慢性肾脏疾病患者的先天性门静脉缺失I型畸形。

J. Kopeć, M. Krzanowski, J. Królczyk, J. Jaworek-Troć, K. Krzanowska, W. Sułowicz
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引用次数: 2

摘要

1012及免疫指标均为阴性。根据脑电图和头部计算机断层扫描(CT),诊断为非惊厥性癫痫(丙戊酸治疗)。腹部CT扫描显示多脾(图1A)和门静脉缺失(图1B)。它还显示脾脏和肠静脉血通过脾静脉和肠系膜静脉流入下腔静脉和左肾静脉,并通过肝脏流出(图1C)。在静脉造影和闭塞试验(图1D)中,肝脏没有门静脉灌注,肠系膜上静脉和脾静脉没有连接形成汇合处(IB型畸形)。一名31岁男性慢性膜非增殖性肾小球肾炎(活检证实)患者接受免疫抑制治疗(类固醇、环孢素、霉酚酸酯),因慢性肾病肾病肾病综合征合并3期昏迷(双侧巴宾斯基征瞳孔僵硬)入院。血液检查显示氨(288 μmol/l)、血清生成酶(258 μmol/l)、尿素(31.2 mmol/l)、血清白蛋白(27 g/l)、丙氨酸转氨酶(37 U/l)、天冬氨酸转氨酶(30 U/l)、胆红素(24 μmol/l)、γ -谷氨酰转肽酶(138 U/l)、胆碱酯酶(3678 U/l)、铜蓝蛋白(0.26 g/l)和碱性磷酸酶(205 U/l)的浓度升高。A、B、C、G型肝炎病毒的血清学标志物,临床影像
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Abernethy malformation type I (congenital absence of the portal vein) in a patient with chronic kidney disease.
1012 as well as immunological markers were negative. On the basis of electroencephalography and head computed tomography (CT), the patient was di‐ agnosed with nonconvulsive epilepsy (treated with valproic acid). An abdominal CT scan re‐ vealed polysplenia (FIGURE 1A) and the absence of the portal vein (FIGURE 1B). It also showed that the splenic and intestinal venous blood flowed via the splenic and mesenteric veins to the in‐ ferior vena cava and to the left renal vein with‐ out passing through the liver (FIGURE 1C). On por‐ tovenography with the occlusion test (FIGURE 1D), the liver was not perfused with portal blood and the superior mesenteric vein and splenic vein did not join to form confluence (abernethy malfor‐ mation type IB). A 31 ‐year ‐old male patient with chronic membra‐ noproliferative glomerulonephritis (confirmed by biopsy) treated with immunosuppression (steroids, cyclosporine, mycophenolate mofetil) was admitted to the hospital due to nephrotic syndrome with chronic kidney disease stage 3 and coma (rigid pupils with bilateral Babinski sign). Blood tests revealed elevated concentra‐ tions of ammonia (288 μmol/l), serum creati‐ nine (258 μmol/l), urea (31.2 mmol/l), serum albumin (27 g/l), alanine aminotransferase (37 U/l), aspartate aminotransferase (30 U/l), biliru‐ bin (24 μmol/l), γ‐glutamyltranspeptidase (138 U/l), cholinesterase (3678 U/l), ceruloplasmin (0.26 g/l), and alkaline phosphatase (205 U/l). Se‐ rological markers for hepatitis A, B, C, G viruses, CLINICAL IMAGE
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[Relapsing polychondritis]. [Inclusion body myositis]. [Nephrotic syndrome]. [Mesangium]. [Hepatorenal syndrome].
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