常染色体隐性遗传性多囊肾病人的肾脏钙化:流行与病因

常染色体隐性遗传性多囊肾是一种遗传性疾病。作者回顾性研究了9例确诊病人的临床影象学特征,诊断标准是:典型影象学表现,如肾增大;排泄性尿路造影对比剂在髓质呈条纹状表现;超声肾回声质地弥漫增加,肾边界不清。提示性影象学征象有肾或肝脏组织活检结果阳性;同胞中也有该病病人。9例中女6例,男3例,年龄2个月～15岁。行临床检查,US、CT和肾功能试验。US扫描使用5或3.5MHz扇形探头。除2例外,CT扫描     

Tolosa—Hunt综合征的病理、临床及影像学表现

Tolosa—Hunt综合征是一种少见的神经内科及眼科疾病，发病率较低。就Tolosa-Hunt综合征的病理、临床及影像学表现进行综述。     

肾移植后肺部真菌感染病理及影像学表现

肾移植术后肺部真菌感染发病率不断增加，各致病菌的胸部影像学表现相互重叠较多，综述不同真菌肺部感染的病理、影像学表现，着重总结特征性表现，以提高对各类真菌感染评价的准确性。     

特发性肺纤维化的临床与影像学表现

特发性肺纤维化(Idiopathic pulmonary Fibrosis．IPF)为一种原因不明的慢性肺间质性疾病，临床上起病隐袭，呈进行性加重，由于肺大量纤维化．最终将导致肺、心功能衰竭。     

肾移植后肺部真菌感染病理及影像学表现

肾移植术后肺部真菌感染发病率不断增加,各致病菌的胸部影像学表现相互重叠较多,综述不同真菌肺部感染的病理、影像学表现,着重总结特征性表现,以提高对各类真菌感染评价的准确性.     

肺原发性脑膜瘤的组织病理与影像学表现

肺原发性脑膜瘤(primary pulmonary meningioma．PPM)又称肺异位脑膜瘤(Lung ectopic meningioma)，是于中枢神经系统以外、原发于肺内的异位脑膜瘤，为肺内极罕见的肿瘤。为对本病有较全面的了解，笔者复习了以往文献，现综述如下。     

淋巴管平滑肌瘤病的临床、病理及影像学表现

淋巴管平滑肌瘤病(lymphangioleiomyomatosis,LAM)是一种少见病,特点是平滑肌细胞在终末细支气管、淋巴管、小血管周围异常增生[1],而产生一系列临床症状和体征.病因和病理机制不明.国内见个案报告[2～13].     

评《骨放射学正常与早期病理表现的界定》

骨关节正常，包括正常变异的影像学表现与病变早期影像学变化的辨认、界定非常困难，但对发现早期病变和诊断十分重要。CT与MRI的广泛应用，加深了对骨关节正常变异与病变早期影像学表现的认识，为解决这一难题提供了条件。德国著名骨放射学专家Juergen Freyschmidt教授等主编的《Borderlands of Normal and Early Pathological Findings in Skeletal Radiolography》即《骨放射学正常与早期病理表现的界定》，就是介绍这方面的专著，为国际上公认的权威著作。     

骨嗜酸性肉芽肿的影像学表现与病理对照分析

目的研究骨嗜酸性肉芽肿(EGB)影像学表现与生物学行为相关性,提高对EGB的认识。方法回顾分析31例EGB的影像表现及病理特征。结果影像表现:早期病灶5例(16.1%),中期病灶23例(74.2%),晚期病灶3例(9.7%),病理所见:早期病灶3例(9.7%),中期病灶26例(83.9%),晚期病灶2例(6.4%)。结论EGB影像学表现与病理过程有较一致的相对应关系,只是早期病理改变略早于X线表现,EGB影像学分期有利于指导临床治疗。     

两种透析方式对常染色体显性多囊肾病预后影响

目的比较血液透析与腹膜透析对进入终末期肾病(ESRD)的常染色体显性多囊肾病(ADPKD)患者预后的影响。方法回顾性分析自1999年3月至2014年8月第二军医大学附属长海医院肾内科收治的43例ADPKD患者的临床资料。根据初始透析方法的不同,将43例患者分为血液腹膜透析组(n=20)及血液透析组(n=23)。随访期间,记录心血管事件发生情况,并对两组患者心血管事件发生率进行比较。以全因死亡为终点事件,绘制生存曲线,采用Kaplan Meier法和LogRank检验对两组患者存活率进行比较。结果本研究共43例患者,最长随访147个月。腹膜透析组心血管事件发生率5.0%(1/20),血液透析组26.1%(6/23),两组间比较,差异有统计学意义(P<0.01)。随访期内,腹膜透析组患者,存活率85.0%(17/20),血液透析组存活率73.9%(17/23),两组间比较,差异无统计学意义(P>0.05)。结论腹膜透析与血液透析均是ADPKD肾衰竭患者的有效肾替代治疗方法。与腹膜透析比较,血液透析患者心血管事件发生概率较低。     

