PDF(Pubmed)
Abstract：
UNASSIGNED: Tuberous sclerosis disorder (TSD) is a rare genetic disease that causes abnormal growths or tumors in various organs of the body. They are usually benign and asymptomatic. However, severe, rapidly growing tuberous sclerosis can be fatal. Renal angiomyolipomas are commonly associated with TSD, which can be further worsened by the presence of aneurysms and put the patient at risk for life-threatening hemorrhage.
UNASSIGNED: A 29-year-old female presented to the emergency room complaining of right flank pain with an unknown past medical history of tuberous sclerosis. The patient was suspected to have TSD as she fulfilled one of the major features of TSD required to establish a possible diagnosis. On computed tomography scan imaging, bilateral fat-density nodules were revealed in both kidneys. The largest is 7 cm in the left kidney, located at the upper pole, and was associated with a bleeding aneurysm measuring 4 cm in diameter. While the other fatty nodule was recorded at 6 cm in the right kidney at the lower pole.
UNASSIGNED: After evaluation, the patient was planned for diagnostic catheterization of the left kidney, through which selective angiography of the left kidney was done, and eventually, selective embolization of the branch supplying the left angiomyolipoma was performed.
UNASSIGNED: The authors finally conclude that thorough investigations, including systemic manifestations, must be taken into consideration when suspecting tuberous sclerosis, and a conservative approach must always be prioritized before taking any decision toward invasive approaches.
UNASSIGNED: A 29-year-old female presented to the emergency room complaining of right flank pain with an unknown past medical history of tuberous sclerosis. The patient was suspected to have TSD as she fulfilled one of the major features of TSD required to establish a possible diagnosis. On computed tomography scan imaging, bilateral fat-density nodules were revealed in both kidneys. The largest is 7 cm in the left kidney, located at the upper pole, and was associated with a bleeding aneurysm measuring 4 cm in diameter. While the other fatty nodule was recorded at 6 cm in the right kidney at the lower pole.
UNASSIGNED: After evaluation, the patient was planned for diagnostic catheterization of the left kidney, through which selective angiography of the left kidney was done, and eventually, selective embolization of the branch supplying the left angiomyolipoma was performed.
UNASSIGNED: The authors finally conclude that thorough investigations, including systemic manifestations, must be taken into consideration when suspecting tuberous sclerosis, and a conservative approach must always be prioritized before taking any decision toward invasive approaches.
摘要：
■结节性硬化症（TSD）是一种罕见的遗传性疾病，可导致身体各个器官的异常生长或肿瘤。它们通常是良性的和无症状的。然而,严重,快速增长的结节性硬化症可能是致命的。肾血管平滑肌脂肪瘤通常与TSD有关，动脉瘤的存在会进一步恶化，并使患者面临危及生命的出血风险。
■一名29岁女性出现在急诊室，主诉右侧腹部疼痛，既往有不明的结节性硬化症病史。该患者被怀疑患有TSD，因为她满足了建立可能诊断所需的TSD的主要特征之一。在计算机断层扫描成像中，双侧脂肪密度结节在两个肾脏均显示.最大的是左肾7厘米，位于上极点，并伴有直径为4厘米的出血动脉瘤。而另一个脂肪结节记录在右肾下极6厘米处。
■评估后，患者计划对左肾进行诊断性导管插入术，对左肾进行选择性血管造影，最终，对左血管平滑肌脂肪瘤的分支进行选择性栓塞。
■作者最终得出结论，彻底的调查，包括系统性表现，怀疑结节性硬化症时必须考虑，在对侵入性方法做出任何决定之前，必须始终优先考虑保守的方法。
■一名29岁女性出现在急诊室，主诉右侧腹部疼痛，既往有不明的结节性硬化症病史。该患者被怀疑患有TSD，因为她满足了建立可能诊断所需的TSD的主要特征之一。在计算机断层扫描成像中，双侧脂肪密度结节在两个肾脏均显示.最大的是左肾7厘米，位于上极点，并伴有直径为4厘米的出血动脉瘤。而另一个脂肪结节记录在右肾下极6厘米处。
■评估后，患者计划对左肾进行诊断性导管插入术，对左肾进行选择性血管造影，最终，对左血管平滑肌脂肪瘤的分支进行选择性栓塞。
■作者最终得出结论，彻底的调查，包括系统性表现，怀疑结节性硬化症时必须考虑，在对侵入性方法做出任何决定之前，必须始终优先考虑保守的方法。
