Acute Intermittent Porphyria

McEwin, Roderick

doi:10.1111/j.1445-2197.1973.tb06811.x

Cited by 3 publications

(2 citation statements)

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“…Numerous cases have also been reported from Denmark (57), from other parts of Europe (3, 6, 1 1 , 12, 17), from the United States (29. 43, 53) and from Australia (32). The acute porphyric attack, common to all hereditary hepatic porphyrias, has been well documented in these reports.…”

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confidence: 83%

Hereditary Hepatic Porphyrias in Finland

Mustajoki

Koskelo

1976

Journal of Internal Medicine

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The occurrence of hepatic porphyrias --acute intermittent porphyria (AIP) and variegate porphyria (VP)-in Finland has been studied. During a period of 9 years 107 patients with AIP and 45 patients with VP were found. The prevalence of hereditary hepatic porphyrias was calculated to be 3.4per 100 OOO inhabitants. The patients belonged to 42 different families. Eighty-nine patients (59%) had had acute attacks, whereas 63 were symptomless latent cases. Precipitating factors, symptoms and excretion of porphyrins and their precursors did not significantly differ from what has been reported earlier from other parts of the world. A slight fragility of the skin on the back of the hands was noted in some 50% of VP patients. Abnormal sensitivity to sunlight could not be seen in a single case. However, about 50% of patients with VP showed an abnormal reaction when irradiated with artificial ultraviolet light. The difference in the skin symptoms in South African and Finnish VP patients is discussed.

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confidence: 83%

Hereditary Hepatic Porphyrias in Finland

Mustajoki

Koskelo

1976

Journal of Internal Medicine

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“…These attacks can often get complicated by neurologic manifestations like mental changes, convulsions, and peripheral neuropathies, which can progress to respiratory failure/paralysis and hyponatremia [4]. Respiratory paralysis is the most dreaded complication, with a mortality rate of 90%, and is most commonly triggered by drugs [5].…”

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confidence: 99%

Evaluating the Efficacy of a Small Interfering Ribonucleic Acid Molecule, Givosiran, in Treating Acute Intermittent Porphyria: A Systematic Review

Patel,

Midha,

Shukla

et al. 2023

Cureus

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Acute intermittent porphyria (AIP) is a severe multiorgan dysfunction disorder that can be fatal if not treated promptly. The newest treatment modality involving small interfering RNA (siRNA) molecules, givosiran, is administered for AIP. Although it has very beneficial effects in treating attacks of AIP, it comes with an extensive side effect profile that is not fully understood or studied. Hence, this novel drug model treatment's risk-benefit evaluation is still necessary. For relevant medical literature, we explored medical databases such as PubMed/Medline, PubMed Central, Cochrane Library, Internet Archive Scholar, Google Scholar, and Wiley Online Library. The selected papers were screened based on eligibility criteria and filtered through quality appraisal tools, and 13 finalized research papers were included in the study. Of the 13 identified papers, three were clinical trials, and 10 were review articles. The selected papers all discussed the effectiveness and side effects of givosiran in acute and recurrent attacks of AIP. The research papers showed decreased rates of acute attacks of AIP with givosiran and terminating recurrent attacks. But there are certain non-serious side effects, like fatigue and nausea. Also, there are some severe side effects, like pain. There is limited information on renal and liver function impairment using givosiran and the use of givosiran in patients with kidney and liver disease, for which further studies are required.

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The Inheritance of Porphyria Cutanea Tarda

McEwin¹

1973

Medical Journal of Australia

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Acute Intermittent Porphyria

Cited by 3 publications

References 23 publications

Hereditary Hepatic Porphyrias in Finland

Hereditary Hepatic Porphyrias in Finland

Evaluating the Efficacy of a Small Interfering Ribonucleic Acid Molecule, Givosiran, in Treating Acute Intermittent Porphyria: A Systematic Review

The Inheritance of Porphyria Cutanea Tarda

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