Dubin-Johnson Syndrome是一個少見的隱性遺傳疾病。此病的特徵是慢性、良性的高結合性膽紅素血症。臨床上,病人常出現的症狀為黃疸。但其肝功能及膽道系統均正常。病人的壽命長短亦不受影響。過去此病的診斷方法為肝組織切片檢查或是sulfobromophthalein(BSP)試驗。但BSP可能會引發休克反應,故不再生產;而肝組織切片則具侵犯性,因此限制了臨床的實用性。近來,核子醫學的肝膽攝影及尿中coproporphyrines同位酶的測量,已證實足以診斷此病,而提供了診斷此病較為簡便及安全的方法。
Dubin-Johnson syndrome (DJS) is a rare congenital metabolic disorder of bilirubin excretion. It exhibits a clinical picture of uncharacteristic and discrete symptoms. Presently, the classical pathognomonic landmarks are dark black liver grossly and melanin-like material retention in the hepatocytes. During the past, the disease was diagnosed classically by liver biopsy or sulfobromophthalein (BSP) test. Recently, Tc^99m Disopropyl iminodiacetic acid (Tc^99m-DISIDA) cholescintigraphy was reported to be used in diagnosing the disease in order to reduce the risk of invasive procedure (i.e. liver biopsy) or anaphylactoid reaction induced by BSP. Here we present the pathognomonic picture of Tc^99m-DISIDA cholescintigraphy in DJS and conclude that this noninvasive procedure may diagnose the disease.