Хроническая интестинальная псевдообструкция: трудности диагностики и лечения. Клиническое наблюдение
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Khatkov IE, Tsvirkun VV, Parfenov AI, Akhmadullina OV, Krums LM, Subbotin VV, Bykova SV, Kuzmina TN, Novikova EV, Shishin KV, Khomeriki SG, Degterev DA, Lashchenkova ZP. Chronic intestinal pseudoobstruction: difficulties in diagnosis and treatment. Case report. Terapevticheskii Arkhiv (Ter. Arkh.). 2021; 93 (8): 936–942. DOI: 10.26442/00403660.2021.08.200976
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Ключевые слова: хроническая интестинальная псевдообструкция, нейромиопатия, мальротация толстой кишки, мальабсорбция, синдром избыточного бактериального роста, парентеральное питание, прокинетики, кишечная декомпрессия
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The article presents a clinical case of a 23-year-old patient with an extremely severe congenital form of chronic intestinal pseudoobstruction coupled with a neuromyopathy,colon malrotation, malabsorption, bacterial overgrowth syndrome, cholelithiasis and gastrostasis, which excluded bowel transplantation. Long-term treatment in the intensive care unit with combined, mainly parenteral nutrition for 6 months, using antibiotics, prokinetics, intestinal decompression allowed to achieve partial stabilization of the patient’s condition and transfer to home treatment with the continuation of adequate complex therapy.
Keywords: chronic intestinal pseudoobstruction, neuromyopathy, colon malrotation, malabsorption, bacterial overgrowth syndrome, parenteral nutrition, prokinetics, intestinal decompression
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DOI:10.26442/00403660.2020.12.200426
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12. Аверьянова Ю.В., Разумовский А.Ю., Степанов А.Э. Этиология и патофизиология хронического интестинального псевдообструктивного синдрома у детей. II часть. Первичные и вторичные хронические псевдообструктивные синдромы у детей и взрослых. Российский вестник детской хирургии, анестезиологии и реанимации. 2016;6(2):102-11 [Averyanova YuV, Razumovsky AYu, Stepanov AE. Etiology and pathophysiology of chronic intestinal syndrome of pseudo-obstruction in children. Part II. Primary and secondary chronic syndrome of pseudo-obstruction in children and adults. Russian journal of pediatric surgery, anesthesia and intensive. 2016;6(2):102-11 (in Russian)].
13. Klar J, Raykova D, Gustafson E, et al. Phenotypic expansion of visceral myopathy associated with ACTG2 tandem base substitution. Eur J Hum Genet. 2015;23(12):1679-83. DOI:10.1038/ejhg.2015.49
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1. Dudley HA, Sinclair IS, Mclaren IF, et al. Intestinal pseudo-obstruction. J R Coll Surg Edinb. 1958;3(3):206-17.
2. Di Nardo G, Di Lorenzo C, Lauro A, et al. Chronic intestinal pseudo-obstruction in children and adults: diagnosis and therapeutic options. Neurogastroenterol Motil. 2017;29(1). DOI:10.1111/nmo.12945
3. Auricchio A, Brancolini V, Casari G, et al. The locus for a novel syndromic form of neuronal intestinal pseudoobstruction maps to Xq28. Am J Hum Genet. 1996;58(4):743-8.
4. Wangler MF, Beaudet AL. ACTG2-Related Disorders. 2015 Jun 11. In: Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Stephens K, Amemiya A, editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993–2020. PMID: 26072522
5. Miazga A, Osiński M, Cichy W, Żaba R. Current views on the etiopathogenesis, clinical manifestation, diagnostics, treatment and correlation with other nosological entities of SIBO. Adv Med Sci. 2015;60(1):118-24. DOI:10.1016/j.advms.2014.09.001
6. Collins RRJ, Barth B, Megison S, et al. ACTG2-associated visceral myopathy with chronic intestinal pseudoobstruction, intestinal malrotation, hypertrophic pyloric stenosis, choledochal cyst, and a novel missense mutation. Int J Surg Pathol. 2019;27(1):77-83. DOI:10.1177/1066896918786586
7. Cogliandro RF, Antonucci A, De Giorgio R, et al. Patient-reported outcomes and gut dysmotility in functional gastrointestinal disorders. Neurogastroenterol Motil. 2011;23(12):1084-91. DOI:10.1111/j.1365-2982.2011.01783.x
8. Wu JJ, Rothman TP, Gershon MD. Development of the interstitial cell of Cajal: origin, kit dependence and neuronal and nonneuronal sources of kit ligand. J Neurosci Res. 2000;59(3):384-401. DOI:10.1002/(SICI)1097-4547(20000201)59:3<384::AID-JNR13>3.0.CO;2-4
9. Parfenov AI, Krums LM, Bykova SV, Ahmadullina OV. Chronic intestinal pseudo-obstruction. Terapevticheskii Arkhiv (Ter. Arkh.). 2020;92(12):4-9 (in Russian)
DOI:10.26442/00403660.2020.12.200426
10. Yaroshetskiy AI, Konanykhin VD, Stepanova SO, Rezepov NA. Hypophosphatemia and refeeding syndrome in the resumption of nutrition in critical care patients (review). Vestnik intensivnoy terapii im. A.I. Saltanova. 2019;2:82-91 (in Russian)
DOI:10.21320/1818-474X-2019-2-82-91
11. Zhu CZ, Zhao HW, Lin HW, et al. Latest developments in chronic intestinal pseudo-obstruction. World J Clin Cases. 2020;8(23):5852-65. DOI:10.12998/wjcc.v8.i23.5852
12. Averyanova YuV, Razumovsky AYu, Stepanov AE. Etiology and pathophysiology of chronic intestinal syndrome of pseudo-obstruction in children. Part II. Primary and secondary chronic syndrome of pseudo-obstruction in children and adults. Russian journal of pediatric surgery, anesthesia and intensive. 2016;6(2):102-11 (in Russian)
13. Klar J, Raykova D, Gustafson E, et al. Phenotypic expansion of visceral myopathy associated with ACTG2 tandem base substitution. Eur J Hum Genet. 2015;23(12):1679-83. DOI:10.1038/ejhg.2015.49
ГБУЗ «Московский клинический научно-практический центр им. А.С. Логинова» Департамента здравоохранения г. Москвы, Москва, Россия
*asfold@mail.ru
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Igor E. Khatkov, Viktor V. Tsvirkun, Asfold I. Parfenov*, Olga V. Akhmadullina, Larisa M. Krums, Valerii V. Subbotin, Svetlana V. Bykova, Tatiana N. Kuzmina, Elena V. Novikova, Kirill V. Shishin, Sergei G. Khomeriki, Daniil A. Degterev, Zoia P. Lashchenkova
Loginov Moscow Clinical Scientific Center, Moscow, Russia
*asfold@mail.ru