Home parenteral nutrition a life-saving therapy in a primary intestinal lymphangiectasia patient affecting the entire GI tract - 3 year follow-up case report

Authors

Dorota Mańkowska-Wierzbicka, Department of Gastroenterology, Dietetics and Internal Diseases, Poznan University of Medical Sciences, 49 Przybyszewskiego Street, 60-355, Poznan, PL, Poland.
Michal KloskaFollow
Katarzyna Karwowska, Department of Anesthesiology and Intensive Therapy, Poznan University of Medical Sciences, 1/2 Dluga Street, 61-848, Poznan, PL, Poland.
Marcin A. Kucharski, Department of Gastroenterology, Dietetics and Internal Diseases, Poznan University of Medical Sciences, 49 Przybyszewskiego Street, 60-355, Poznan, PL, Poland.
Adrianna Wierzbicka, Department of Gastroenterology, Dietetics and Internal Diseases, Poznan University of Medical Sciences, 49 Przybyszewskiego Street, 60-355, Poznan, PL, Poland.
Katarzyna Iwanik, Department of Clinical Pathomorphology, Poznan University of Medical Sciences, 49 Przybyszewskiego Street, 60-355, Poznan, PL, Poland.
Katarzyna Karmelita-Katulska, Department of General Radiology and Neuroradiology, Poznan University of Medical Sciences, 49 Przybyszewskiego Street, 60-355, Poznan, PL, Poland.
Marta Stelmach-Mardas, Department of Biophysics, Poznan University of Medical Sciences, 6 Grunwaldzka Street, 60-780, Poznan, PL, Poland.
Agnieszka Dobrowolska, Department of Gastroenterology, Dietetics and Internal Diseases, Poznan University of Medical Sciences, 49 Przybyszewskiego Street, 60-355, Poznan, PL, Poland.

Document Type

Article

Publication Date

7-1-2021

Publication Title

Annals of medicine and surgery (2012)

ISSN

2049-0801

Department(s)

Department of Medicine; Fellows and Residents

Keywords

Case report, Enteral nutrition, Parenteral nutrition, Primary intestinal lymphangiectasia, Protein-losing gastroenteropathy, Waldmann's disease

Abstract

Introduction and Importance: Primary intestinal lymphangiectasia (PIL) is a rare protein-losing gastroenteropathy of unknown etiology, characterized by impaired lymphatic vessels drainage. The pathological changes in PIL result in usually localized or diffuse dilatation of intestinal lacteals, leading to leakage of lymphatic fluid rich of proteins, lymphocytes, and immunoglobulins into the intestinal lumen. PIL may be asymptomatic or mildly symptomatic in moderate forms of the disease. In some patients, though, the outcome may be poor or even life-threatening. This case report demonstrates the severity of protein malnutrition, in some cases, and the extent of GI tract affected, requiring to start PN early and the need for its continuation as home parenteral nutrition (HPN). Case presentation: We present a case of 39-year-old male with Factor V Leiden deficiency, who presented initially with symptoms of malnutrition and anasarca. The diagnosis was confirmed by histopathological findings pathognomonic for PIL from biopsies of the stomach, small intestine and colon. Clinical discussion: The patient was started on low fat, high protein parenteral nutrition from the beginning of the treatment and required a long-term HPN for 3 years, because trials of tapering off and discontinuation of PN led to worsening of the biochemical results and recurrence of symptoms. Patient gradually improved and stabilized with persistent nutritional support. Conclusions: The presented case report shows the magnitude of nutritional support (HPN) needed for severe PIL patients. HPN offers PIL patients with poor outcome and life-threatening complications a chance to improve and lead a normal life.

Volume

67

First Page

102483

DOI

10.1016/j.amsu.2021.102483

PubMed ID

34168874

Recommended Citation

Mańkowska-Wierzbicka, D., Kloska, M., Karwowska, K., Kucharski, M. A., Wierzbicka, A., Iwanik, K., Karmelita-Katulska, K., Stelmach-Mardas, M., & Dobrowolska, A. (2021). Home parenteral nutrition a life-saving therapy in a primary intestinal lymphangiectasia patient affecting the entire GI tract - 3 year follow-up case report. LVHN Scholarly Works. Retrieved from https://scholarlyworks.lvhn.org/research-historical-works/55
DOI: 10.1016/j.amsu.2021.102483