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KMID : 1040420200240020120
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Childhood Kidney Diseases
2020 Volume.24 No. 2 p.120 ~ p.125
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Gorham-Stout Syndrome with Focal Segmental Glomerulosclerosis: A Case Report
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Kim Ji-Hyun
Kim You-Sun
Lim Seon-Hee
Ahn Yo-Han
Ko Jung-Min
Suh Dong-In
Lee Kyoung-Bun
Moon Kyung-Chul
Ha Il-Soo
Cheong Hae-Il
Kang Hee-Gyung
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Abstract
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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.
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KEYWORD
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Gorham disease, Osteolysis, Proteinuria, Focal Segmental Glomerulosclerosis, Adolescents
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