Pediatric Hematology/Oncology and ImmunopathologyPediatric Hematology/Oncology and ImmunopathologyВопросы гематологии/онкологии и иммунопатологии в педиатрии1726-17082414-9314Fund Doctors, Innovations, Science for Children55310.24287/1726-1708-2021-20-3-140-144LITERATURE REVIEWОБЗОР ЛИТЕРАТУРЫApplication of high-performance liquid chromatography in porphyrias diagnosticsПрименение высокоэффективной жидкостной хроматографии в диагностике порфирийhttps://orcid.org/0000-0001-7848-8112PuzikovaА. I.ПузиковаА. И.
Russian Federation

Alexandra I. Puzikova, Student

8-2 Trubetskaya St., Moscow 119991 

Александра Игоревна Пузикова, студентка

119991, Москва, ул. Трубецкая, 8-2

puzikova_a_i@student.sechenov.ruhttps://orcid.org/0000-0002-6331-5339LitvinЕ. А.ЛитвинЕ. А.
Russian Federation

Moscow

Москва

https://orcid.org/0000-0001-8807-2145KildyushkinD. А.КильдюшкинД. А.
Russian Federation

Moscow

Москва

https://orcid.org/0000-0003-1308-8622DruyА. Е.ДруйА. Е.
Russian Federation

Moscow

Москва

I.M. Sechenov First Moscow State Medical University of Ministry of Healthcare of the Russian FederationФГАОУ ВО «Первый Московский государственный медицинский университет им. И.М. Сеченова» Минздрава России (Сеченовский Университет)Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian FederationФГБУ «Национальный медицинский исследовательский центр детской гематологии, онкологии и иммунологии им. Дмитрия Рогачева» Минздрава России081020212031401440810202108102021Copyright ©; 2021, «D. Rogachev NMRCPHOI»Copyright ©; 2021, ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава России2021«D. Rogachev NMRCPHOI»ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава Россииhttps://creativecommons.org/licenses/by/4.0https://hemoncim.com/jour/article/view/553

Porphyrias are distinguished by the stage of heme synthesis at which a failure occurs, leading to the accumulation of intermediate products – porphyrins. Due to the low specificity of clinical manifestations of porphyria and the latent course of the disease, their timely diagnosis is difficult. This article substantiates the effectiveness of high-performance liquid chromatography method in the determination of porphyrins. The method is suitable for porphyrin determination in urine, blood and feces of patients. Examples of its work are shown.

Порфирии различаются по этапу синтеза гема, на котором происходит сбой, приводящий к накоплению промежуточных продуктов – порфиринов. Ввиду низкой специфичности клинических проявлений порфирий и латентного течения заболеваний их своевременная диагностика затруднена. В статье дается обоснование применения высокоэффективной жидкостной хроматографии в определении порфиринов. Метод пригоден к выявлению порфиринов в моче, крови и кале пациентов. Продемонстрированы примеры его работы.

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