References

nogr

Экспериментальная и клиническая гастроэнтерология

Experimental and Clinical Gastroenterology

1682-8658

«Global Media Technologies»

10.31146/1682-8658-ecg-211-3-98-111

nogr-2341

Research Article

ОБЗОР

REVIEW

Муковисцидоз: коморбидность с другими тяжелыми заболеваниями

Cystic fibrosis: comorbidity with other serious diseases

https://orcid.org/0000-0001-5792-2012

Янкина

Г. Н.

Yankina

G. N.

noemail@neicon.ru

https://orcid.org/0000-0001-6395-0407

Кондратьева

Е. И.

Kondratieva

E. I.

noemail@neicon.ru

https://orcid.org/0000-0002-3043-8674

Лошкова

Е. В.

Loshkova

E. V.

loshkova@rambler.ru

https://orcid.org/0000-0002-0747-5952

Дорошенко

И. В.

Doroshenko

I. V.

noemail@neicon.ru

https://orcid.org/0000-0003-2302-4446

Ребриенко

М. В.

Rebrienko

M. V.

noemail@neicon.ru

https://orcid.org/0000-0002-3281-803X

Рафикова

Ю. С.

Rafikova

Yu. S.

noemail@neicon.ru

https://orcid.org/0000-0001-9949-4562

Боженко

Е. А.

Bozhenko

E. A.

noemail@neicon.ru

https://orcid.org/0000-0001-7308-7280

Хавкин

А. И.

Khavkin

A. I.

noemail@neicon.ru

https://orcid.org/0000-0001-5214-8072

Одинаева

Н. Д.

Odinaeva

N. D.

noemail@neicon.ru

https://orcid.org/0000-0002-2133-665X

Желев

В. А.

Zhelev

V. A.

noemail@neicon.ru

https://orcid.org/0000-0003-2048-1852

Люлька

Т. С.

Liulka

T. S.

noemail@neicon.ru

https://orcid.org/0000-0003-0339-2030

Прудникова

В. К.

Prudnikova

V. K.

noemail@neicon.ru

Федеральное государственное бюджетное научное учреждение «Медико-генетический научный центр имени академика Н. П. Бочкова»; Научно-исследовательский клинический институт детства Министерства здравоохранения Московской областиРоссияResearch Centre for Medical Genetics; Reasearch Clinical Institute of Childhood of the Ministry of Health of the Moscow RegionRussian Federation

Федеральное Государственное Бюджетное Образовательное Учреждение Высшего Образования «Сибирский государственный медицинский университет» Министерства здравоохранения Российской Федерации; Федеральное государственное бюджетное научное учреждение «Медико-генетический научный центр имени академика Н. П. Бочкова»РоссияSiberian state medical University; Research Centre for Medical GeneticsRussian Federation

Федеральное государственное бюджетное научное учреждение «Медико-генетический научный центр имени академика Н. П. Бочкова»; Федеральное государственное автономное образовательное учреждение высшего образования «Российский национальный исследовательский медицинский университет имени Н. И. Пирогова» Министерства здравоохранения Российской ФедерацииРоссияResearch Centre for Medical Genetics; Russian National Research Medical University named after N. I. PirogovRussian Federation

Федеральное государственное бюджетное научное учреждение «Медико-генетический научный центр имени академика Н. П. Бочкова»РоссияResearch Centre for Medical GeneticsRussian Federation

2023

25072023

0398111

2023

Янкина Г.Н., Кондратьева Е.И., Лошкова Е.В., Дорошенко И.В., Ребриенко М.В., Рафикова Ю.С., Боженко Е.А., Хавкин А.И., Одинаева Н.Д., Желев В.А., Люлька Т.С., Прудникова В.К.

Yankina G.N., Kondratieva E.I., Loshkova E.V., Doroshenko I.V., Rebrienko M.V., Rafikova Y.S., Bozhenko E.A., Khavkin A.I., Odinaeva N.D., Zhelev V.A., Liulka T.S., Prudnikova V.K.

Данная работа распространяется под лицензией Creative Commons Attribution 4.0.

This work is licensed under a Creative Commons Attribution 4.0 License.

https://www.nogr.org/jour/article/view/2341

Многочисленные эпидемиологические исследования демонстрируют, что самое частое орфанное заболевание во всем мире - муковисцидоз может протекать не изолированно, а сочетаться и с другими тяжелыми заболеваниями. Самым частым таким сочетанием во всех популяциях традиционно является кистозный фиброз и целиакия, которые с одной стороны отличаются ведущим механизмом воспаления, при муковисцидозе - хроническое микробное воспаление, при целиакии - аутоиммунное, с другой стороны эти два заболевания имеют ряд общих звеньев патогенеза, в частности, реализуя синдром мальабсорбции, который лишь затрудняет своевременную диагностику этих сочетанных патологических состояний. Кроме того, авторы обзора литературы делают акцент на клинических примерах позднего выявления аутоиммунной непереносимости глютена на фоне кистозного фиброза. А также рассматривают сочетание муковисцидоза и с другими тяжелыми, инвалидизирующими заболеваниями (ревматоидный артрит, фенилкетонурия, онкологические заболевания), которые порою требуют серьезного изменения терапевтической тактики.

Numerous epidemiological studies demonstrate that cystic fibrosis, the most common orphan disease in the world, can not occur in isolation, but can be combined with other serious diseases. The most common such combination in all populations is traditionally cystic fibrosis and celiac disease, which, on the one hand, differ in the leading mechanism of inflammation, in cystic fibrosis - chronic microbial inflammation, in celiac disease - autoimmune, on the other hand, these two diseases have a number of common pathogenesis links, in particular, realizing the syndrome of malabsorption, which only complicates the timely diagnosis of these combined pathological conditions. In addition, the authors of the literature review focus on clinical examples of late detection of autoimmune gluten intolerance against the background of cystic fibrosis. They also consider the combination of cystic fibrosis with other severe, disabling diseases (rheumatoid arthritis, phenylketonuria, oncological diseases), which sometimes require a serious change in therapeutic tactics.

муковисцидозцелиакиядиагностикаболезнь Кронаязвенный колитреаматоидный артритхроническая болезнь почекмикробиом

cystic fibrosisceliac diseasediagnosisCrohn’s diseaseulcerative colitisrheamatoid arthritischronic kidney diseasemicrobiome

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The authors declare that there are no conflicts of interest present.