Pregnancy and labor problems in patients with acute porphyria

Pustovoĭt Ia.S.; Karpova I.V.; Shmakov R.G.; Makhinia S.A.

doi:https://doi.org/

Pustovoĭt Ia.S.

Gematologicheskiĭ nauchnyĭ tsentr Minzdrava Rossii

Karpova I.V.

Gematologicheskiĭ nauchnyĭ tsentr Minzdrava Rossii

Shmakov R.G.

Federal State Budget Institution «Research Center for Obstetrics, Gynecology and Perinatology» Ministry of Healthcare of the Russian Federation, Moscow, Russia

ORCID: 0000-0002-2206-1002

Makhinia S.A.

Gematologicheskiĭ nauchnyĭ tsentr Minzdrava Rossii

Pregnancy and labor problems in patients with acute porphyria

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Pustovoĭt Ia.S., Karpova I.V., Shmakov R.G., Makhinia S.A.

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Journal: Therapeutic Archive. 2013;85(10): 47‑55

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Pustovoĭt IaS, Karpova IV, Shmakov RG, Makhinia SA. Pregnancy and labor problems in patients with acute porphyria. Therapeutic Archive. 2013;85(10):47‑55. (In Russ.)

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Abstract:

Aim. To evaluate the impact of developing pregnancy on porphyrin metabolism in reproductive-aged women with acute porphyria (AP). Subjects and methods. The prospective clinical data of 33 pregnancies were analyzed in 28 patients with the established diagnosis of AP. The latter was verified by the quantitative analysis of 24-hour urinary porphyrin excretion and the diminished activity of the pathognomonic enzyme. Results. Each case was analyzed in detail according to different criteria. Poor prognostic factors for pregnancy are identified in AP. The used curation policy for pregnant patients is described. The pregnant women with occurring AP episodes are subdivided into clinical groups requiring different curation approaches. The scheme for the used working protocol is given. Conclusion. The accumulated experience with curating the patients with AP will be able to avoid the existing prohibitory practice, providing a way to develop a new quality of life in the patients' families.

Keywords:

porphyria

pregnancy

phorphobilinogen

Authors:

Pustovoĭt Ia.S.

Gematologicheskiĭ nauchnyĭ tsentr Minzdrava Rossii

Karpova I.V.

Gematologicheskiĭ nauchnyĭ tsentr Minzdrava Rossii

Shmakov R.G.

Federal State Budget Institution «Research Center for Obstetrics, Gynecology and Perinatology» Ministry of Healthcare of the Russian Federation, Moscow, Russia

ORCID: 0000-0002-2206-1002

Makhinia S.A.

Gematologicheskiĭ nauchnyĭ tsentr Minzdrava Rossii

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