The ClinicianThe ClinicianКлиницист1818-83382412-8775Publishing House ABV Press62210.17650/1818-8338-2024-18-4-K727REVIEWОБЗОРMental disorders in patients with cystic fibrosisМентальные расстройства у больных муковисцидозомhttps://orcid.org/0009-0000-0769-201XKlimenkoA. A.КлименкоА. А.
Russian Federation

Aleksandr Anatolyevich Klimenko

123423; 2 / 44 Salyama Adillya St.; 117997; 1 Ostrovityanova St.; Moscow

Александр Анатольевич Клименко

123423; ул. Саляма Адиля, 2 / 44; 117997; ул. Островитянова, 1, стр. 10; Москва

alexaless@yandex.ruhttps://orcid.org/0009-0003-5105-6784AleksandrovaA. Yu.АлександроваА. Ю.
Russian Federation

Faculty of Fundamental Medicine

119192; 27 Lomonosovskiy Avenue; Moscow

факультет фундаментальной медицины

119192; Ломоносовский просп., 27, корп. 1; Москва

L.A. Vorohobov City Clinical Hospital № 67, Moscow Healthcare DepartmentГБУЗ г. Москвы «Городская клиническая больница № 67 им. Л. А. Ворохобова Департамента здравоохранения г. Москвы»Federal Brain and Neurotechnology Center, Federal Medical and Biological Agency of RussiaФГБУ «Федеральный центр мозга и нейротехнологий» Федерального медико­биологического агентства РоссииM. V. Lomonosov Moscow State UniversityФГБОУ ВО «Московский государственный университет им. М.В. Ломоносова»1510202418412192601202526012025Copyright ©; 2024, Klimenko A.A., Aleksandrova A.Y.Copyright ©; 2024, Клименко А.А., Александрова А.Ю.2024Klimenko A.A., Aleksandrova A.Y.Клименко А.А., Александрова А.Ю.https://creativecommons.org/licenses/by/4.0https://klinitsist.abvpress.ru/Klin/article/view/622

   Introduction. Cystic fibrosis (CF) is a genetic disease that leads to dysfunction of internal organs and significantly increases the risk of respiratory infections. According to the N. P. Bochkov Medical and Genetic Research Center, 1 out of 9,000 newborns suffers from CF. Chronic disease has a negative impact on mental health and can contribute to the developmentof anxiety and depressive disorders, as well as accentuated traits and personality disorders.

   Aim. To summarize the currently available data on mental disorders in patients with CF, as well as to summarize current recommendations regarding the management of patients with this disease.

   Materials and methods. Articles were searched in Google Scholar databases, eLIBRARY.ru, Elsevier, PubMed from 2014 to 2024 by key words in Russian and English in accordance with the purpose of the study.

   Results. It has been established that mental health problems are associated with worse compliance to the treatment, as well as difficulties with self-organization and self-care. Some hypotheses that explain the pathogenesis of mental disorders have been provided. The key characteristics of course and clinical presentation of mental disorders in this group of patients have been described. Some potential recommendations for the treatment are provided, taking into the account the specificities of the metabolism of psychotropic drugs in people with CF. The tactics of management, including the creation of a multidisciplinary team that would consist of a doctor of somatic profile, psychiatrist and a psychologist. A strategy for development of this aspect of medical care for patients with CF has also been proposed.

   Conclusion. Drawing the attention of the scientific community to the problem of mental disorders in patients with CF will allow the introduction of methods for monitoring mental status and early detection of symptoms, and will also entail the creation of multidisciplinary teams, including a psychiatrist and a medical psychologist.

   Введение. Муковисцидоз (МВ) – хроническое генетическое заболевание, приводящее к нарушению функции внутренних органов, а также повышающее в значительной мере частоту развития инфекционных процессов. По данным фГБНУ «Медико-генетический научный центр им. акад. Н. П. Бочкова», у 1 из 9 тыс. новорожденных обнаруживается МВ. Наличие хронического заболевания негативно влияет на ментальное здоровье и предрасполагает к развитию тревожно-депрессивных расстройств, определенных акцентуаций характера и расстройств личности.

   Цель обзора – суммирование имеющихся на сегодняшний день данных о ментальных расстройствах у пациентов с МВ, а также обобщение актуальных рекомендаций, касающихся тактики ведения пациентов с этим заболеванием.

