摘要 目的：通过评估腹膜后纤维化（RPF）患者进展过程中多种预后因素的有效性，建立切实有效的分形分析方法判断RPF患者病情的严重程度。

方法：本研究选取了2004.1—2008.12被诊断为特发性腹膜后纤维化（IRF）伴双侧梗阻性肾衰竭的患者共19例(男/女:5/14)。本研究采用回顾性研究的方法，研究对象选择确诊IRF的病人。诊断标准包括：腹膜后活检证实；影像学检查，尤其是CT检查证实腹膜后肿物，并除外任何可引起上述表现的肾疾病、输尿管疾病或其他疾病。CT检查对于评估腹膜后肿物与主动脉及下腔静脉的关系，肿物的大小，在随访中监测外科手术的效果均具有重要意义。收集如下数据：病史、查体、实验室检查（血尿素氮，肌酐，血糖，血钠，血钾，碳酸氢根离子水平，血清PH，尿酸，红细胞比容，白细胞计数），影像学检查（肾脏B超，腹部CT、MRI）。入院时，所有的病人病情活动，表现为梗阻性肾衰竭并需应用双侧输尿管支架保证血清BUN在正常水平。待血BUN降至正常水平后，行输尿管松解术，手术1月后移除输尿管支架，肾功能大约在术后5个月（2-10个月）有所恢复。每3个月随访1次，至少随访1年。

结果：19例病人中，5例男性，14例女性。诊断腹膜后纤维化的平均年龄为50岁（42-62岁）。最常见的症状依次为背痛或腹痛、乏力、体重减轻、少尿、高血压、低热。从出现症状到诊断的时间6-18个月不等。就诊时所有病人病情活动，肾功能不全，血清肌酐水平平均为5.18mg/dl(1-15.4mg/dl)。大部分病人有中度双侧肾积水(2度肾积水)。我们的研究中，所有病人预后良好，78%（15例）病人肾功能完全恢复。肿物轮廓的分形维数与肾功能损害的程度呈正相关，并且肿物的分形维数与CT评分之间呈轻度相关。提示非侵袭性的肿物可能提示更好的预后。

结论：影像学参数不能直接用于判断疾病的严重度，但是肿物表面分形维数的增加提示预后不良。对多数病例而言，双侧输尿管支架植入对于改善肾功能疗效欠佳。尽管入院时存在肾功能不全，但经过双侧输尿管支架植入/肾造口术联合输尿管松解术，大部分病人肾功能可完全恢复。

附原文：Abstract The aim of this study is to evaluate effective prognostic factors in the evolution of patients with retroperitoneal fibrosis and to establish the validity of fractal analysis in determining the disease severity in these patients. MATERIAL AND METHODS: This study included 19 patients (M/F: 5/14) treated for idiopathic retroperitoneal fibrosis and bilateral obstructive renal failure between Jan 2004-Dec 2008. Patients were identified retrospectively, searching for patients diagnosed with IRF, after retroperitoneal biopsy or, in most cases the diagnosis rested on radiological findings, especially CT, with identification of a retroperitoneal mass, the absence of other demonstrable renal or ureteric disease or any other pathology that could explain the findings. CT was very useful in describing the retroperitoneal mass around the aorta and inferior vena cava, the extent of the lesion and for monitoring the response to surgical treatment during the follow-up. The data were evaluated about medical history, physical examination findings, laboratory tests (serum urea and creatinine, blood sugar, sodium, potassium, bicarbonate levels, serum pH, uric acid, haematocrit, white blood cell count), imaging methods (renal ultrasound, abdominal CT-scan, MRI). At admission all patients had active disease with obstructive renal failure and underwent bilateral ureteric stenting in order to normalize the BUN levels. After normalizing of BUN levels, ureterolysis and omental wrapping was performed. Postoperatively, ureteric stents were removed after 1 month and remission of renal disfunction was obtained in approximately 5 months (range 2-10 months). All patients were followed for at least 1 year. Patients were regularly checked every 3 months.RESULTS: Of the 19 patients, there were 5 men and 14 women. The median age at diagnosis of RF was 50 years (range 42-64 years). The most frequent presenting symptoms were back or abdominal pain, weakness, weight loss, oligoanuria, arterial hypertension and mild fever. The duration of symptoms before diagnosis ranged from 6 to 18 months. At presentation all patients had active disease, presenting renal dysfunction with a median serum creatinine of 5.18 mg/dl (range 1-15.4 mg/dl). Most of the patients had moderate bilateral hydronephrosis (2nd degree hydronephrosis). In our study, all patients had excellent prognosis, with full recovery of renal function in 78% of cases (15 patients). The fractal dimension of the fibrosis mass contour correlates with level of renal function impairment. Even more, the fractal dimension seems to slightly variate between CT evaluations (1.30 +/- 0.1), suggesting a non aggressive pattern of extension of the fibrotic mass characteristic for benign lesions.CONCLUSIONS: The imaging parameters did not predict the disease severity, except the increase in fractal dimension of fibrosis surface area. Efficacy of bilateral ureteric stenting in improving renal function is limited in most of the cases. Dispite the level of renal function impairment at admission, full recovery can be achieved after bilateral ureteric stenting/nephrostomy and ureterolisis.

引自：Sinescu I, Surcel C, Mirvald C, Chibelean C, G?ngu C, Avram D, H?rza M, Manu M, Lazar R, Savu C, Udrea A. Prognostic factors in retroperitoneal fibrosis. J Med Life. 2010 Jan-Mar;3(1):19-25.