胎儿常染色体隐性遗传性多囊肾的MRI表现(附10例分析)

目的：探讨胎儿双侧常染色体隐性遗传性多囊肾（ARPKD）的MRI表现及其诊断价值。方法：对10例经超声和病理证实的胎儿双侧ARPKD的MRI影像资料进行回顾性分析。结果：①8例双肾体积明显均匀增大,但肾外形保持;②T2WI上,1例双肾信号呈中等以上增高,均匀一致;8例双肾皮髓质内见弥漫性针尖大小信号增高的囊泡影,呈放射状排列,笔者称为＂苦瓜样＂改变,1例信号正常;③羊水无法显示5例,羊水极少5例;④8例未见膀胱显示;⑤合并9种畸形,其中前脑无裂畸形1例,脑积水2例,Dandy-Walker综合征2例,枕部脑膜膨出2例,颅内出血并孔洞脑1例,肺发育不良5例,多发性肝囊肿1例,唇裂1例,Meckel-Gruber综合征1例。结论：ARPKD的MRI表现具有特异性,且不受羊水量和胎儿体位的影响,可作为产前超声检查的重要补充手段,并提高其合并畸形的检出率。     

Autosomal recessive polycystic kidney disease: radiologic-pathologic correlation.

Autosomal recessive polycystic kidney disease is a heritable but phenotypically variable disorder characterized by varying degrees of nonobstructive renal collecting duct ectasia, hepatic biliary duct ectasia and malformation, and fibrosis of both liver and kidneys. In the kidney, the dilated collecting ducts and interstitial fibrosis, when severe, may significantly impair renal function and result in hypertension and renal failure. Imaging typically shows large but reniform kidneys, diffusely increased renal parenchymal echogenicity at ultrasonography, and a striated nephrogram after contrast material administration. In the liver, periportal fibrosis accompanies the malformed and dilated bile ducts; this may result in portal hypertension. The liver may appear normal or may show intrahepatic biliary dilatation; once portal hypertension develops, splenomegaly and varices are usually evident. The relative degrees of kidney and liver involvement tend to be inverse: Children with severe renal disease usually have milder hepatic disease, and those with severe hepatic disease tend to evidence mild renal impairment. Presently, treatment consists of supportive management and control of hypertension. Replacement therapy for renal failure (dialysis or kidney transplantation) and control of portal hypertension (portal circulatory diversion or liver transplantation) may be necessary.     

Autosomal dominant polycystic kidney disease: MR imaging evaluation using current techniques

PURPOSE: To determine the MR imaging findings of autosomal dominant polycystic kidney disease using current imaging techniques. MATERIALS AND METHODS: We reviewed our five-year experience with MR imaging of autosomal dominant polycystic kidney disease (ADPKD) to determine the spectrum of appearance of kidney disease, the occurrence of cysts in other abdominal organs, the size and number of cysts in the kidneys and other organs, and the association with other benign or malignant disease. Thirty patients (17 men and 13 women, age range 30 to 88 years old) with ADPKD were included in this study. All patients were examined by MR imaging including T2-weighted single-shot echo-train spin-echo and pre- and post-gadolinium chelate spoiled gradient-echo imaging. RESULTS: All kidneys were involved with multiple, varying sized cysts scattered throughout the parenchyma. Giant renal cysts (>8 cm) were associated with pain in the only two patients who possessed them. Hemorrhage in renal cysts was observed in all kidneys with a heterogeneous pattern of involvement on non-contrast T1- and T2-weighted images, reflecting hemorrhage of varying age. The mean kidney size for the right kidney was 17.4 cm in length, 10.3 cm in transverse, and 9.4 cm in antero-posterior diameter (AP); and for the left kidney, 15.9 cm in the length, 9.3 cm in the transverse, and 9.3 cm in AP diameter. Other organs involved included the liver (22 patients), the pancreas (three patients), with two of the above-mentioned patients having both liver and pancreas cysts, and the spleen (one patient) who had both liver and splenic cysts. Massive liver involvement with large cysts was associated with abdominal pain. Malignant disease was present in five patients, including two patients with renal cell carcinoma, one with bladder cancer, one with lung cancer, and one patient with anal adenocarcinoma. Comparison of pre- and post-contrast T1-weighted images was essential to detect renal cancer. CONCLUSION: All kidneys in patients with ADPKD had extensive, varying-sized cysts and in all cases some cysts showed evidence of hemorrhage. The liver was the second most common organ to be involved with cystic disease, in 73% of patients. Large cysts in the kidneys and liver were associated with abdominal pain.     

