Progressive mesenteric lymphadenopathy with protein-losing enteropathy; a devastating complication in Gaucher disease.
Mesenteric lymphadenopathy has been rarely reported in pediatric patients with Gaucher disease, developing despite the enzyme replacement therapy. The clinical implication of this condition is undetermined, with no consensus on treatment strategies. However, this condition can reflect the progression of Gaucher disease. Moreover, it can be accompanied by the serious complication, protein-losing enteropathy. Our experience underlines the importance of careful monitoring and early intervention for mesenteric lymphadenopathy, especially in pediatric patients with neuronopathic Gaucher disease.
Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, Korea.
Beom Hee
BH
Lee
Dae-Yeon
DY
Kim
Gu-Hwan
GH
Kim
Kyung-Ja
KJ
Cho
Hye-Kyung
HK
Yoon
Han-Wook
HW
Yoo
D002648:Child; D018450:Disease Progression; D056947:Enzyme Replacement Therapy; D005776:Gaucher Disease / Q000150:complications* / Q000473:pathology; D006801:Humans; D008206:Lymphatic Diseases / Q000209:etiology* / Q000473:pathology; D008297:Male; D008640:Mesenteric Lymphadenitis / Q000209:etiology* / Q000473:pathology; D011504:Protein-Losing Enteropathies / Q000209:etiology* / Q000473:pathology; D056910:Republic of Korea