Рецепт. 2019; : 438-453
Надпочечниковая недостаточность: определение, классификация, диагностика, современные принципы лечения
Аннотация
В настоящее время в медицине огромное значение придается эндокринной системе, которая обеспечивает регуляцию жизненно важных процессов в человеческом организме. Одним из наиболее значимых компонентов этой системы являются надпочечники. В корковом и мозговом веществе надпочечников вырабатываются минералокортикоиды, глюкокортикоиды, половые гормоны и катехоламины. Эти гормоны участвуют практически во всех видах обмена веществ.
Одной из патологий надпочечников является надпочечниковая недостаточность: клинический синдром, обусловленный недостаточной секрецией гормонов коры надпочечников в результате нарушения функционирования одного или нескольких звеньев гипоталамо-гипофизарно-надпочечниковой системы.
Актуальность изучения надпочечниковой недостаточности обусловлена трудностью диагностики причин этой патологии. Ее причиной может служить множество факторов.
В статье приводится современная классификация надпочечниковой недостаточности. Диагностика этой патологии включает оценку клинических проявлений, лабораторное подтверждение синдрома гипокортицизма, этиологическую диагностику. При трудностях в диагностике надпочечниковой недостаточности используют стимуляционные пробы.
В статье представлен расчет доз препаратов, которые в настоящее время используются для заместительной терапии. Для определения эффективности назначенного лечения используются критерии адекватности глюкокортикоидной и минералокортикоидной терапии надпочечниковой недостаточности. В публикации приводятся перспективы заместительной терапии хронической надпочечниковой недостаточности, включающие назначение дегидроэпиандростерона; назначение нового лекарственного средства с модифицированным двойным высвобождением гидрокортизона и однократным приемом в сутки; введение гидрокортизона через подкожную помпу; лечение ритуксимабом и тетракозактидом; исследование регенеративного потенциала стволовых адренокортикальных клеток в сочетании с иммуномодуляторами; использование генотерапии и др.
Только четкое понимание специалистом этиопатогенеза этой патологии, ее клинических проявлений позволяет правильно трактовать результаты диагностических тестов, установить диагноз, в последующем назначить адекватное лечение и существенно снизить смертность пациентов данного профиля, улучшить качество их жизни.
Список литературы
1. Akmayev I. (2001) Neyro-immunno-endokrinnye vzaimodeystviya: ikh rol v disregulyatornoy patologii [Neuro-immune-endocrine interactions: their role in dysregulatory pathology]. Pathological physiology and experimental therapy, no 4, pp. 3–10.
2. Belyaeva A., Ladygina D., Ryzhkova Ye. (2017) Raznoobraziye prichin gipopituitarizma: slozhny klinichesky sluchay [Variety of causes of hypopituitarism: a complex clinical case]. Kremlevskaya meditsina. Klinichesky vestnik, no 4 (1), pp. 177–183.
3. Chernikova V., Solovyuk A., Solovyuk Ye. (2017) Autoimmunny poliglandulyarny sindrom 2-go tipa (klinichesky sluchay sindroma Karpentera) [Autoimmune polyglandular type 2 syndrome (a clinical case of Carpenter syndrome)]. International endocrinology journal, no 13 (8), pp. 638–640.
4. Dedov I., Fadeyev V., Melnichenko G. (2006) Nedostatochnost nadpochechnikov [Adrenal insufficiency]. M.: VUNMTs.
5. Dedov I., Marova Ye., Vaks V. (2000) Nadpochechnikovaya nedostatochnost (etiologiya, patogenez, klinika, diagnostika, lecheniye): metodicheskoye posobiye dlya vrachey [Adrenal insufficiency (etiology, pathogenesis, clinic, diagnosis, treatment): methodological guide for the doctors]. M.: Meditsina.
6. Dedov I., Melnichenko G. (2018) Jendokrinologija. Nacional'noe rukovodstvo [Endocrinology. National leadership]. M.: GEOTAR-Media.
7. Fadeyev V. (2003) Ostraya nadpochechnikovaya nedostatochnost [Acute adrenal insufficiency]. Vrach, no 3, pp. 60–61.
8. Fadeyev V. (2008) Nadpochechnikovaya nedostatochnost (klinika, diagnostika, lecheniye): rekomendatsii dlya vrachey [Adrenal insufficiency (clinic, diagnosis, treatment): recommendations for the doctors]. M.: Medpraktika.
