Cimetidine and Acute Intermittent Porphyria

Cherem, José Halabe; Malagon, Jose; Nellen, Haiko

doi:10.7326/0003-4819-143-9-200511010-00023

Cited by 16 publications

(12 citation statements)

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“…The patient participated in the present study had been given oral Cimetidine 800 mg daily, 7 days ahead of the menstruation cycle and thereafter. The acute attack did not happen in her menstrual period, indicating the prophylaxis effect on her acute attacks [12,13]. More clinical data is required to verify whether the routine application of Cimetidine can be replaced with Hematin as preventive medicine.…”

Section: Discussionmentioning

confidence: 99%

“…Although Cimetidine has obvious effects on AIP acute attack, the interval between each attack seems not affected. Cherem et al [12] documented the case history of 4 patients managed with the oral administration of Cimetidine 800 mg daily for 1-year and the followup showed only 1 attack episode in some patients while the others did not experience any attack. This could demonstrate the promising efficacy of this drug in preventing the attack.…”

Section: Discussionmentioning

confidence: 99%

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Cimetidine in the Treatment of Acute Intermittent Porphyria from Onset to Death

Xie¹,

Yan²

2015

J Blood Disord Transfus

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Acute intermittent porphyria (AIP) is a rare, frequently misdiagnosed disease characterized by abdominal pain and psychiatric symptoms. Abdominal pain is the most common. At present, there is no radical cure for AIP. Hematin is considering the effective treatment to relieve the symptom. However, it is not available in china. A 25-year-old Chinese woman complained about intermittent abdominal pain for 2 years. He was misdiagnosed as acute abdominal disease many times. During attacks, abdominal pain relief always took more than 1 week without effective treatment. In our hospital, the patient was diagnosed AIP definitely and treated with intravenous cimetidine 400mg Q6h, symptoms especially abdomen pain relieved within 48-72 hours completely in each acute attack. Few cases of cimetidine to treat AIP have been reported. More clinical date is required in order to verify whether the routine treatment of cimetidine can replace hematin or be used as a preventive treatment. This is the first case to report that cimetidine has obvious effect during AIP acute attacks.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Cimetidine in the Treatment of Acute Intermittent Porphyria from Onset to Death

Xie¹,

Yan²

2015

J Blood Disord Transfus

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“…En caso de ataques cíclicos estos pueden prevenirse con la administración semanal o quincenal de hemina 14 . También se ha propuesto el tratamiento con cimetidina, como alternativa en los casos en los que no se dispone de hemina de forma inmediata 15 ; en nuestro caso, este tratamiento no se demostró eficaz con una progresión de la enfermedad hasta que fue posible la administración de hemina. Asimismo, en mujeres con incidencia elevada de crisis durante la fase luteínica de ciclo menstrual se ha descrito la eficacia de la utilización de la hormona liberadora de la hormona luteinizante durante los primeros días del ciclo para evitar las crisis 16 .…”

Section: Discussionunclassified

La porfiria aguda intermitente, un problema diagnóstico

Sistere

Mavila

Ribelles

et al. 2010

Gastroenterología y Hepatología

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“…Its use seems to have been predicated upon earlier reports that cimetidine inhibits certain isoforms of cytochrome P-450 and ameliorated acute porphyria in rodent models and in scattered case reports in humans with acute intermittent porphyria 21–24. On the basis of the different sources of excess heme precursors in acute hepatic porphyrias (liver) vs in EPP/XLPP (bone marrow), and on the lack of demonstrable effects of cimetidine on FECH or ALAS2 activities, it seems doubtful that cimetidine would benefit patients with EPP or XLPP.…”

Section: Overview Of Management Optionsmentioning

confidence: 99%

Advances in the management of erythropoietic protoporphyria – role of afamelanotide

Lane¹,

McKay²,

Bonkovsky³

2016

TACG

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Erythropoietic protoporphyria (EPP) and the phenotypically similar disease X-linked protoporphyria (XLPP) are inherited cutaneous porphyrias characterized clinically by acute non-blistering photosensitivity, intolerance to sunlight, and significantly reduced quality of life. They are due to marked overproduction of protoporphyrin (PP) chiefly by erythroblasts and reticulocytes. In EPP, the underlying genetic defect is in the ferrochelatase gene, which encodes the final enzyme in the heme synthetic pathway. In XLPP, the genetic defect is a gain-of-function mutation, usually a four-base deletion, in the gene that encodes the enzyme 5-aminolevulinic acid synthase-2, the first and rate-controlling enzyme of heme synthesis in developing red blood cells. The excess PP causes acute and painful photosensitivity, being activated by light in the long ultraviolet to blue spectrum (380–420 nm, the Soret band). Although several treatments have been proposed, presently no very effective treatment exists for EPP or XLPP. Afamelanotide (Scenesse®) is a first-in-class synthetic analog of α-melanocyte stimulating hormone. Afamelanotide mimics the naturally occurring hormone to increase skin pigmentation by increasing melanin production in melanocytes, resulting in increased sunlight tolerance in those with EPP/XLPP. Afamelanotide is currently approved for use in the European Union and Switzerland, and it is under review in the United States by the Food and Drug Administration for use in patients with EPP/XLPP. This paper provides a review of the clinical characteristics and current therapies for EPP/XLPP. We discuss the pharmacology, clinical efficacy, safety, and tolerability of afamelanotide and summarize the results of several key Phase II and III clinical trials. These data indicate that afamelanotide is a promising therapy for those with these debilitating diseases.

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Cimetidine and Acute Intermittent Porphyria

Cited by 16 publications

References 3 publications

Cimetidine in the Treatment of Acute Intermittent Porphyria from Onset to Death

Cimetidine in the Treatment of Acute Intermittent Porphyria from Onset to Death

La porfiria aguda intermitente, un problema diagnóstico

Advances in the management of erythropoietic protoporphyria – role of afamelanotide

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Cimetidine and Acute Intermittent Porphyria

Cited by 16 publications

References 3 publications

Cimetidine in the Treatment of Acute Intermittent Porphyria from Onset to Death

Cimetidine in the Treatment of Acute Intermittent Porphyria from Onset to Death

La porfiria aguda intermitente, un problema diagnóstico

Advances in the management of erythropoietic protoporphyria &ndash; role of afamelanotide

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Advances in the management of erythropoietic protoporphyria – role of afamelanotide