ACUTE HEPATIC PORPHYRY. NEW APPROACHES IN THE STUDY OF ACUTE HEPATIC PORFIRIIA IN THE REPUBLIC OF TATARSTAN

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Relevance. With AIP, there are practically no methods for diagnosing the "harbingers" of an acute attack and ways to alleviate the further course of an attack. The consequence of this is its low and late detection. Therefore, in AIP, there is a difficulty in predicting and planning the need for drugs for their relief. Hence, during the “undiagnosed” initial phase of an AIP attack, the patient does not receive medications to help alleviate the course of an attack or prevent it. In such cases, apparently, it is advisable to prescribe a hepatoprotector and a sorbent. With their prophylactic use, in turn, it would be possible to achieve a decrease in the frequency of exacerbation of AP attacks, and alleviate the course of the disease. Theoretically, the sorbent can bind and remove toxic products (ALA and PBG) from the body. In this regard, the search for and use of drugs that help prevent or ease the course of an acute attack of porphyria is very important. Target. Search for methods for diagnosing the "harbingers" of an acute attack and increasing the effectiveness of therapy in patients with an orphan disease: acute hepatic porphyria. Materials and methods. The domestic and foreign literature on orphan diseases related to AIP was analyzed. A search was made for patients with AIP in the Republic of Tatarstan. Results. An analysis of the literature shows that the frequency of spontaneous exacerbations of porphyria has a large variability from several months to several years, which is associated with a significant deterioration in the quality of life of patients. In the Republic of Tatarstan for 2023 8 patients with AIP were registered, which is 0.2 per 100,000 of the population of the Republic of Tatarstan. Their age varies from 20 to 63 years. Women predominate among patients (ratio W:M = 7:1). 5 patients live in Kazan, 3 - from regions of the Republic of Tatarstan. From the anamnesis, many patients noted from childhood such complaints as abdominal pain, for which they were hospitalized many times and diagnosed with intestinal colic. Due to the deterioration of the condition against the background of triggers, after the diagnosis of AIP was verified at the SRC RAS, patients with frequent recurrences of AIP repeatedly received inpatient and outpatient treatment (hemin, glucose, UDCA). A hypothesis was put forward and trends in the development of the theory of evaluating the effectiveness of treatment in patients with acute porphyria and the development of highly sensitive signs - "harbingers" of an attack of acute porphyria and managing patients before the attack period, during an attack and after its relief were identified. Conclusions. Achieving success in the management and treatment of patients with AIP is the search for "harbingers" of an acute attack of the disease and the appointment of pharmacotherapeutic agents to prevent attacks and alleviate their course.

About the authors

Ekaterina Viktorovna Salyakhova

Email: kareglazka_1@mail.ru
ORCID iD: 0000-0001-5043-7401
SPIN-code: 2988-0835
Russian Federation, 36, Butlerova str.,Republic of Tatarstan, Kazan, 420012, Russia

Rafik Galimzyanovich Saifutdinov

Author for correspondence.
Email: rgsbancorp@mail.ru
ORCID iD: 0000-0003-2839-100X
Russian Federation, 36, Butlerova str.,Republic of Tatarstan, Kazan, 420012, Russia