9. Kalinin A., Maystrenko N. (2000) Khirurgiya nadpochechnikov [Adrenal surgery]. M.: Meditsina.
10. Kettayl V.M., Arki R.A. (2001) Patofiziologiya endokrinnoy sistemy: per. s angl. [Pathophysiology of the endocrine system: translated from English]. SPb.: Nevsky Dialekt, M.: BINOM.
11. Komissarenko Yu., Sidorova I., Bobrik M., Reznichenko V. (2015) Pervichnaya nadpochechnikovaya nedostatochnost: diagnostika i menedzhment (po materialam 17th European Congress of Endocrinology, Dublin, Ireland 16–20 May, 2015) [Primary adrenal insufficiency: diagnosis and management (according to materials of the 17th European Congress on Endocrinology, Dublin, Ireland, May 16–20, 2015)]. International endocrinology journal, no 7 (71), pp. 51–58.
12. Lashin D., Burets Ye., Naumova N. (2017) Khronicheskaya nadpochechnikovaya nedostatochnost [Chronic adrenal insufficiency]. Mnogoprofilny statsionar, no 2 (4), pp. 88–90.
13. Levkina M., Bondarenko Ye., Shilova L. (2018) Autoimmunnye poliglandulyarnye sindromy [Autoimmune polyglandular syndromes]. Lekarstvenny vestnik, no 2 (70), pp. 41–45.
14. Litvitskiy P. (2011) Patologiya endokrinnoy sistemy. Etiologiya i patogenez endokrinopaty: narusheniya funktsy nadpochechnikov [Pathology of the endocrine system. Etiology and pathogenesis of endocrinopathy: adrenal dysfunction]. Current pediatrics, no 10 (5), pp. 63–73.
15. Manusharova R., Cherkezova E. (2001) Gipopituitarizm [Hypopituitarism]. Meditsinsky sovet, no 7–8, pp. 25–27.
16. Nadeau S., Rivest S. (2003) Glucocorticoids play a fundamental role in protecting the brain during innate immune response. J. Neurosci, vol. 23, no 13, pp. 5536–5544. doi.org/10.1523/ JNEUROSCI.23-13-05536.2003
17. O’Connor K.A., Johnson J.D., Hammack S.E., Brooks L.M., Spencer R.L., Watkins L.R., Maier S.F. (2003) Inescapable shock induces resistance to the effects of dexamethasone. Psychoneuroendocrinology, vol. 28, no 4, pp. 481–500. doi.org/10.1016/S0306-4530(02)00035-5
18. Pankiv V. (2013) Pervichnaya nedostatochnost kory nadpochechnikov [Primary adrenal insufficiency]. International endocrinology journal, no 3 (51), pp. 110–118.
19. Potemkin V. (2017) Endokrinologiya. Rukovodstvo dlya vrachey [Endocrinology. A guide for the doctors]. M.: MIA.
20. Potemkin V., Starostina Ye. (2008) Neotlozhnaya endokrinologiya: Rukovodstvo dlya vrachey [Emergency Endocrinology: A Guide for the Doctors]. M.: MIA.
21. Prilutsky A., Prilutskaya O., Strelchenko Ye. (2014) Autoimmunnye poliendokrinnye sindromy: klassifikatsiya, klinika, diagnostika, lecheniye [Autoimmune polyendocrine syndromes: classification, clinic, diagnosis, treatment]. International endocrinology journal, no 4 (60), pp. 15–20.
22. Shustov S., Baranov V., Khalimov Yu. (2012) Klinicheskaya endokrinologiya [Clinical endocrinology]. M.: MIA.
23. Straub R.H., Besedovsky H.O (2003) Integrated evolutionary, immunological, and neuroendocrine framework for the pathogenesis of chronic disabling inflammatory diseases. FASEB J., vol. 17, no 15, pp. 2176–2183. doi.org/10.1096/fj.03-0433hyp
24. Tishenina R., Stotskaya T., Borodina Ye. (2008) Problemy diagnostiki i lecheniya yatrogennoy khronicheskoy nadpochechnikovoy nedostatochnosti [Diagnostic and therapeutic problems of chronic iatrogenic adrenal insufficiency]. Almanac of clinical medicine, no 8, pp. 57–62.
25. Tomilova Ye., Demidova T. (2017) Addisonichesky kriz kak debyut pervichnoy khronicheskoy nadpochechnikovoy nedostatochnosti [Addisonian crisis as onset of primary chronic adrenal insufficiency]. Therapy, no 5 (15), pp. 45–48.
