• Journal of Chest Surgery
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• 제52권1호
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• pp.44-46
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• 2019

Gorham-Stout disease (GSD) was first described by Gorham and colleagues in 1954, but its precise mechanism and cause remain to be elucidated. In this condition, voluminous and potentially fatal chylous effusions into the thorax can occur. Herein, we describe a case of GSD in which the patient presented with massive pleural effusions and mottled osteolytic bone lesions. We performed multiple operations, including thoracic duct ligation using video-assisted thoracoscopic surgery and thoracotomic decortication, but these procedures did not succeed in preventing recurrent pleural effusion and chest wall lymphedema. After administering sirolimus ($0.8mg/m^2$, twice a day) and propranolol (40 mg, twice a day), the process of GSD in this patient has been controlled for more than 2 years.

• Journal of Chest Surgery
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• 제48권1호
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• pp.90-94
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• 2015

Gorham-Stout Syndrome (GSS) is a rare disease characterized by localized bone resorption. Any part of the skeleton may be affected; therefore, symptoms can vary depending on the site involved. Pathological analysis reveals lymphovascular proliferation and osteolysis in the affected lesion, but the etiology of the disease is poorly understood. When GSS occurs in the chest, chylothorax or respiratory failure may occur. Thus far, a standard treatment for GSS has not been established, and the prognosis remains unknown. The following case report describes a successfully treated case of GSS in a 16-year-old boy with an affected sternum and ribs.

• Childhood Kidney Diseases
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• 제24권2호
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• pp.120-125
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• 2020

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.