Primarne intestinalne limfangiektazije kod odraslih – dijagnostički i terapijski izazov

  • Tatiana Jocic Clinical Center of Vojvodina, Clinic of Gastroenterology and Hepatology
  • Olgica Latinovic Bosnjak University of Novi Sad, Medical faculty, Novi Sad, Serbia
  • Mirjana Zivojinov University of Novi Sad, Medical faculty, Novi Sad, Serbia
  • Mirjana Stojsic
  • Jelena Ilic Sabo
Ključne reči: dijareja;, limfangiektazija;, hipoalbuminemija;, limfocitopenija;, enteropatije sa gubitkom proteina

Sažetak


Uvod. Primarne intestinalne limfangiektazije su retko oboljenje koje karakterišu abnormalno dilatirani crevni limfni sudovi i ekstenzivan enterični gubitak limfe koja je bogata proteinima plazme, limfocitima i hilomikronima. Osnovne karakteristike oboljenja su hipoalbuminemija, hipogamaglobulinemija, limfocitopenija, ređe deficit liposolubilnih vitamina i anemija. Osim primarne, limfangiektazije mogu biti i sekundarne, u sklopu celijakije, malignih, infektivnih i zapaljenskih bolesti tankog creva, fibroze, bolesti jetre i kardiovaskularnih oboljenja. Prikaz bolesnika. Muškarac, star 33 godine, javio se na pregled sa tegobama u vidu proliva i edema potkolenica. Dijagnoza je postavljena patohistološkim pregledom biopsija tankog creva pri double balloon enteroskopiji gde su promene viđene samo u jednom segmentu pregledanog creva, što je kasnije potvrđeno i videoendoskopskom kapsulom. Zaključak. Dijagnoza intestinalnih limafangiektazija postavlja se uglavnom pre treće godine života, ali može se dijagnostikovati i kod starijih osoba. Osnov dijagnoze je patohistološka analiza bioptata sluznice creva dobijenih endoskopskim procedurama. Dijagnoza primarnih intestinalnih limfangiektazija se postavlja i isključivanjem sekundarnih uzroka.

Biografije autora

Tatiana Jocic, Clinical Center of Vojvodina, Clinic of Gastroenterology and Hepatology

specijalista interne medicine- gastroenterolog,

asistent u nastavi katedre za internu medicinu, Medicinski fakultet, Univerzitet u Novom Sadu

Olgica Latinovic Bosnjak, University of Novi Sad, Medical faculty, Novi Sad, Serbia
Clinical Centre of Vojvodina, Clinic for gastroenterology and hepatology, Novi Sad, Serbia
Mirjana Zivojinov, University of Novi Sad, Medical faculty, Novi Sad, Serbia

Clinical centre of Vojvodina, Center for Pathology and Histology, Novi Sad, Serbia

Mirjana Stojsic

Institute of Child and Youth Health Care, Department of Gastroenterohepatology and Nutrition, Novi Sad, Serbia

 

Jelena Ilic Sabo
Clinical centre of Vojvodina, Center for Pathology and Histology, Novi Sad, Serbia

Reference

Waldmann TA, Steinfeld JL, Dutcher TF, Davidson JD, Gordon RS. The role of the gastrointestinal system in "idiopathic hypoproteinemia". Gastroenterology 1961; 41: 197−207.

Lai Y, Yu T, Qiao XY, Zhao LN, Chen QK. Primary intestinal lymphangiectasia diagnosed by double-balloon enteroscopy and treated by medium-chain triglycerides: A case report. J Med Case Rep 2013;7: 19.

Ingle SB, Hinge IC. Primary intestinal lymphangiectasia: Minireview. World J Clin Cases 2014; 2(10): 528‒33.

Damle RP, Suryawanshi KH, Dravid NV, Newadkar DV. A Case of Primary Intestinal Lymphangiectasia. Ann Pathol Lab Med 2015; 2(4): 248‒51.

Park MS, Lee BJ, Gu DH, Pyo JH, Kim KJ, Lee YH, et al. Ileal polypoid lymphangiectasia bleeding diagnosed and treated by double balloon enteroscopy. World J Gastroenterol 2013; 19(45): 8440‒4.

Suehiro K, Morikage N, Murakami M, Yamashita O, Hamano K. Late-onset primary intestinal lymphangiectasia successfully managed with octreotide: A case report. Ann Vasc Dis 2012; 5(1): 96‒9.

Xinias I, Mavroudi A, Sapountzi E, Thomaidou A, Fotoulaki M, Kalambakas A, et al. Primary intestinal lymphangiectasia: Is it always bad? Two cases with different outcome. Case Rep Ga-stroenterol 2013; 7(1): 153‒63.

Troskot R, Jurčić D, Bilić A, Gomerčić-Palčić M, Težak S, Brajković I. How to treat an extensive form of primary intestinal lym-phangiectasia?. World J Gastroenterol 2015; 21(23): 7320‒5.

Lee SJ, Song HJ, Boo SJ, Na SY, Kim HU, Hyun CL. Primary in-testinal lymphangiectasia with generalized warts. World J Ga-stroenterol 2015; 21(27): 8467‒72.

Trovato FM, Musumeci G, Bonanno G, Pirri C, Catalano D. Chronic diarrhea and abdominal mass: A case of intestinal lymphangiectasia. OA Case Reports 2013; 2(11): 105‒10.

Altit G, Patel H, Morinville VD. Octreotide management of intestinal lymphangiectasia in a teenage heart transplant pa-tient. J Pediatr Gastroenterol Nutr 2012; 54(6): 824‒7.

Sasidharan S, Sivadas A. Congenital intestinal lymphangiecta-sia: A case study. World J Pharm Pharm Sci 2014; 3(11): 253‒6.

Balaban VD, Popp A, Grasu M, Vasilescu F, Jinga M. Severe Refractory Anemia in Primary Intestinal Lymphangiectasia. A Case Report. J Gastrointestin Liver Dis 2015; 24(3): 369‒73.

Desai AP, Guvenc BH, Carachi R. Evidence for medium chain triglycerides in the treatment of primary intestinal lymphan-giectasia. Eur J Pediatr Surg 2009; 19(4): 2415.

Kuroiwa G, Takayama T, Sato Y, Takahashi Y, Fujita T, Nobuoka A, et al. Primary intestinal lymphangiectasia successfully treated with octreotide. J Gastroenterol 2001; 36(2): 129‒32.

MacLean JE, Cohen E, Weinstein M. Primary intestinal and tho-racic lymphangiectasia: A response to antiplasmin therapy. Pe-diatrics 2002; 109(6): 1177‒80.

Huber T, Paschold M, Eckardt AJ, Lang H, Kneist W. Surgical therapy of primary intestinal lymphangiectasia in adults. J Surg Case Rep 2015; 2015(7): rjv081.

Bouhnik Y, Etienney I, Nemeth J, Thevenot T, Lavergne-Slove A, Matuchansky C. Very late onset small intestinal B cell lymphoma associated with primary intestinal lymphangiectasia and diffuse cutaneous warts. Gut 2000; 47(2): 296‒300.

Vignes S, Bellanger J. Primary intestinal lymphangiectasia (Waldmann's disease). Orphanet J Rare Dis 2008; 3: 5.

Objavljeno
2020/10/22
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Prikaz bolesnika