Hyperimmunoglobulinemia D syndrome successfully treated with a corticosteroid
Hyperimmunoglobulinemia D syndrome successfully treated with a corticosteroid
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皮质类固醇成功治疗高免疫球蛋白血症 D 综合征
DOI：
10.1046/j.1442-200x.2002.01541.x
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发表时间：
2002
影响因子：
1.4
通讯作者：
H. Wakiguchi
中科院分区：
文献类型：
--
作者：
K. Yoshimura;H. Wakiguchi
高免疫球蛋白血症D和周期性发热（高IgD综合征，HIDS）被定义为一种疾病实体组成的反复高尖峰热。该病伴有淋巴结肿大、腹痛、头痛、关节受累、皮肤损害和血清IgD水平升高。一些报告的数值包括>100 U/mL 1，2和>14 mg/dL。3大多数这种综合征病例在欧洲报告。Drenth等人最近发现了导致该综合征的基因。4然而，该综合征的发病机制仍不清楚，有效的治疗方法尚未建立。本报告涉及一名日本男孩与艾滋病，成功地治疗与皮质类固醇治疗。
The hyperimmunoglobulinemia D and periodic fever (hyper IgD syndrome, HIDS) is defined as a disease entity consisting of recurrent high spiking fever. The disease is accompanied with lymphadenopathy, abdominal distress, headache, joint involvement, skin lesions and an elevated serum IgD level. Some reported values include >100 U/mL1,2 and also >14 mg/dL.3 Most cases of this syndrome have been reported in Europe. Drenth et al. recently identified the gene responsible for the syndrome.4 However, the pathogenesis of this syndrome remains unclear and an effective therapy has not yet been established. This report involves a Japanese boy with HIDS, successfully treated with corticosteroid therapy.