Gorham-Stout Syndrome with Focal Segmental Glomerulosclerosis: A Case Report
10.3339/jkspn.2020.24.2.120
- Author:
Ji Hyun KIM
1
;
You Sun KIM
;
Seon Hee LIM
;
Yo Han AHN
;
Jung-Min KO
;
Dong In SUH
;
Kyoung Bun LEE
;
Kyung Chul MOON
;
Il-Soo HA
;
Hae Il CHEONG
;
Hee Gyung KANG
Author Information
1. Department of Pediatrics, Seoul National University Bundang Hospital, Seongnam, Korea
- Publication Type:3
- From:Childhood Kidney Diseases
2020;24(2):120-125
- CountryRepublic of Korea
- Language:English
-
Abstract:
Gorham-Stout syndrome is a rare bone disorder characterized by progressive massive osteolysis and proliferation of vascular and lymphatic vessels. A 15-year-old boy was initially diagnosed with Gorham-Stout at the age of 8 years based on clinical and radiological findings. Following diagnosis, he was treated with pamidronate, interferon alfa, propranolol, oral corticosteroids, and sirolimus. He developed proteinuria at the age of 15 and progressed into the nephrotic range 2 years later. A renal biopsy revealed focal segmental glomerulosclerosis, not otherwise specified variant. The sequential increase in proteinuria associated with medications suggested that the focal segmental glomerulosclerosis may be caused by pamidronate and sirolimus, but cannot completely rule out the possibility of kidney involvement of GSS itself.
