Everolimus for Primary Intestinal Lymphangiectasia With Protein-Losing Enteropathy

Pediatrics
Michio OzekiToshiyuki Fukao

Abstract

Primary intestinal lymphangiectasia (PIL), also known as Waldmann's disease, is an exudative enteropathy resulting from morphologic abnormalities in the intestinal lymphatics. In this article, we describe a 12-year-old boy with PIL that led to protein-losing enteropathy characterized by diarrhea, hypoalbuminemia associated with edema (serum albumin level: 1.0 g/dL), and hypogammaglobulinemia (serum IgG level: 144 mg/dL). Severe hypoalbuminemia, electrolyte abnormalities, and tetany persisted despite a low-fat diet and propranolol. Everolimus (1.6 mg/m(2)/day) was added to his treatment as an antiangiogenic agent. With everolimus treatment, the patient's diarrhea resolved and replacement therapy for hypoproteinemia was less frequent. Hematologic and scintigraphy findings also improved (serum albumin level: 2.5 g/dL). There were no adverse reactions during the 12-month follow-up. To the best of our knowledge, this is the first report of everolimus use in a patient with PIL.

References

May 14, 1998·Archives of Disease in Childhood·M PersićI Prpić
Mar 3, 2001·Journal of Gastroenterology·G KuroiwaY Niitsu
Apr 3, 2004·Drugs·Therese M Chapman, Caroline M Perry
Feb 25, 2005·Annals of Oncology : Official Journal of the European Society for Medical Oncology·S VignotE Raymond
Nov 17, 2007·Journal of Gastroenterology and Hepatology·Ryota HokariSoichiro Miura
Nov 26, 2008·The British Journal of Radiology·T MatsumotoT Nishimura
Mar 30, 2011·Pediatric Blood & Cancer·Adrienne M HammillDenise M Adams
Apr 8, 2011·The New England Journal of Medicine·Michio OzekiNaomi Kondo
Dec 22, 2012·The Tohoku Journal of Experimental Medicine·Michio OzekiNaomi Kondo
Sep 23, 2014·Seminars in Pediatric Surgery·Cameron C Trenor, Gulraiz Chaudry
Oct 11, 2014·Pediatric Radiology·Ladonna J MaloneLorna P Browne
Jun 5, 2015·European Journal of Pediatrics·Herwig LacknerChristian E Urban

❮ Previous
Next ❯

Citations

Mar 5, 2017·Journal of Perinatal Medicine·Shi-Min Yuan
May 29, 2020·Journal of Paediatrics and Child Health·Robert N Lopez, Andrew S Day
Jun 11, 2020·World Journal of Clinical Cases·Wen-Hao LinLan-Lan Geng
Dec 1, 2020·Pediatric Radiology·Christian A BarreraDavid M Biko
Jan 29, 2021·World Journal of Gastroenterology : WJG·Rudolf HuberChristian Datz
Jun 25, 2021·Circulation Research·Angela QueisserMiikka Vikkula
May 30, 2021·Pediatrics·Erica G SchmittMaleewan Kitcharoensakkul

❮ Previous
Next ❯

Related Concepts

Related Feeds

CSF & Lymphatic System

This feed focuses on Cerebral Spinal Fluid (CSF) and the lymphatic system. Discover the latest papers using imaging techniques to track CSF outflow into the lymphatic system in animal models.