ppharmПедиатрическая фармакологияPediatric pharmacology1727-57762500-3089Издательство «ПедиатрЪ»10.15690/pf.v19i1.2377ppharm-2132Research ArticleКРАТКИЕ СООБЩЕНИЯSHORT REPORTСовременные принципы лечения детей с первичной интестинальной лимфангиэктазией: систематический обзор обсервационных исследованийModern Treatment Principles for Children with Primary Intestinal Lymphangiectasia: Observational Studies Systematic Reviewhttps://orcid.org/0000-0001-6395-028XШукенбаеваР. А.ShukenbaevaRegina A.

Ординатор 1-го года обучения кафедры факультетской педиатрии ФГАОУ ВО РНИМУ им. Н.И. Пирогова МЗ РФ

119333, Москва, ул. Фотиевой, д. 10, стр. 1, тел.: +7 (926) 996-87-61

eLibrary-SPIN: 8731-3023

10 Fotievoy Str., 119333 Moscow; +7 (499) 400-47-33

Shukenbaevar@mail.ruhttps://orcid.org/0000-0002-4955-0121ТуртиТ. В.TurtiTatiana V.

Доктор медицинских наук, профессор; eLibrary-SPIN: 5536-2226

Москва

MD, PhD, Professor; eLibrary-SPIN: 5536-2226

Moscow

turtit@mail.ruРоссийский национальный исследовательский медицинский университет имени Н.И. ПироговаРоссияPirogov Russian National Research Medical UniversityRussian FederationРоссийский национальный исследовательский медицинский университет имени Н.И. Пирогова; ЦКБ, РАНРоссияPirogov Russian National Research Medical University; Research Institute of Pediatrics and Children’s Health in Central Clinical Hospital, Russian Academy of SciencesRussian Federation2022040320221917277Copyright © Шукенбаева Р.А., Турти Т.В., 20222022Шукенбаева Р.А., Турти Т.В.Shukenbaeva R.A., Turti T.V.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.pedpharma.ru/jour/article/view/2132Обоснование

Обоснование. Первичная интестинальная лимфангиэктазия (ПИЛ) — это тяжелое, редкое заболевание с неблагоприятным прогнозом. Его лечение представляет сложность в связи с отсутствием клинических рекомендаций — как отечественных, так и зарубежных. Поэтому рассмотрение существующих современных принципов лечения данной патологии является актуальным, так как может позволить улучшить качество и продолжительность жизни таких пациентов.

Цель исследования — систематизировать и обобщить актуальные данные о случаях ПИЛ за последние 10 лет, определить современные принципы лечения для оказания своевременной медицинской помощи.

Методы

Методы. Был проведен систематический обзор публикаций в электронной базе данных PubMed с 2011 по 2021 г. Включены клинические случаи, наблюдательные исследования, обзоры литературы.

Результаты

Результаты. Диетотерапия при лечении ПИЛ имеет множество преимуществ и является терапией первой линии. Однако при недостаточности коррекции питания современная медицина позволяет помогать детям с данной патологией при помощи препаратов из группы аналогов соматостатина, иммунодепрессантов и оперативного вмешательства.

Заключение

Заключение. Первичная интестинальная лимфангиэктазия представляет научный интерес в практике педиатров, гастроэнтерологов. Знание болезни, раннее установление диагноза и локализации распространения патологического процесса позволяют подобрать правильную диету, определить необходимость оперативного вмешательства и назначить целенаправленную медикаментозную терапию.

Background

Background. Primary intestinal lymphangiectasia (PIL) is severe and rare disease with poor prognosis. Its management is complex due to the lack of clinical guidelines (both Russian and international). Therefore, consideration of modern principles of this pathology treatment is still crucial, it also can improve the quality and life expectancy in these patients.

Objective

Objective. The aim of the study is to schematize and unify all relevant data on PIL cases over the past 10 years, to identify modern treatment principles for provision of timely medical car. Methods. Systematic review of publications from 2011 to 2021 in PubMed database was performed. Clinical cases, observational studies, and literature reviews were included.

Results

Results. Nutrition therapy in the treatment of PIL has many advantages and it is the firstline therapy. However, modern medicine allows to help children with this pathology via somatostatin group drugs, immunosuppressants, and surgery in case of insufficient nutrition correction.

Conclusion

Conclusion. Primary intestinal lymphangiectasia has certain scientific interest in pediatricians and gastroenterologists’ practice. Understanding of the disease, its early diagnosis and pathological process localization allow us to choose the correct diet, to determine the need for surgical intervention, and to prescribe targeted drug therapy.