26. Troshina Ye. (2017) Sbornik metodicheskikh rekomendatsy [Collection of guidelines]. Tver: Triada.
Recipe. 2019; : 438-453
Adrenal Insufficiency: Definition, Classification, Diagnostics, Modern Treatment Principles
Abstract
Currently, in medicine, great importance is attached to the endocrine system, which provides for the regulation of vital processes in the human body. One of the most significant components of this system are the adrenal glands. In the cortex and medulla of the adrenal glands, mineralocorticoids, glucocorticoids, sex hormones and catecholamines are produced. These hormones are involved in almost all types of metabolism.
One of the pathologies of the adrenal glands is adrenal insufficiency: a clinical syndrome due to insufficient secretion of the adrenal cortex hormones as a result of a dysfunction of one or several links of the hypothalamic-pituitary-adrenal system.
The relevance of studying adrenal insufficiency is due to the difficulty of diagnosing the causes of this pathology. It can be caused by many factors.
The article provides a modern classification of adrenal insufficiency. The diagnosis of this pathology includes an assessment of clinical manifestations, laboratory confirmation of the hypocorticism syndrome, etiological diagnosis. For difficulties in diagnosing adrenal insufficiency, stimulation tests are used.
The article presents the calculation of doses of drugs that are currently used for replacement therapy. To determine the effectiveness of the prescribed treatment, the criteria for the adequacy of glucocorticoid and mineralocorticoid therapy of adrenal insufficiency are used. The publication presents the prospects for the substitution treatment of chronic adrenal insufficiency, including the administration of dehydroepiandrosterone; the appointment of a new drug with a modified double release of hydrocortisone and a single dose per day; the introduction of hydrocortisone through the subcutaneous pump; treatment with rituximab and tetracosactide; investigation of the regenerative potential of stem adrenocortical cells in combination with immunomodulators; use of gene therapy, etc.
Only a clear understanding by the specialist of the etiopathogenesis of this pathology, its clinical manifestations allows us to correctly interpret the results of diagnostic tests, establish the diagnosis, subsequently prescribe adequate treatment and significantly reduce the mortality of patients of this profile, improve their quality of life.
References
1. Akmayev I. (2001) Neyro-immunno-endokrinnye vzaimodeystviya: ikh rol v disregulyatornoy patologii [Neuro-immune-endocrine interactions: their role in dysregulatory pathology]. Pathological physiology and experimental therapy, no 4, pp. 3–10.
2. Belyaeva A., Ladygina D., Ryzhkova Ye. (2017) Raznoobraziye prichin gipopituitarizma: slozhny klinichesky sluchay [Variety of causes of hypopituitarism: a complex clinical case]. Kremlevskaya meditsina. Klinichesky vestnik, no 4 (1), pp. 177–183.
3. Chernikova V., Solovyuk A., Solovyuk Ye. (2017) Autoimmunny poliglandulyarny sindrom 2-go tipa (klinichesky sluchay sindroma Karpentera) [Autoimmune polyglandular type 2 syndrome (a clinical case of Carpenter syndrome)]. International endocrinology journal, no 13 (8), pp. 638–640.
4. Dedov I., Fadeyev V., Melnichenko G. (2006) Nedostatochnost nadpochechnikov [Adrenal insufficiency]. M.: VUNMTs.
5. Dedov I., Marova Ye., Vaks V. (2000) Nadpochechnikovaya nedostatochnost (etiologiya, patogenez, klinika, diagnostika, lecheniye): metodicheskoye posobiye dlya vrachey [Adrenal insufficiency (etiology, pathogenesis, clinic, diagnosis, treatment): methodological guide for the doctors]. M.: Meditsina.
6. Dedov I., Melnichenko G. (2018) Jendokrinologija. Nacional'noe rukovodstvo [Endocrinology. National leadership]. M.: GEOTAR-Media.
7. Fadeyev V. (2003) Ostraya nadpochechnikovaya nedostatochnost [Acute adrenal insufficiency]. Vrach, no 3, pp. 60–61.
8. Fadeyev V. (2008) Nadpochechnikovaya nedostatochnost (klinika, diagnostika, lecheniye): rekomendatsii dlya vrachey [Adrenal insufficiency (clinic, diagnosis, treatment): recommendations for the doctors]. M.: Medpraktika.
