SAGES Magazine

THE SOUTH AFRICAN GASTROENTEROLOGY REVIEW 2023 | VOLUME 21 | ISSUE 1 | 16 Hypocholesterolemia was observed in four patients in our series. The diagnosis of PLE is based on the fecal excretion of isotope marked tracers coupled to proteins or macromolecules, injected intravenously (67Cu caeruloplasmin, 111 In transferrin, 99mTc albumin) 19 . Nevertheless, these methods have been abandoned due to their high cost, limited accessibility, collection difficulties and the use of a radioactive tracer. The diagnosis of PLE is therefore made by measuring the fecal clearance of alpha 1 antitrypsin. A1AT is resistant to intestinal and colonic proteolysis, whether enzymatic or bacterial. Its faecal clearance calculated from a plasma and a faecal measurement, (usually performed over 3 consecutive days) is normally less than 24 mL per day 20, 21, 22 . A higher flow rate is indicative of PLE. However, in cases of profuse diarrhea, elevations in fecal A1-AT clearance have been described independently of the presence of PLE, up to 56 mL per day 3 . In addition, it should be noted that the measurement of fecal 1-AT may be falsely negative in cases of purely gastric protein leakage due to A1-AT destruction at acidic pH. In this situation, it is possible to measure A1-AT clearance after removal of gastric acidity by a proton pump inhibitor 23 . PLE aetiology has 2 major subgroups - anomalies of the lymphatic flow and increased permeability of the digestive mucosa. Upper and lower endoscopy and, if necessary enteroscopy and capsule endoscopy are the most sensitive for the detection of lymphangiectasia and loss of mucosa. Histology, if obtainable, is key 24 . Figure 1 highlights aetiologies 25 . In our series, endoscopy was performed in four patients. Nutritional support is key. It consists of reducing the intake of long-chain triglycerides (< 50 g/d) and substituting with medium-chain triglycerides (MCT). The diet must also be high in protein and may include the addition of protein powder 26 . It is necessary to prevent possible deficiencies in essential fatty acids and fat-soluble vitamins (A.D.E.K). Albumin infusions combined with diuretics are also indicated. The management of lymphedema is generally disappointing. Diuretics are generally ineffective in lymphedema. Hypogammaglobulinemia increases infection risk and polyvalent human immunoglobulin may be needed. Octreotide has been suggested in some studies for the treatment of PLE associated with intestinal lymphangiectasia. Surgical treatment may be offered to PLE secondary to a Fontan procedure, such as atrial fenestration if symptomatic measures have failed 1 . Few data have been published in the literature regarding the morbidity and mortality associated with PLE 5 . This depends on the underlying diseases and the availability of appropriate treatment. Complications are relatively frequent including osteopenia or osteoporosis and recurrent infections. Regarding post-Fontan PLE, studies by Lin et al 27 have demonstrated the increased mortality with an overall rate of 62 percent. The seriousness of this pathology was well demonstrated in our series since we deplore two cases of death occurring in two patients operated for complex cardiopathy (Fontan procedure). The fatal outcome occurred respectively after 9 months and 7 years of the operation. References 1. Mertens L, Hagler DJ, Sauer U et al. Protein-losing enteropathy after the Fontan operation: an interna- tional multicenter study. J Thorac Cardiovasc Surg. 1998; 115(5):1063–1073 2. Sarah Umar,John K. Diabaise. Protein losing en- teropathy: case illustrations and clinical review. Am J Gastroenterol. 2010;105(1):43-9 3. Waldmann TA, Steinfeld JL, Dutcher TF et al. The role of the gastrointestinal system in “idiopathic hypopro- teinemia”. Gastroenterology.1961; 41:197–207 4. Feldt RH, Driscoll DJ, Offord KP et al. Protein-losing enteropathy after the Fontan operation. J Thorac Cardiovasc Surg.1996; 112:672–680 5. Marjet J, A. M. Braamskamp, Koert M, Dolman Merit M, Tabbers. Protein-losing enteropathy in children. Eur J Pediatr. 2010; 169:1179–1185 6. Zheng W, Tian X, Li L, Jing H, Li F, Zeng X, et al. Pro- tein-Losing Enteropathy in Systemic Lupus Erythe- matosus: Analysis of the Clinical Features of Fifteen Patients. JCR: Journal of Clinical Rheumatology. 2007;13(6):313. 7. Shani M, Theodor E, Frand M, Goldman B. A Family with Protein-Losing Enteropathy. Gastroenterology. 1974 ;66(3) :433-45. 8. Dray X, Vahedi K, Rousseau S, Lavergne-Slove A, Boudiaf M, Marteau P. Gastroenteropathies exsuda- tives. EMC - Gastroenterologie. 2006 ;1(2):1-7. 9. Navarro J, Mougenot J. Enteropathies exsudatives protidiques pediatriques: a` propos de 12 formes majeures. Paris, Medecine sciences, Flammari- on.1976 ;240-52. 10. Vardy PA, Lebenthal E, Shwachman H. Intesti- nal Lymphangiectasia: A Reappraisal. Pediatrics. 1975;55(6):842-51. 11. Dray X, Vahedi K, Rousseau S, et al. Gastroenteropa- thies exsudatives. Hepato-Gastro.2008; 15:139–48. 12. Nagra N, Dang S. Protein Losing Enteropathy. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing. 2019; 1-13 13. Heresbach D, Raoul J, Genetet N, Noret P, Siproud- his L, Ramee M. P, Bretagne J. F. and Gosselin M. Immunological study in primary intestinal lymphangi- ectasia. Digestion 1994; 55(1): 59–64. 14. [14] Muller, C., Wolf, H., Gottlicher, J., Zielinski, C. C. and Eibl, M. M. Cellular immunodeficiency in protein-losing enteropathy, predominant reduction of CD3+ and CD4+ lymphocytes. Dig. Dis. Sci. 1991;36: 116–122. 15.Marteau P.Gastroenteropathies exsudatives.In: Rambaud J.Traite´ de gatroenterologie.Paris,me- decines-sciences,Flammarion 2005 ;177-82. 16. Craven MD, Washabau RJ. Comparative pathophys- iology and management of protein-losing enteropa- thy. J Vet Intern Med. 2019;33(2):383-402. 17.Waldmann TA. Protein -losing enteropathy. Gastroen- terology 1966; 50: 422-43. 18. Takeda H, Ishihama K, Fukui T, Fujishima S, Orii T, Nakazawa Y, et al. Significance of rapid turnover proteins in protein-losing gastroenteropathy. Hepato- gastroenterology. 2003 ;50(54) :1963-5. 19. [19] Wang S-J, Tsai S-C, Lan J-L. Tc-99m albumin scintigraphy to monitor the effects of treatment in protein-losing gastroenteropathy. Clin Nucl Med .2000; 25(3):197–199. CASE SERIES