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摘要


本文報告一例由紅斑性狼瘡母親所生之女嬰,出生後即發生在臉部出現許多圓盤狀紅斑,並於生後逐漸消褪;但一個半月後,由於上呼吸道感染合併輕微腸炎,皮膚病灶轉劇且擴散至全身。理學檢查顯示典型的狼瘡性圓盤狀紅斑分布於頭部(尤其是頭皮、額頭、眼睛與嘴巴周圍),驅幹與四肢。心跳規則,但有Grade Ⅲ/Ⅵ收縮性心雜音於心尖與與左下胸骨緣。病嬰胸部X光呈現兩側輕微的間質性浸潤發炎。24小時心電圖檢查爲正常,心臟超音波發現有小的心房中膈缺損與輕微的三尖瓣逆流。血液檢查顯示有貧血,一過性血小板過低,與紅血球沈降率增高。免疫學檢查發現母親Ro(SSA)與La(SSB)抗體均爲陽性,病嬰之La(SSB)亦為陽性,但anti-cardiolipin Ab則爲陰性。病灶復發時,其血中之ANA,IgA與IgM濃度均有增高,而C3與C4則爲下降。其病嬰與其母親在臨床上和免疫學檢查上之配合表現爲Ro(SSA)及/或La(SSB)抗體與此病之密切關係作了一個很好的例證。

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並列摘要


A female newborn bf SLE mother developed transient typical discoid-like lupus skin lesions over her face soon after birth and had severe relapse with generalized spreading following an episode of upper respiratory tract infection at 50 days of age. Blood picture showed anemia, transient thrombocytopenia and high ESR. Cardiac echo disclosed small ASD with minimal TR. Both EKG and 24 hrs EKG monitor presented normal findings. Serological studies at the early relapse stage of this disease showed increased serum ANA, IgA and IgM level with normal IgG and decrease of C3 and C4. Both Ro (SSA) and La (SSB) antibody systems were positive in mother but only positive for La (SSB) antibody system in this baby. The α-anticardiolipin antibody was negative. We suggest that the Ro (SSA) and/or La (SSB) antibody systems may play a role in the pathogenesis of neonatal lupus erythematosus.

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