первичная интестинальная лимфангиэктазияболезнь Вальдманнадиета со среднецепочечными триглицеридамиоктреотидсиролимусэверолимусprimary intestinal lymphangiectasiaWaldmann diseasediet with medium-chain triglyceridesoctreotidesirolimuseverolimusНе указанNot specifiedReferencesАтлас редких болезней / под ред. А.А. Баранова, Л.С. Намазовой-Барановой. — 2-е изд., испр. и доп. — М.: ПедиатрЪ; 2016. — 420 с.Atlas redkih boleznej. Baranov AA, NamazovaBaranova LS, eds. 2nd ed., upd. Moscow: Pediatr; 2016. 420 p. (In Russ).Vignes S, Bellanger J. Primary intestinal lymphangiectasia (Waldmann’s disease). Orphanet J Rare Dis. 2008;3:5. d oi: https://doi.org/10.1186/1750-1172-3-5Vignes S, Bellanger J. Primary intestinal lymphangiectasia (Waldmann’s disease). Orphanet J Rare Dis. 2008;3:5. d oi: https://doi.org/10.1186/1750-1172-3-5Ingle SB, Hinge Ingle CR. Primary intestinal lymphangiectasia: Minireview. World J Clin Cases. 2014;2(10):528–533. doi: https://doi.org/10.12998/wjcc.v2.i10.528Ingle SB, Hinge Ingle CR. Primary intestinal lymphangiectasia: Minireview. World J Clin Cases. 2014;2(10):528–533. doi: https://doi.org/10.12998/wjcc.v2.i10.528Kwon Y, Kim MJ. The Update of Treatment for Primary Intestinal Lymphangiectasia. Pediatr Gastroenterol Hepatol Nutr. 2021;24(5):413–422. doi: https://doi.org/10.5223/pghn.2021.24.5.413Kwon Y, Kim MJ. The Update of Treatment for Primary Intestinal Lymphangiectasia. Pediatr Gastroenterol Hepatol Nutr. 2021;24(5):413–422. doi: https://doi.org/10.5223/pghn.2021.24.5.413Kwon Y, Kim ES, Choe YH, Kim MJ. Individual approach for treatment of primary intestinal lymphangiectasia in children: single-center experience and review of the literature. BMC Pediatr. 2021;21(1):21. doi: https://doi.org/10.1186/s12887-020-02447-5Kwon Y, Kim ES, Choe YH, Kim MJ. Individual approach for treatment of primary intestinal lymphangiectasia in children: single-center experience and review of the literature. BMC Pediatr. 2021;21(1):21. doi: https://doi.org/10.1186/s12887-020-02447-5Mari J, Kovacs T, Pasztor G, et al. Pediatric localized intestinal lymphangiectasia treated with resection. Int Med Case Rep J. 2019;12:23–27. doi: https://doi.org/10.2147/IMCRJ.S192940Mari J, Kovacs T, Pasztor G, et al. Pediatric localized intestinal lymphangiectasia treated with resection. Int Med Case Rep J. 2019;12:23–27. doi: https://doi.org/10.2147/IMCRJ.S192940Kwon Y, Kim ES, Choe YH, et al. Therapeutic Lymphatic Embolization in Pediatric Primary Intestinal Lymphangiectasia. Yonsei Med J. 2021;62(5):470–473. doi: https://doi.org/10.3349/ymj.2021.62.5.470Kwon Y, Kim ES, Choe YH, et al. Therapeutic Lymphatic Embolization in Pediatric Primary Intestinal Lymphangiectasia. Yonsei Med J. 2021;62(5):470–473. doi: https://doi.org/10.3349/ymj.2021.62.5.470El Haddar Z, Sbia Y, Rkain M, et al. Complicated primary intestinal lymphangiectasia (Waldmann’s disease) in a child successfully treated with octreotide: A case report from a lowresource setting. Ann Med Surg (Lond). 2021;68:102588. doi: https://doi.org/10.1016/j.amsu.2021.102588El Haddar Z, Sbia Y, Rkain M, et al. Complicated primary intestinal lymphangiectasia (Waldmann’s disease) in a child successfully treated with octreotide: A case report from a lowresource setting. Ann Med Surg (Lond). 2021;68:102588. doi: https://doi.org/10.1016/j.amsu.2021.102588Niu Y, Wu Q, Wang Y, et al. Primary intestinal lymphangiectasia in children: Twelve years of experience in the diagnosis and management. Asia Pac J Clin Nutr. 2021;30(3):358–364. doi: https://doi.org/10.6133/apjcn.202109_30(3).0002Niu Y, Wu Q, Wang Y, et al. Primary intestinal lymphangiectasia in children: Twelve years of experience in the diagnosis and management. Asia Pac J Clin Nutr. 2021;30(3):358–364. doi: https://doi.org/10.6133/apjcn.202109_30(3).0002Li S, Liu X, He Y, et al. Nutritional therapy and effect assessment of infants with primary intestinal lymphangiectasia: Case reports. Medicine (Baltimore). 