Barbiturate and Ethanol Sleeping Times and Pharmacokinetics of Propranolol in Mice after Intravenous Administration of Haem Arginate

Tokola¹

1987

Pharmacology & Toxicology

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Haem arginate is a new haem compound recently introduced for treatment of porphyrias. Previously haematin has been reported to increase certain hydroxylase activities in extrahepatic tissues, but even in therapeutic doses it impairs the microsomal foreign substance metabolism in the liver. Haem arginate at a dose equivalent to haem 10 mg/kg (threefold therapeutic dose) did not prolong the hexobarbital sleeping time of mice, 20 mg/kg did prolong the hexobarbital and possibly also the ethanol sleeping time. Haem arginate administered in high doses prior to oral propranolol did not alter the bioavailability of the latter. With regard to drug interactions haem arginate may be safer than haematin.

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Treatment of Acute Intermittent Porphyria With Large Doses of Propranolol

Cited by 25 publications

References 9 publications

Hematin and Propranolol in Acute Intermittent Porphyria

Hematin and Propranolol in Acute Intermittent Porphyria

Premenstrual attacks of acute intermittent porphyria: hormonal and metabolic aspects - a case report

Barbiturate and Ethanol Sleeping Times and Pharmacokinetics of Propranolol in Mice after Intravenous Administration of Haem Arginate

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