[On the classification of porphyria: congenital porphyria and late cutaneous porphyria].
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Among women with acute intermittent porphyria (AIP) a small group exists whose clinical symptoms are precipitated by menses or pregnancy. In three such cases studied, the cyclical use of the oral progestational agents was apparently successful in preventing the appearance of symptoms. The individual administration of androgen, estrogen, and a progestin preparation to one patient showed the same protective effect. This suggests that inhibition of gonadotropin secretion, with stabilization of endogenous ovarian steroid production at a low level, may be the effective mechanism. Use of the progestins is occasionally associated with signs and symptoms of hepatotoxicity. Such side effects constitute the main limitation in the use of these drugs. Untoward effects can be minimized by the use of alternative preparations and lower dosage.
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Journal Article Acute intermittent porphyria: A non-surgical cause of abdominal pain Get access C W Pattison, C W Pattison East Birmingham Hospital Correspondence to: C. W. Pattison, 54 High Point, Richmond Hill Road, Edgbaston, Birmingham Search for other works by this author on: Oxford Academic Google Scholar I G Haynes I G Haynes East Birmingham Hospital Search for other works by this author on: Oxford Academic Google Scholar British Journal of Surgery, Volume 69, Issue 9, September 1982, Page 553, https://doi.org/10.1002/bjs.1800690919 Published: 07 December 2005 Article history Accepted: 24 March 1982 Published: 07 December 2005
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Porphyria is a constitutional state marked by the excretion of porphyrins, either in greater than physiologic amounts or of kinds not normally demonstrable. Clinically it presents two widely different pictures: the acute, with dynamic ileus and peripheral neuritis, and the congenital, with cutaneous manifestations. Since Linser1in 1906 first recognized the repeated simultaneous occurrence of porphyria and hydroa aestivale, considerable attention has been accorded this association. The facts that hematoporphyrin was then thought to be the porphyrin excreted in this disease and that it was soon shown to have remarkable photosensitizing powers2seemed to offer a ready explanation of the coexistence of the two conditions. It has been only recently that doubts of the validity of the early explanation have drawn attention to the fact that other dermatoses may be found with porphyria. The present study concerns such an association. CHEMICAL CONSIDERATIONS Porphyrins are widely distributed,
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Intravenous infusions of hematin in a young woman with acute porphyria in profound relapse was followed within 48 hours by remission of symptoms and rapid recovery. From a state of severe central and peripheral nervous-system involvement, the patient recovered so completely that she was able to leave the hospital in less than a month, with only a residual weakness of her arms. Serial studies of serum and urinary levels of porphyrin precursors and serum level of hematin provided highly important information about the effect of hematin on acute porphyria. (JAMA235:520-522, 1976)
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SummaryCase 1 In 1981, at the age of 22, this patient had an uncomplicated spontaneous abortion at 10 weeks. In 1982 she had a first trimester legal abortion because of severe abdominal colic, thought to be caused by kidney disease. Postoperatively she developed tetraparesis and respiratory paralysis, requiring assisted ventilation. Acute intermittent porphyria was diagnosed.
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