   Материалы и методы. Проведен поиск статей с 2014 по 2024 гг. в базах Google Scholar, eLIBRARY.ru, Elsevier, PubMed по ключевым словам на русском и английском языках в соответствии с целью исследования.

   Результаты. Показано, что ментальное неблагополучие является прогностически неблагоприятным фактором, в том числе и потому, что такие пациенты демонстрируют более низкую приверженность в отношении лечения основного заболевания, сложности с самоорганизацией и заботой о себе. Проведен анализ актуальных данных касательно психических расстройств у пациентов с МВ, представлен ряд гипотез, позволяющих объяснить патогенез развития ментальных расстройств у данной группы пациентов. Описаны варианты течения и особенности клинической картины ментальных расстройств у пациентов с МВ, приведены методические рекомендации по купированию психопатологических симптомов с учетом особенностей метаболизма психофармакологических препаратов у данной группы пациентов, описана тактика ведения, включая необходимость формирования мультидисциплинарных команд (врач соматического профиля, психиатр и психолог). Предложена потенциальная траектория развития данногоаспекта помощи больным с МВ.

   Заключение. Привлечение внимания научного сообщества к проблеме ментальных расстройств у пациентов с МВ позволит внедрить методики мониторинга психического статуса и раннего выявления симптомов, а также повлечет за собой создание мультидисциплинарных команд, включающих психиатра и медицинского психолога.

