Protein losing enteropathy in children

Öz

Protein losing enteropathy is a medical condition which is characterized by loss of protein vitamin and trace elements into the intestines which can be complicated by various diseases The main causes of protein losing enteropathy in children are primary or secondary intestinal lymphangiectesia Menetrier disease inflammatory and immunological disorders Hypoalbuminemia developed due to intestinal protein loss provides the basis of clinical findings Treatment and prognosis of the disease is closely related to the underlying disease In this paper the causes of protein losing enteropapathy clinical findings and treatment approaches were reviewed in the light of current information Turk Arch Ped 2013; 48: 7 12

Anahtar Kelimeler

• Child, hypoalbuminemia, protein-losing enteropathyDO I: 10.4274/tpa.1816

Çocuklarda protein kaybettiren enteropati

Öz

Protein kaybettiren enteropati altında farkı hastalıkları barındıran bağırsaktan protein vitamin ve eser elementlerin kaybı ile giden bir klinik durumdur Hastalık sindirim sistemine özgü hastalıklardan kaynaklanabildiği gibi sindirim sistemi dışı bazı sistemik hastalıklardan da kaynaklanabilir Klinik bulguların temelini bağırsaktan protein kaybı sonucu gelişen hipoalbüminemi oluşturur Hastalığın tedavisi ve seyri altta yatan hastalıkla yakın ilişkilidir Bu derlemede protein kaybettiren enteropatilerin nedenleri klinik bulguları ve tedavi yaklaşımları güncel bilgiler ışığında anlatılmıştır.

Anahtar Kelimeler

• Çocuk
• hipoalbüminemi
• protein kaybettiren enteropati

Kaynakça

1. Braamskamp MJ, Dolman KM, Tabbers MM. Clinical practice. Proteinlosing enteropathy in children. Eur J Pediatr 2010; 169: 1179-85.
2. Umar SB, DiBaise JK. Protein-losing enteropathy: case illustraions and clinical review. Am J Gastroenterol 2010; 105(1): 43-9.
3. Proujansky R. Protein- losing enteropathy. In: Walker WA, Goulet OJ, Kleinman RE, Sanderson IR, Sherman PM, Shneider BL, (eds). Pediatric gastrointestinal disease. Ontario: BC Decker Inc, 2004: 194-202.
4. Greenwald D. Protein-losing gastroenteropathy. In: Feldman M, Friedman LS, Brandt LJ, Sleisinger MH, (eds). Gastrointestinal and liver disease. Philadelphia: Saunders, 2006: 557-63
5. Bode L, Murch S, Freeze HH. Heparan sulfate plays a central role in a dynamic in vitro model of protein-losing enteropathy. J Biol Chem 2006; 281(12): 7809-15.
6. Iwama I, Kagimoto S, Takano T, Sekijima T, Kishimoto H, Oba A. Case of pediatric Ménétrier disease with cytomegalovirus and Helicobacter pylori co-infection. Pediatr Int 2010; 52(4): e200-3.
7. Chehade M, Magid MS, Mofidi S, Nowak-Wegrzyn A, Sampson HA, Sicherer SH. Allergic eosinophilic gastroenteritis with protein-losing enteropathy: intestinal pathology, clinical course, and long-term followup. J Pediatr Gastroenterol Nutr 2006; 42(5): 516-21.
8. Yazici Y, Erkan D, Levine DM, Parker TS, Lockshin MD. Protein-losing enteropathy in systemic lupus erythematosus: report of a severe, persistent case and review of pathophysiology . Lupus 2002; 11(2): 119Waldmann TA, Steinfeld JL, Dutcher TF, Davidson JD, Gordon RS Jr. The role of the gastrointestinal system in "idiopathic hypoproteinemia”. Gastroenterology 1961; 41: 197-207.

9. Hokari R, Kitagawa N, Watanabe C, et al. Changes in regulatory molecules for lymphangiogenesis in intestinal lymphangiectasia with enteric protein loss. J Gastroenterol Hepatol 2008; 23: e88-95.
10. Vignes S, Bellanger J. Primary intestinal lymphangiectasia ( Waldmann’s disease). Orphanet J Rare Dis 2008; 22: 3-5.
11. Suresh N, Ganesh R, Sankar J, Sathiyasekaran M. Primary intestinal lymphangiectasia. Indian Pediatr 2009; 46(10): 903-6.
12. Lin WS, Hwang MS, Chung HT, et al. Protein-losing enteropathy after the Fontan operation: clinical analysis of nine cases. Chang Gung Med J 2006; 29(5): 505-12.
13. Ghanayem NS, Berger S, Tweddell JS. Medical management of the failing Fontan. Pediatr Cardiol 2007; 28(6): 465-71.
14. Mertens L, Hagler DJ, Sauer U, Somerville J, Gewillig M. Protein-losing enteropathy after the Fontan operation: an international multicenter study. PLE study group. J Thorac Cardiovasc Surg 1998; 115(5): 1063-73.
15. Driscoll DJ, Offord KP, Feldt RH, Schaff HV, Puga FJ, Danielson GK. Five- to fifteen year follow-up after Fontan operations. Circulation 1992; 85(2): 469-96.
16. Rychik J, Gui-Yang S. Relation of mesenteric vascular resistance after Fontan operation and protein-losing enteropathy. Am J Cardiol 2002; 90: 672-4.
17. Ostrow AM, Freeze H, Rychik J. Protein-losing enteropathy after Fontan operation: investigations into possible pathophysiologic mechanisms. Ann Thorac Surg 2006; 82(2): 695-700.
18. Stiller B. Protein-losing enteropathy in patients with Fontan operations. J Pediatr Gastroenterol Nutr 2003; 37(5): 528-30.
19. Liem YS, Bode L, Freeze HH, Leebeek FW, Zandbergen AA, Paul Wilson J. Using heparin therapy to reverse protein-losing enteropathy in a patient with CDG-Ib. Nat Clin Pract Gastroenterol Hepatol 2008; 5(4): 220-4.
20. Westphal V, Murch S, Kim S, et al. Reduced heparan sulfate accumulation in enterocytes contributes to protein-losing enteropathy in a congenital disorder of glycosylation. Am J Pathol 2000; 157(6): 1917-25.
21. Murch SH, Winyard PJ, Koletzko S, et al. Congenital enterocyte heparan sulphate deficiency with massive albumin loss, secretory diarrhoea, and malnutrition. Lancet 1996; 347: 1299-301. 11