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伴有脊髓压迫的假假性甲状旁腺功能减退症。

Pseudopseudohypoparathyroidism with spinal cord compression.

作者信息

Van Dop C, Wang H, Mulaikal R M, Tolo V T, Rosenbaum A E

机构信息

Department of Pediatrics, Johns Hopkins Medical Institutions, Baltimore, Maryland.

出版信息

Pediatr Radiol. 1988;18(5):429-31. doi: 10.1007/BF02388057.

Abstract

We describe a patient with pseudopseudohypoparathyroidism who had an osseous tubercle on the anterolateral margin of the foramen magnum causing compression of the spinal cord. This patient had no evidence for any endocrinopathies and had no other spinal canal anomalies. We suggest that the morphologic phenotype found in patients with pseudopseudohypoparathyroidism, also known as Albright's hereditary osteodystrophy, has an associated risk for spinal cord compression due to congenital vertebral anomalies. The poor recovery of neurologic function following spinal decompression mandates prompt recognition and therapy of this condition in patients with Albright's hereditary osteodystrophy.

摘要

我们描述了一名患有假性假甲状旁腺功能减退症的患者,其枕骨大孔前外侧缘有一个骨结节,导致脊髓受压。该患者没有任何内分泌疾病的证据,也没有其他椎管异常。我们认为,在假性假甲状旁腺功能减退症患者(也称为奥尔布赖特遗传性骨营养不良)中发现的形态学表型,因先天性椎体异常而有脊髓受压的相关风险。脊髓减压后神经功能恢复不佳,这就要求对患有奥尔布赖特遗传性骨营养不良的患者及时识别并治疗这种情况。

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