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[戈德博姆综合征:伴有蛋白异常血症的持续性发热综合征。病例报告]

[Goldboom's syndrome: prolonged febrile syndrome with dysproteinemia. Case report].

作者信息

Vázquez Mercedes G, Affranchino Pablo N, Schon Andrea, Otero Eduardo, Young Pablo, Breyter Patricia

机构信息

Área ambulatoria, Servicio de Mediano Riesgo, Hospital de Pediatría J. P. Garrahan.

Servicio de Diagnóstico por Imágenes, Hospital de Pediatría J. P. Garrahan.

出版信息

Arch Argent Pediatr. 2018 Apr 1;116(2):e331-e335. doi: 10.5546/aap.2018.e331.

Abstract

Goldbloom syndrome is a rare clinical entity, of unknown etiology that happens almost exclusively in pediatric population. It is a prolonged febrile syndrome with periosteal hyperostosis and dysproteinemia, and often simulates an hematooncology or lymphoproliferative disease. The diagnosis is to rule out the different causes of bone pain associated with hypergammaglobulinemia, hypoalbuminemia, high erythrocyte sedimentation rate and periostitis at the radiographies. Symptomatology, radiology and laboratory parameters refer in a variable time, usually from 3 to 12 months. We report the case of a six-year-old boy with diffuse bone pain, prolonged febrile syndrome (of 8 months of evolution), weight loss and elevated acute phase reactants with dysproteinemia (hypergammaglobulinemia and hypoalbuminemia). Goldbloom syndrome should be considered in patients with prolonged febrile syndrome and cortical hyperostosis after the exclusion of infectious, lymphoproliferative or inflammatory disease.

摘要

戈德布卢姆综合征是一种罕见的临床病症,病因不明,几乎仅发生于儿童群体。它是一种伴有骨膜增生和蛋白异常血症的持续性发热综合征,常类似血液肿瘤学或淋巴增殖性疾病。诊断需排除影像学检查中与高球蛋白血症、低白蛋白血症、高红细胞沉降率及骨膜炎相关的不同骨痛病因。症状、放射学及实验室参数在不同时间出现,通常为3至12个月。我们报告一例6岁男孩,有弥漫性骨痛、持续性发热综合征(病程8个月)、体重减轻,且急性期反应物升高伴蛋白异常血症(高球蛋白血症和低白蛋白血症)。在排除感染性、淋巴增殖性或炎性疾病后,对于有持续性发热综合征和皮质增生的患者应考虑戈德布卢姆综合征。

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