mucoviscidosiscystic fibrosisanxietydepressionmental disorderpersonality disordereating disorderpsychosomatic disordertarget therapygene CFTRмуковисцидозкистозный фиброзтревогадепрессияментальное расстройстворасстройство личностирасстройство пищевого поведенияпсихосоматическое расстройствотаргетная терапияген CFTRThe work was performed without external fundingРабота выполнена без спонсорской поддержкиQuittner A.L., Abbott J., Georgiopoulos A.M. et al. International Committee on Mental Health; EPOS Trial Study Group. International Committee on Mental Health in Cystic Fibrosis: Cystic Fibrosis Foundation and European Cystic Fibrosis Society consensus statements for screening and treating depression and anxiety. Thorax 2016;71(1):26–34. DOI: 10.1136/thoraxjnl-2015-207488VanElzakker M.B., Tillman E.M., Yonker L.M. et al. Neuropsychiatric adverse effects from CFTR modulators deserve a serious research effort. Curr Opin Pulm Med 2023;29(6):603–9. DOI: 10.1097/MCP.0000000000001014Elgudin L., Kishan S., Howe D. Depression in children and adolescent with cystic fibrosis: case studies. Int J Psychiatry Med 2004;34(4):391–7. DOI: 10.2190/N7DL-L6PE-MKYH-4910Nieddu E. Cystic fibrosis: CFTR modulators and their mechanism of action. Curr Pharm Des 2013;19(19):3474–5. DOI: 10.2174/13816128113199990322The register of patients with cystic fibrosis in the Russian Federation for 2021. Edited by S.A. Krasovsky, M.A. Starikova, A.Y. Voronkova еt al. St. Petersburg: Charitable Foundation “Islands”, 2023. (In Russ.).Регистр пациентов с муковисцидозом в Российской Федерации. 2021 год. Под ред. С.А. Красовского, М.А. Стариновой, А.Ю. Воронковой и др. СПб.: Благотворительный фонд «Острова», 2023.Schechter M.S., Ostrenga J.S., Fink A.K. et al. Decreased survival in cystic fibrosis patients with a positive screen for depression. J Cyst Fibros 2021;20(1):120–6. DOI: 10.1016/j.jcf.2020.07.020Amerio A., Sibilla F., Pescini R. et al. Mental health and cystic fibrosis: time to move from secondary prevention to predictive medicine. Pediatr Pulmonol 2020;55(9):2204–6. DOI: 10.1002/ppul.24928Fidika A., Herle M., Goldbeck L. Symptoms of depression impact the course of lung function in adolescents and adults with cystic fibrosis. BMC Pulm Med 2014;14:205. DOI: 10.1186/1471-2466-14-205Cystic Fibrosis Foundation Patient Registry. Annual data report 2021. Bethesda, Maryland. Available at: https://www.cff.org/sites/default/files/2021-11/Patient-Registry-Annual-Data-Report.pdfQuittner A.L., Goldbeck L., Abbot J. et al. Prevalence of depression and anxiety in patients with cystic fibrosis and parent caregivers: results of The International Depression Epidemiological Study across nine countries. Thorax 2014;69(12):1090–7. DOI: 10.1136/thoraxjnl-2014-205983Pearson D.A., Pumariega A.J., Seilheimer D.K. The development of psychiatric symptomatology in patients with cystic fibrosis. J Am Acad Child Adolesc Psychiatry 1991;30(2):290–7. DOI: 10.1097/00004583-199103000-00019Bathgate C.J., Hjelm M., Filigno S.S. et al. Management of mental health in cystic fibrosis. Clin Chest Med 2022;43(4):791–810. DOI: 10.1016/j.ccm.2022.06.014Talwalkar J.S., Koff J.L., Lee H.B. et al. Cystic fibrosis transmembrane regulator modulators: implications for the management of depression and anxiety in cystic fibrosis. Psychosomatics 2017;58(4):343–54. DOI: 10.1016/j.psym.2017.04.001Bathgate C.J., Muther E., Georgiopoulos A.M. et al. Positive and negative impacts of elexacaftor/tezacaftor/ivacaftor: Healthcare providers’ observations across US centers. Pediatr Pulmonol 2023;58(9):2469–77. DOI: 10.1002/ppul.26527Dagenais R.V.E., Su V.C.H., Quon B.S. Real-world safety of CFTR modulators in the treatment of cystic fibrosis : a systematic review. J Clin Med 2020;10(1):23. DOI: 10.3390/jcm10010023Heo S., Young D. C., Safirstein J. et al. Mental status changes during elexacaftor/tezacaftor/ivacaftor therapy. J Cyst Fibros 2022;21(2):339–43. DOI: 10.1016/j.jcf.2021.10.002McKinzie C.J., Goralski J.L., Noah T.L. et al. Worsening anxiety and depression after initiation of lumacaftor/ivacaftor combination therapy in adolescent females with cystic fibrosis. J Cyst Fibros 2017;16(4):525–7. DOI: 10.1016/j.jcf.2017.05.008Piehler L., Thalemann R., Lehmann C. et al. Effects of elexacaftor/tezacaftor/ivacaftor therapy on mental health of patients with cystic fibrosis. Front Pharmacol 2023;14(1):1179208. DOI: 10.3389/fphar.2023.1179208Baroud E., Chaudhary N., Georgiopoulos A.M. Management of neuropsychiatric symptoms in adults treated with elexacaftor/tezacaftor/ivacaftor. Pediatr Pulmonol 2023;58(7):1920–30. DOI: 10.1002/ppul.26412Reyes-Martinez S., Segura-Real L., Gomez-Garcia A.P. et al. Neuroinflammation, microbiota-gut-brain axis, and depression: the vicious circle. J Integr Neurosci 2023;22(3):65. DOI: 10.31083/j.jin2203065Yaltonskiy V.M., Abrosimov I.N. Coping behavior in adults with cystic fibrosis. Nacional’nyj psihologicheskij zhurnal = National Journal of Psychology 2014;(3):60–5. (In Russ.). DOI: 10.11621/npj.2014.0307Ялтонский В.