Acute intermittent porphyria as a cause of maternal mortality (case report)

Автор: Kornilova T.Yu., Tadzhieva V.D., Iglina M.A., Semenova N.L., Vasilchev A.V., Savina I.E.

Журнал: Ульяновский медико-биологический журнал @medbio-ulsu

Рубрика: Клиническая медицина

Статья в выпуске: 4, 2017 года.

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Many clinicians, who determine the state of medicine in the country, are not aware of the fact that a number of diseases, that were considered incurable not long ago, are curable today. And one of such diseases, which hematologists deal with, is acute intermittent porphyria (AIP). Case report of maternal mortality caused by AIP was carried out in order that not only a hematologist, but also any clinician, could diagnose the patient timely and correctly. The authors show the chronology of the detection and setting the clinical diagnosis under concomitant multiple pregnancies, which interfere with the clinical performance. Analysing the case report, the authors conclude that it is very difficult to set a right diagnosis under pregnancy and the ambiguity of objective data, and, thus, to prevent fatality.

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Heme synthesis, porphyria, porphyrinogens, porphobilinogen, acute intermittent porphyria, heme arginate, polyneuropathy

Короткий адрес: https://sciup.org/14113324

IDR: 14113324   |   DOI: 10.23648/UMBJ.2017.28.8744

Статья научная