NC Sin, CK Li, MK Shing, KW Chik, PMP Yuen

Abstract

Treatment of life threatening and yet steroid non-responsive Kasabath-Merritt syndrome patient with interferon alpha during infancy is associated with potentially irreversible spastic diplegia. Physicians should balance the risk and benefit of interferon treatment. Repeated clinical assessment of neuro-developmental status is mandatory in those interferon treated infants. Early discontinuation of interferon alpha treatment has the potential of reversal of early onset spastic diplegia.

本例卡沙巴梅綜合征（Kasabach-Merritt Syndrome, KMS）患兒對全身性激素治療不敏感，在1月齡時改用 α-干擾素治療，歷時一年。到 3 月齡時，表現出一定程度的臨床和血液學改善。但在 2 歲時患兒發生痙攣性雙癱，提示可能為 α-干擾素治療的併發症。與本例相似，文獻報導在嬰幼兒期應用α-干擾素治療卡-梅綜合征時，可能會發生不可逆性痙攣性雙癱。由於早期停藥可能可以逆轉早期的痙攣性雙癱，對應用 α-干擾素治療的嬰幼兒，必須定期進行神經發育方面的評價。應用干擾素治療時，醫師應權衡其利弊，僅在患兒有生命危險時才應用。