2017;96(51):e9240. doi: https://doi.org/10.1097/MD.0000000000009240Li S, Liu X, He Y, et al. Nutritional therapy and effect assessment of infants with primary intestinal lymphangiectasia: Case reports. Medicine (Baltimore). 2017;96(51):e9240. doi: https://doi.org/10.1097/MD.0000000000009240Aroor S, Mundkur S, Kanaparthi S, Kumar S. Waldmann’s Disease (Primary Intestinal Lymphangiectasia) with Atrial Septal Defect. J Clin Diagn Res. 2017;11(4):SD03–SD04. doi: https://doi.org/10.7860/JCDR/2017/24989.9742Aroor S, Mundkur S, Kanaparthi S, Kumar S. Waldmann’s Disease (Primary Intestinal Lymphangiectasia) with Atrial Septal Defect. J Clin Diagn Res. 2017;11(4):SD03–SD04. doi: https://doi.org/10.7860/JCDR/2017/24989.9742Surampalli V, Ramaswamy S, Surendran D, et al. Primary Intestinal Lymphangiectasia (Waldmann’s Disease) Presenting with Chylous Effusions in a 15-Year-Old. J Clin Diagn Res. 2017;11(8):OD17–OD18. doi: https://doi.org/10.7860/JCDR/2017/29055.10522Surampalli V, Ramaswamy S, Surendran D, et al. Primary Intestinal Lymphangiectasia (Waldmann’s Disease) Presenting with Chylous Effusions in a 15-Year-Old. J Clin Diagn Res. 2017;11(8):OD17–OD18. doi: https://doi.org/10.7860/JCDR/2017/29055.10522Xinias I, Mavroudi A, Sapountzi E, et al. Primary intestinal lymphangiectasia: is it always bad? Two cases with different outcome. Case Rep Gastroenterol. 2013;7(1):153–163. doi: https://doi.org/10.1159/000348763Xinias I, Mavroudi A, Sapountzi E, et al. Primary intestinal lymphangiectasia: is it always bad? Two cases with different outcome. Case Rep Gastroenterol. 2013;7(1):153–163. doi: https://doi.org/10.1159/000348763Mohammad L, Omran R, Ibrahim A, et al. Primary intestinal lymphangiectasia in a 23-month- old girl. Oxf Med Case Reports. 2019;2019(7):omz065. doi: https://doi.org/10.1093/omcr/omz065Mohammad L, Omran R, Ibrahim A, et al. Primary intestinal lymphangiectasia in a 23-month- old girl. Oxf Med Case Reports. 2019;2019(7):omz065. doi: https://doi.org/10.1093/omcr/omz065Khayat AA. Primary intestinal lymphangiectasia presenting as limb hemihyperplasia: a case report and literature review. BMC Gastroenterol. 2021;21(1):225. doi: https://doi.org/10.1186/s12876-021-01813-6Khayat AA. Primary intestinal lymphangiectasia presenting as limb hemihyperplasia: a case report and literature review. BMC Gastroenterol. 2021;21(1):225. doi: https://doi.org/10.1186/s12876-021-01813-6Lai Y, Yu T, Qiao XY, et al. Primary intestinal lymphangiectasia diagnosed by double-balloon enteroscopy and treated by mediumchain triglycerides: a case report. J Med Case Rep. 2013;7:19. doi: https://doi.org/10.1186/1752-1947-7-19Lai Y, Yu T, Qiao XY, et al. Primary intestinal lymphangiectasia diagnosed by double-balloon enteroscopy and treated by mediumchain triglycerides: a case report. J Med Case Rep. 2013;7:19. doi: https://doi.org/10.1186/1752-1947-7-19Wang X, Jin H, Wu W. Primary Intestinal Lymphangiectasia Manifested as Unusual Edemas and Effusions: A Case Report. Medicine (Baltimore). 2016;95(10):e2849. doi: https://doi.org/10.1097/MD.0000000000002849Wang X, Jin H, Wu W. Primary Intestinal Lymphangiectasia Manifested as Unusual Edemas and Effusions: A Case Report. Medicine (Baltimore). 2016;95(10):e2849. doi: https://doi.org/10.1097/MD.0000000000002849Isa HM, Al-Arayedh GG, Mohamed AM. Intestinal lymphangiectasia in children. A favorable response to dietary modifications. Saudi Med J. 2016;37(2):199–204. doi: https://doi.org/10.15537/smj.2016.2.13232Isa HM, Al-Arayedh GG, Mohamed AM. Intestinal lymphangiectasia in children. A favorable response to dietary modifications. Saudi Med J. 2016;37(2):199–204. doi: https://doi.org/10.15537/smj.2016.2.13232

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