Zhonghua nei ke za zhi · 2009
[Clinical features of Gorham-Stout syndrome and literature review].
Аннотация на английскомСтатьяОбзорНазвание и аннотация: Перевод в очереди
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Huang Hui, Xu Zuo-jun, Wang Li, Xia Wei-bo, Li Jing-xian, Feng Rui-e
Аннотация
OBJECTIVE: To describe the clinical presentations, radiographic findings and histological pathology of bones, diagnosis, treatment options and prognosis for patients with Gorham-Stout syndrome (GSS).
METHODS: Clinical data of 5 GSS patients seen from January 1980 to January 2008 were reviewed.
RESULTS: (1) There were 2 males and 3 females, aged 15 years to 37 years (mean age was 30.2 years). (2) All of them had osteolysis, but the site and extent of involved bones were not the same. Three cases had large amount of bloody pleural effusion and two of them had also chylous effusion. All of the 5 cases had no evidence of malignancies. Four cases accepted bone biopsy. Among them,2 cases having local puncture and open biopsy showed typical bone pathologic manifestations. (3) Various forms of treatment including bisphosphonates, calcium supplementation, active vitD3 treatment, local radiation therapy and surgical ligation of thoracic duct were tried. (4) Follow up and clinical outcomes: the two cases, who had only bone osteolysis remained stable. Of the other three cases who had bone osteolysis associated with pleural effusion, one patient needed interrupted effusion drainage with stable bone impairment and the other two cases were out of contact.
CONCLUSIONS: GSS is a rare disorder characterized by progressive osteolysis. The clinical presentations of this disease are variable and depend on the sites of involvement. There were no standard therapy available. Prognosis depends on the site of involvement, extent of the disease and presence of complications. Those who have pleural effusion had poor prognosis.
METHODS: Clinical data of 5 GSS patients seen from January 1980 to January 2008 were reviewed.
RESULTS: (1) There were 2 males and 3 females, aged 15 years to 37 years (mean age was 30.2 years). (2) All of them had osteolysis, but the site and extent of involved bones were not the same. Three cases had large amount of bloody pleural effusion and two of them had also chylous effusion. All of the 5 cases had no evidence of malignancies. Four cases accepted bone biopsy. Among them,2 cases having local puncture and open biopsy showed typical bone pathologic manifestations. (3) Various forms of treatment including bisphosphonates, calcium supplementation, active vitD3 treatment, local radiation therapy and surgical ligation of thoracic duct were tried. (4) Follow up and clinical outcomes: the two cases, who had only bone osteolysis remained stable. Of the other three cases who had bone osteolysis associated with pleural effusion, one patient needed interrupted effusion drainage with stable bone impairment and the other two cases were out of contact.
CONCLUSIONS: GSS is a rare disorder characterized by progressive osteolysis. The clinical presentations of this disease are variable and depend on the sites of involvement. There were no standard therapy available. Prognosis depends on the site of involvement, extent of the disease and presence of complications. Those who have pleural effusion had poor prognosis.