Cimetidine in the Treatment of Acute Intermittent Porphyria

Baccino, É.

doi:10.1001/jama.1989.03430210038024

Cited by 17 publications

(5 citation statements)

References 4 publications

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“…In AIP, cimetidine may have decreased the urinary excretion of ALA and PBG transiently, but the pathological condition returned to the initial stage after this agent was withdrawn. 13,16 A family survey clarified the involvement of three EPP carriers among our patient's family members. They are all infants, and therefore follow-up is necessary.…”

Section: Discussionmentioning

confidence: 95%

“…Cimetidine, an H 2 -receptor antagonist, has recently been reported to ameliorate the clinical features of AIP and EPP by the inhibition of hepatic cytochrome P-450 synthesis and heme oxidase activity. [13][14][15][16] Yamamoto et al 14,15 have shown that oral cimetidine decreased the levels of erythrocyte PP and serum bilirubin in a patient with EPP. However, in that patient, these values returned to the initial levels after cimetidine was withdrawn, and the patient died of hepatic failure.…”

Section: Discussionmentioning

confidence: 99%

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Erythropoietic protoporphyria with severe liver dysfunction and acute pancreatitis

Komatsu

Shimosegawa

Uchi

et al. 2000

Journal of Gastroenterology

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A case of erythropoietic protoporphyria associated with severe hepatic dysfunction and acute pancreatitis is reported. The patient, a 33-year-old man, was admitted to our hospital complaining of upper abdominal pain, nausea, and vomiting of 3 days' duration. Laboratory tests on admission demonstrated liver dysfunction, anemia, and thrombocytopenia. On the third hospital day, the intensity of the upper abdominal pain increased, concomitantly with elevated levels of serum amylase. Ultrasonography and computed tomography scanning revealed a slightly enlarged pancreas. During this episode, he also complained of various neurological symptoms, including reduced mental alertness, weakness of extremities, constipation, profound sweating, and urinary retention. Porphyrin studies demonstrated markedly elevated erythrocyte and fecal protoporphyrin levels. Laparoscopic findings obtained after the attack subsided were compatible with porphyric liver cirrhosis. We therefore concluded that neurologic disorders and acute pancreatitis could develop in patients with erythropoietic protoporphyria with severe liver dysfunction.

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Section: Discussionmentioning

confidence: 95%

Section: Discussionmentioning

confidence: 99%

Erythropoietic protoporphyria with severe liver dysfunction and acute pancreatitis

Komatsu

Shimosegawa

Uchi

et al. 2000

Journal of Gastroenterology

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“…These findings were supported by subsequent cases of AIP successfully treated with cimetidine. 3,4 In addition, cimetidine has demonstrated clear benefit in the treatment of PCT. 6,7 In 1993, Yamamoto et al 8 reported reduction in the levels of erythrocyte protoporphyrin with the use of cimetidine in a 74-year-old woman with EPP.…”

Section: Discussionmentioning

confidence: 99%

Novel Treatment Using Cimetidine for Erythropoietic Protoporphyria in Children

Sheu

Teng

2016

JAMA Dermatol

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rythropoietic protoporphyria (EPP) is an inherited disease of heme biosynthesis resulting from a mutation in the ferrochelatase gene, leading to the accumulation of protoporphyrin. Erythropoietic protoporphyria manifests in early childhood as burning pain, edema, bullae, and erosions in response to sun exposure. 1 There have been reports that treatment with cimetidine, a histamine H 2 -receptor antagonist that inhibits δ-aminolevulinic acid synthase, results in symptomatic improvement in patients with acute intermittent porphyria (AIP) and porphyria cutanea tarda (PCT). 2-7 A single case report 8 also describes the use of cimetidine for the effective treatment of an adult patient with EPP. Herein we report the first case series, to our knowl-edge, describing the safe and effective use of cimetidine in 3 pediatric patients with EPP.

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“…(i) Cimetidine has well known inhibitory effects on hepatic P450 synthesis and this has been suggested for use in the therapy of acute intermittent porphyria. The drug has been shown to cause a 50% reduction in hepatic ALA‐S activity in an animal model 47 and the dose of 800 mg/day has been reported to result in diminished ALA excretion and amelioration of neurological symptoms in patients 48 . However, these studies were poorly controlled and larger studies are required to confirm the effectiveness of this treatment.…”

Section: Managementmentioning

confidence: 99%

The Acute Porphyrias

Lip

Mccoll

Moore

1993

Int J Clinical Practice

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SUMMARYThe porphyrias are a heterogeneous group of rare inborn errors of metabolism caused by inherited enzyme defects in the haem biosynthetic pathway, resulting in overproduction of porphyrins. The porphyrias can be distinguished biochemically but may be difficult to differentiate clinically. Considerable advances have been made in the understanding of the enzymology and molecular biology of the porphyrias. The acute attack of porphyria may be a life‐threatening condition, and an understanding of its many precipitating factors, clinical features and management is of importance in a disease with a significant mortality.

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Cimetidine in the Treatment of Acute Intermittent Porphyria

Cited by 17 publications

References 4 publications

Erythropoietic protoporphyria with severe liver dysfunction and acute pancreatitis

Erythropoietic protoporphyria with severe liver dysfunction and acute pancreatitis

Novel Treatment Using Cimetidine for Erythropoietic Protoporphyria in Children

The Acute Porphyrias

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