Primary adrenal lymphoma: radiological; pathological, clinical correlation

Objective

The purpose of this study was to analyze CT and MR imaging (MRI) manifestation of primary adrenal lymphoma in order to better understand of this rare disease.

Materials and methods

Six patients (4 men, 2 women; median age, 65.5 years) with pathologically proven primary adrenal lymphoma were retrospectively reviewed. Imaging findings (CT&;MRI, n = 3; only CT, n = 3) were analyzed and correlated with clinical and pathologic findings.

Results

All cases were pathologically proven primary adrenal diffuse large B-cell lymphoma with bilateral lesions in 3 cases respectively. Maximum diameters of the 9 lesions were 6.1–14.8 cm, median 10.5 cm. Seven lesions were round, oval or oblong, and 2 lesions irregular. Seven lesions were well-defined and 2 had ill-defined margins. Unenhanced CT density of 9 lesions and MR signal intensity of T1 weighted images of 6 lesions were similar to that of muscle, and all 6 lesions were hyperintense on T2-weighted MR images. Following intravenous injection of contrast media, 6 lesions had mild enhancement and 3 lesions had moderate enhancement on parenchymal phase imaging. Enhancement was homogenous in three, slightly inhomogeneous in four, and heterogeneous in two. Seven lesions, in 5 cases, infiltrated the adjacent tissues or organs in patients with large tumors.

Conclusion

Primary adrenal lymphoma usually manifests as large, well-defined, soft-tissue masses replacing the adrenal gland with homogeneous or slightly inhomogeneous enhancement. Large tumors especially tend to infiltrate adjacent structures.     

Autosomal recessive (infantile) polycystic kidney disease demonstrated by Tc-99m DMSA renal imaging.

A neonate with infantile polycystic kidney disease underwent Tc-99m DMSA imaging. The pattern of uptake in infantile polycystic disease is different from the multiple cystic lesions reported in the literature for adult polycystic kidney disease. The infantile pattern of uptake shows large kidneys with diffuse, symmetric localization of the radiopharmaceutical, which seems to be characteristic and may be pathognomonic of the disease process.     

Pseudolipoma of inverted Meckel's diverticulum: clinical, radiological and pathological correlation

Three cases of isolated inverted Meckel's diverticulum are described. In two cases an initial pathological diagnosis of small bowel lipoma was suggested. In a third case central fat was demonstrated on CT and peristalsis of the intraluminal polypoid mass was observed during US examination. In all three cases small bowel enema examination demonstrated the lesion. Correlation of the clinical, radiological and pathological features is emphasised, as this will allow the correct diagnosis. Received 19 August 1996; Revision received 4 November 1996; Accepted 8 November 1996     

The choledochocele: correlation of radiological, clinical and pathological findings.

Two cases of choledochocele are presented and 14 cases in the literature reviewed. Choledochocele is defined as a herniation of the common bile duct into the duodenum. This entity is distinguishable radiographically from duodenal diverticulum and duodenal duplication cyst by filling during cholangiography but not during upper gastrointestinal series. The duodenal diverticulum fills on upper gastrointestinal series but not on cholangiography. The duplication cyst will not fill with either method.     

Renal xanthogranulomatosis: radiological, clinical, and pathological features in two cases

Xanthogranulomatosis is a multicentric or systemic proliferative disorder of histiocytes. Two cases of xanthogranulomas are reported, each involving the kidneys. One patient also had concomitant orbital and CNS involvement. Primary renal involvement without associated retroperitoneal masses is an uncommon manifestation of this disorder. The CT appearance of renal involvement in both patients was distinctive and remarkably similar. Both patients had bilaterally enlarged kidneys in which the renal sinus fat was replaced by a fibrotic process that caused mild calicectasis. The clinical and roentgenographic features of xanthogranulomatosis bear a close resemblance to those seen in two fibrosclerosing syndromes: sinus histiocytosis with massive lymphadenopathy and retroperitoneal fibrosis.