Preview

Archives of Pediatrics and Pediatric Surgery

Advanced search

Protein-Losing Enteropathy after Fontan Operation: A Clinical Case Description

https://doi.org/10.31146/2949-4664-apps-2-1-163-169

Abstract

The clinical observation of a boy with protein-losing enteropathy after the Fontan operation is presented. The boy, S.M., 2 years old, was admitted with complaints of abdominal pain, bloating, enlarged abdomen, frequent watery stools, and peripheral edema in the arms and legs. The child was diagnosed with a congenital heart defect at birth, for which Fontan surgery was performed. One month later, diarrhea and swelling appeared on the face, arms, and legs. Edema was temporarily relieved by symptomatic therapy. At the age of 2, the child was hospitalized in the gastroenterology department of our center. Examinations revealed low levels of total protein and albumin, and an increase in alpha-1 antitrypsin values. The diagnosis of protein-losing enteropathy after the Fontan operation was established. The child was prescribed a diet enriched with medium-chain triglycerides, spironolactone, and prednisolone. After 6 months of follow-up, no edema was observed, and the child’s overall condition was satisfactory. However, over the past year until the present, the boy has experienced slight swelling in the arms, legs, and face, requiring periodic albumin transfusions. Fontan operation and protein-losing enteropathy represent different aspects of medicine; however, sometimes one diagnosis becomes a consequence of another. As a collective of authors, we conducted the research, processed the data, obtained results, and we believe that we are correct. We affirm that the responsibility for the results and activity in writing the material remains with the authors.

About the Authors

A. T. Kamilova
Republican Specialized Scientific and Practical Medical Center of Pediatrics of the Ministry of Health of the Republic of Uzbekistan
Russian Federation


K. Sh. Usmanova
Republican Specialized Scientific and Practical Medical Center of Pediatrics of the Ministry of Health of the Republic of Uzbekistan
Russian Federation


X. T. Ubayxodjayeva
Republican Specialized Scientific and Practical Medical Center of Pediatrics of the Ministry of Health of the Republic of Uzbekistan
Russian Federation


References

1. Ozen A., Lenardo M. J. Protein-Losing Enteropathy. N Engl J Med. 2023 Aug 24;389(8):733-748. doi: 10.1056/NEJMra2301594.

2. Agrawal A., Pandey V. P., Shende A. Primary Intestinal Lymphangiectasia (Waldmann’s Disease) A Rare Case of Protein Losing Enteropathy. J Assoc Physicians India. 2020 Jan;68(1):89. PMID: 31979845.

3. Kamilova A.T., Sultankhodzhaeva Sh.S., Azizova G. K., Dustmukhamedova D. Kh. A case of primary lymphangiectasia of the small intestine associated with coeliac disease. Vopr. det. dietol. (Pediatric Nutrition). 2016; 14(5): 65-69. (in Russ.) doi: 10.20953/1727-5784-2016-5-65-69.@@ Камилова А. Т., Султанходжаева Ш. С., Азизова Г. К., Дустмухамедова Д. Х. Случай первичной лимфангиэктазии тонкой кишки, ассоциированной с целиакией. Вопросы детской диетологии. 2016; 14(5): 65-69. doi: 10.20953/1727-5784-2016-5-65-69.

4. Kamilova A. T., Geller S. I., Ubaykhodjaeva X. T. Description of a clinical case of combination of celiac disease and ichthyosis in a girl. Meditsinskiy sovet = Medical Council. 2021;(11):134-139. (In Russ.) doi: 10.21518/2079-701X-2021-11-134-139.@@ Камилова А. Т., Геллер С. И., Убайходжаева Х. Т. Описание клинического случая сочетания целиакии и ихтиоза у девочки. Медицинский Совет. 2021;(11):134-139. doi: 10.21518/2079-701X-2021-11-134-139.

5. Usmonova K. Sh., Kamilova A. T., Dustmukhamedova D. Kh., Makhmudov A. S. Severe course of celiac disease complicated by stress-induced hyperglycemia. Vopr. det. dietol. (Pediatric Nutrition). 2023; 21(6): 78-84. (In Russ.) doi: 10.20953/1727-5784-2023-6-78-84.@@ Усмонова К. Ш., Камилова А. Т., Дустмухамедова Д. Х., Махмудов А. С. Тяжелое течение целиакии, осложненной стрессовой гипергликемией. Вопросы детской диетологии. 2023; 21(6): 78-84. doi: 10.20953/1727-5784-2023-6-78-84.

6. Patel J.K., Loomes K. M., Goldberg D. J., Mercer-Rosa L., Dodds K., Rychik J. Early Impact of Fontan Operation on Enteric Protein Loss. Ann Thorac Surg. 2016 Mar;101(3):1025-30. doi: 10.1016/j.athoracsur.2015.09.036.