9. Kalinin A., Maystrenko N. (2000) Khirurgiya nadpochechnikov [Adrenal surgery]. M.: Meditsina.
10. Kettayl V.M., Arki R.A. (2001) Patofiziologiya endokrinnoy sistemy: per. s angl. [Pathophysiology of the endocrine system: translated from English]. SPb.: Nevsky Dialekt, M.: BINOM.
11. Komissarenko Yu., Sidorova I., Bobrik M., Reznichenko V. (2015) Pervichnaya nadpochechnikovaya nedostatochnost: diagnostika i menedzhment (po materialam 17th European Congress of Endocrinology, Dublin, Ireland 16–20 May, 2015) [Primary adrenal insufficiency: diagnosis and management (according to materials of the 17th European Congress on Endocrinology, Dublin, Ireland, May 16–20, 2015)]. International endocrinology journal, no 7 (71), pp. 51–58.
12. Lashin D., Burets Ye., Naumova N. (2017) Khronicheskaya nadpochechnikovaya nedostatochnost [Chronic adrenal insufficiency]. Mnogoprofilny statsionar, no 2 (4), pp. 88–90.
13. Levkina M., Bondarenko Ye., Shilova L. (2018) Autoimmunnye poliglandulyarnye sindromy [Autoimmune polyglandular syndromes]. Lekarstvenny vestnik, no 2 (70), pp. 41–45.
14. Litvitskiy P. (2011) Patologiya endokrinnoy sistemy. Etiologiya i patogenez endokrinopaty: narusheniya funktsy nadpochechnikov [Pathology of the endocrine system. Etiology and pathogenesis of endocrinopathy: adrenal dysfunction]. Current pediatrics, no 10 (5), pp. 63–73.
15. Manusharova R., Cherkezova E. (2001) Gipopituitarizm [Hypopituitarism]. Meditsinsky sovet, no 7–8, pp. 25–27.
16. Nadeau S., Rivest S. (2003) Glucocorticoids play a fundamental role in protecting the brain during innate immune response. J. Neurosci, vol. 23, no 13, pp. 5536–5544. doi.org/10.1523/ JNEUROSCI.23-13-05536.2003
17. O’Connor K.A., Johnson J.D., Hammack S.E., Brooks L.M., Spencer R.L., Watkins L.R., Maier S.F. (2003) Inescapable shock induces resistance to the effects of dexamethasone. Psychoneuroendocrinology, vol. 28, no 4, pp. 481–500. doi.org/10.1016/S0306-4530(02)00035-5
18. Pankiv V. (2013) Pervichnaya nedostatochnost kory nadpochechnikov [Primary adrenal insufficiency]. International endocrinology journal, no 3 (51), pp. 110–118.
19. Potemkin V. (2017) Endokrinologiya. Rukovodstvo dlya vrachey [Endocrinology. A guide for the doctors]. M.: MIA.
20. Potemkin V., Starostina Ye. (2008) Neotlozhnaya endokrinologiya: Rukovodstvo dlya vrachey [Emergency Endocrinology: A Guide for the Doctors]. M.: MIA.
21. Prilutsky A., Prilutskaya O., Strelchenko Ye. (2014) Autoimmunnye poliendokrinnye sindromy: klassifikatsiya, klinika, diagnostika, lecheniye [Autoimmune polyendocrine syndromes: classification, clinic, diagnosis, treatment]. International endocrinology journal, no 4 (60), pp. 15–20.
22. Shustov S., Baranov V., Khalimov Yu. (2012) Klinicheskaya endokrinologiya [Clinical endocrinology]. M.: MIA.
23. Straub R.H., Besedovsky H.O (2003) Integrated evolutionary, immunological, and neuroendocrine framework for the pathogenesis of chronic disabling inflammatory diseases. FASEB J., vol. 17, no 15, pp. 2176–2183. doi.org/10.1096/fj.03-0433hyp
24. Tishenina R., Stotskaya T., Borodina Ye. (2008) Problemy diagnostiki i lecheniya yatrogennoy khronicheskoy nadpochechnikovoy nedostatochnosti [Diagnostic and therapeutic problems of chronic iatrogenic adrenal insufficiency]. Almanac of clinical medicine, no 8, pp. 57–62.
25. Tomilova Ye., Demidova T. (2017) Addisonichesky kriz kak debyut pervichnoy khronicheskoy nadpochechnikovoy nedostatochnosti [Addisonian crisis as onset of primary chronic adrenal insufficiency]. Therapy, no 5 (15), pp. 45–48.
26. Troshina Ye. (2017) Sbornik metodicheskikh rekomendatsy [Collection of guidelines]. Tver: Triada.
