Childhood Kidney Diseases

Korean Society of Pediatric Nephrology (대한소아신장학회)
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Gorham-Stout Syndrome with Focal Segmental Glomerulosclerosis: A Case Report

  • Kim, Ji Hyun (Department of Pediatrics, Seoul National University Bundang Hospital) ;
  • Kim, You Sun (Departments of Pediatrics, Seoul National University Children's Hospital) ;
  • Lim, Seon Hee (Departments of Pediatrics, Seoul National University Children's Hospital) ;
  • Ahn, Yo Han (Departments of Pediatrics, Seoul National University Children's Hospital) ;
  • Ko, Jung-Min (Departments of Pediatrics, Seoul National University Children's Hospital) ;
  • Suh, Dong In (Departments of Pediatrics, Seoul National University Children's Hospital) ;
  • Lee, Kyoung Bun (Departments of Pathology, Seoul National University Hospital) ;
  • Moon, Kyung Chul (Departments of Pathology, Seoul National University Hospital) ;
  • Ha, Il-Soo (Departments of Pediatrics, Seoul National University Children's Hospital) ;
  • Cheong, Hae Il (Departments of Pediatrics, Seoul National University Children's Hospital) ;
  • Kang, Hee Gyung (Departments of Pediatrics, Seoul National University Children's Hospital)
  • Received : 2020.07.27
  • Accepted : 2020.09.29
  • Published : 2020.10.31
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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.

Keywords

References

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