7. Mertens L., Hagler D. J., 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 May;115(5):1063-73. doi: 10.1016/s0022-5223(98)70406-4.

8. Johnson J.N., Driscoll D. J., O’Leary P. W. Protein-losing enteropathy and the Fontan operation. Nutr Clin Pract. 2012 Jun;27(3):375-84. doi: 10.1177/0884533612444532.

9. Thacker D., Patel A., Dodds K., Goldberg D. J., Semeao E., Rychik J. Use of oral budesonide in the management of protein-losing enteropathy after the Fontan operation. Ann Thorac Surg. 2010 Mar;89(3):837-42. doi: 10.1016/j.athoracsur.2009.09.063.

10. Hjortdal V. E. The Fontan operation: when and why? Eur J Cardiothorac Surg. 2022 Feb 18;61(3):495-496. doi: 10.1093/ejcts/ezab395.

11. Rao PS. Fontan Operation: Indications, Short and Long Term Outcomes. Indian J Pediatr. 2015 Dec;82(12):1147-56. doi: 10.1007/s12098-015-1803-6.

12. Guideline: Updates on the Management of Severe Acute Malnutrition in Infants and Children. Geneva: World Health Organization; 2013. PMID: 24649519.

13. Crupi G., Locatelli G., Tiraboschi R., Villani M., De Tommasi M., Parenzan L. Protein-losing enteropathy after Fontan operation for tricuspid atresia (imperforate tricuspid valve). Thorac Cardiovasc Surg. 1980 Oct;28(5):359-63. doi: 10.1055/s-2007-1022109.

14. Senzaki H., Masutani S., Kobayashi J., Kobayashi T., Sasaki N., Asano H., Kyo S., Yokote Y., Ishizawa A. Ventricular afterload and ventricular work in fontan circulation: comparison with normal two-ventricle circulation and single-ventricle circulation with blalock-taussig shunts. Circulation. 2002 Jun 18;105(24):2885-92. doi: 10.1161/01.cir.0000018621.96210.72. Retraction in: Circulation. 2012 Jun 12;125(23): e1020.

15. Rychik J. Protein-losing enteropathy after Fontan operation. Congenit Heart Dis. 2007 Sep-Oct;2(5):288-300. doi: 10.1111/j.1747-0803.2007. 00116.x.

16. Gandaeva L. A., Basargina E. N., Borovik T. E., Zvonkova N. G. Nutritional status correction options in children with congenital heart disease. Kazan medical journal. 2015;96(4):654-659. (in Russ.) doi: 10.17750/KMJ2015-654.@@ Гандаева Л. А., Басаргина Е. Н., Боровик Т. Э., Звонкова Н. Г. Возможности коррекции нутритивного статуса у детей с врождёнными пороками сердца. Казанский медицинский журнал. - 2015. - Т. 96. - № 4. - C. 654-659. doi: 10.17750/KMJ2015-654.

17. Edsbäcker S., Andersson T. Pharmacokinetics of budesonide (Entocort EC) capsules for Crohn’s disease. Clin Pharmacokinet. 2004;43(12):803-21. doi: 10.2165/00003088-200443120-00003.

18. Rothman A., Snyder J. Protein-losing enteropathy following the Fontan operation: resolution with prednisone therapy. Am Heart J. 1991 Feb;121(2 Pt 1):618-9. doi: 10.1016/0002-8703(91)90743-2.

19. Rychik J., Piccoli D. A., Barber G. Usefulness of corticosteroid therapy for protein-losing enteropathy after the Fontan procedure. Am J Cardiol. 1991 Sep 15;68(8):819-21. doi: 10.1016/0002-9149(91)90667-a.

20. Copland A. P. Protein Losing Enteropathy: Diagnosis and Management. PRACTICAL GASTROENTEROLOGY. APRIL 2017. SERIES 162.

21. Feldt R.H., Driscoll D. J., Offord K. P., Cha R. H., Perrault J., Schaff H. V., Puga F. J., Danielson G. K. Protein-losing enteropathy after the Fontan operation. J Thorac Cardiovasc Surg. 1996 Sep;112(3):672-80. doi: 10.1016/S0022-5223(96)70051-X.


Review

For citations:


Kamilova A.T., Usmanova K.Sh., Ubayxodjayeva X.T. Protein-Losing Enteropathy after Fontan Operation: A Clinical Case Description. Archives of Pediatrics and Pediatric Surgery. 2024;2(1):163-169. (In Russ.) https://doi.org/10.31146/2949-4664-apps-2-1-163-169

Views: 389


Creative Commons License
This work is licensed under a Creative Commons Attribution 4.0 License.


ISSN 2949-4664 (Print)