М., Абросимов И.Н. Совладающее с болезнью поведение взрослых с муковисцидозом. Национальный психологический журнал 2014;(3):60–5. DOI: 10.11621/npj.2014.0307Arslan M., Chalmers S., Rentfrow K. et al. Suicide attempts in adolescents with cystic fibrosis on Elexacaftor / Tezacaftor / Ivacaftor therapy. J Cyst Fibros 2023;22(3):427–30. DOI: 10.1016/j.jcf.2023.01.015Akiskal H.S., Mallya G. Criteria for the “soft” bipolar spectrum: treatment implications. Psychopharmacol Bull 1987;23(1):68–73. DOI: 10.3928/0048-5713-19861101-15Akiskal K.K., Akiskal H.S. The theoretical underpinnings of affective temperaments: implications for evolutionary foundations of bipolar disorder and human nature. J Affect Disord 2005;85(1–2):231–9. DOI: 10.1016/j.jad.2004.08.002Serafini G., Geoffroy P.A., Aguglia A. et al. Irritable temperament and lifetime psychotic symptoms as predictors of anxiety symptoms in bipolar disorder. Nord J Psychiatry 2018;72(1):63–71. DOI: 10.1080/08039488.2017.1385851Amerio A., Magnani L., Castellani C. et al. The expression of affective temperaments in cystic fibrosis patients: psychopathological associations and possible neurobiological mechanisms. Brain Sci 2023;13(4):619. DOI: 10.3390/brainsci13040619Vazquez G.H., Tondo L., Mazzarini L. et al. Affective temperaments in general population : a review and combined analysis from national studies. J Affect Disord 2012;139(1):18–22. DOI: 10.1016/j.jad.2011.06.032Niehammer U., Straßburg S., Sutharsan S. et al. How personality influences health outcomes and quality of life in adult patients with cystic fibrosis. BMC Pulm Med 2023;23(1):190. DOI: 10.1186/s12890-023-02463-yAbrosimov I.N. Coping with a chronic illness as a predictor of psychological adaptation. Lichnost’ v menyayushchemsya mire: zdorov’e, adaptaciya, razvitie. Personality in a changing world: health, adaptation, development 2020;8(2):192–200. (In Russ.). DOI: 10.23888/humj20202192-200Абросимов И.Н. Совладание с хронической болезнью как предиктор психологической адаптации к ней. Личность в меняющемся мире: здоровье, адаптация, развитие 2020;8(2):192–200. DOI: 10.23888/humj20202192-200Darukhanavala A., Merjaneh L., Mason K. et al. Eating disorders and body image in cystic fibrosis. J Clin Transl Endocrinol 2021;26(100280):1–8. DOI: 10.1016/j.jcte.2021.100280Bryon M., Shearer J., Davies H. Eating disorders and disturbance in children and adolescents with cystic fibrosis. Children’s Health Care 2008;37(1);67–77. DOI: 10.1080/02739610701766909Petropoulou A., Bakounaki G., Grammatikopoulou M.G. et al. Eating disorders and disordered eating behaviors in cystic fibrosis: a neglected issue. Children (Basel) 2022;9(6):915. DOI: 10.3390/children9060915Bailey J., Krick S., Fontaine K.R. The changing landscape of nutrition in cystic fibrosis: the emergence of overweight and obesity. Nutrients 2022;14(6);1216. DOI: 10.3390/nu14061216Power H.A., Shivak S.M., Kim J. et al. A systematic review of attention-deficit / hyperactivity disorder in people living with cystic fibrosis. Pediatr Pulmonol 2024;59(4):825–33. DOI: 10.1002/ppul.26843.10.1002/ppul.26843Cohen-Cymberknoh M., Dimand I., Tanny T. et al. The association between Attention-Deficit-Hyperactivity-Disorder (ADHD) symptoms and disease severity in people with Cystic Fibrosis (pwCF). J Cyst Fibros 2023;22(4):772–6. DOI: 10.1016/j.jcf.2023.04.004Molitor S.J., Fidler A.L., Sinisterra M. et al. Associations between symptoms of ADHD/ODD and health outcomes in youth with cystic fibrosis. Pediatr Pulmonol 2024;59(3):809–12. DOI: 10.1002/ppul.26811Vaziri S., McGarry M.E., Huang C.Y. et al. Time to be blunt: substance use in cystic fibrosis. Pediatr Pulmonol 2024;59(4): 1015–27. DOI: 10.1002/ppul.26880.10.1002/ppul.26880Richards C.J., Friedman D., Pinsky H. et al. Alcohol and opiate misuse in adults with cystic fibrosis. Pediatr Pulmonol 2023;58(9):2535–42. DOI: 10.1002/ppul.26541Lowery E.M., Afshar M., West N. et al. Self-reported alcohol use in the cystic fibrosis community. J Cyst Fibros 2020;19(1):84–90. DOI: 10.1016/j.jcf.2019.06.004Maléth J., Balázs A., Pallagi P. et al. Alcohol disrupts levels and function of the cystic fibrosis transmembrane conductance regulator to promote development of pancreatitis. Gastroenterology 2015;148(2):427–39. DOI: 10.1053/j.gastro.2014.11.002Villarreal-Zegarra D., Barrera-Begazo J., Otazu-Alfaro S. et al. Sensitivity and specificity of the Patient Health Questionnaire (PHQ-9, PHQ-8, PHQ-2) and General Anxiety Disorder scale (GAD-7, GAD-2) for depression and anxiety diagnosis: a cross-sectional study in a Peruvian hospital population. BMJ Open 2023;13(9):e076193. DOI: 10.1136/bmjopen-2023-076193