BACKGROUND: Budd-Chiari syndrome (BCS) refers to posthepatic portal vein hypertension and/or inferior vena cava hypertension syndrome caused by obstruction of the blood flow at the portal cardinal hepatic vein and/or ...BACKGROUND: Budd-Chiari syndrome (BCS) refers to posthepatic portal vein hypertension and/or inferior vena cava hypertension syndrome caused by obstruction of the blood flow at the portal cardinal hepatic vein and/or posterior hepatic inferior vena cava. The main surgical treatments of BCS include operations on pathological lesioned membrane, shunt, and combined operations. There are more than ten treatments available and reports on their therapeutic effects vary. As to operations on lesioned membrane, there are Kimura's finger rupture, balloon dilatation and membrane removal. With reference to our experience, the clinical value of membrane resection at normal temperature and under direct vision is discussed. METHODS: A total of 292 patients with BCS undergoing membrane resection at normal temperature and under direct vision from June 1996 to June 2005 were retrospectively analyzed. RESULTS: The short-term therapeutic effect in 256 patients was satisfactory and the effective rate was 87.7% (256/292). Within a week, ascitic fluid disappeared, the liver shrank and edema of the lower extremities was greatly relieved or even disappeared. Perioperative death occurred in 14 patients (4.8%). Of these, 3 had acute heart failure (one during the operation, one after 6 hours and one 7 days later). Six patients had thoracic cavity bleeding within 12 hours after the operation, 3 had acute respiratory distress syndrome (ARDS), 2 had disseminated intravascular coagulation (DIC), and I had pulmonary embolism. 158 patients were followed up for 6 months to 12 years, and 12 (7.6%) had recurrences. CONCLUSIONS: After membrane resection at normal temperature and under direct vision, hemodynamics was found to be close to normal, damage was slight, effectiveness was evident and the recurrence rate low. So this method is effective in treating BCS.展开更多
Purpose:To investigate the pathological changes of epiretinal membranes(ERM)and internal limiting membrane (ILM) removed during idiopathic macular hole surgery.Methods:Ten consecutive patients with a unilateral idiopa...Purpose:To investigate the pathological changes of epiretinal membranes(ERM)and internal limiting membrane (ILM) removed during idiopathic macular hole surgery.Methods:Ten consecutive patients with a unilateral idiopathic macular hole underwent pars plana vitrectomy(PPV) with the surgical removal of the ERMs overlying the hole and ILM surrounding the hole. The pathological features of the excised tissues were examined under the microscope. Results:According to the morphological changes, four ERMs showed cellular elements which looked like glia cells, macrophages, plasma cells, lymphocytes and fibroblast cells. Two of the ILM appeared as transparent membranes without cellular elements. The other eight ILM showed cellular elements on the transparent membranes.Conclusion: Our study supports the hypothesis that the tangential traction of vitreous and proliferative cellular elements on the inner surface of ILM causes idiopathic macular holes. Removal of the posterior cortical vitreous, ILM and proliferative cellular tissue is a valid treatment for IMH.展开更多
AIM： To report the long-term surgical outcomes of pathologic myopic foveoschisis（MF） following vitrectomy.METHODS： We performed a retrospective case series analysis of 50 consecutive patients diagnosed with MF who...AIM： To report the long-term surgical outcomes of pathologic myopic foveoschisis（MF） following vitrectomy.METHODS： We performed a retrospective case series analysis of 50 consecutive patients diagnosed with MF who experienced vision loss due to progression of foveoschisis.The 50 patients（67 eyes） were treated in our hospital with vitrectomy with internal limiting membrane（ILM） peeling from December 2004 to September 2010.Best corrected visual acuity（BCVA）,refractive error,optical coherence tomography（OCT）,and routine examination results were analysed.The changes of BCVA,foveal anatomical features on OCT scan,and complications were the main outcome measures.RESULTS： The mean follow-up duration was 42±17mo（range 24 to 93mo）.BCVA improved significantly postoperatively（0.76±0.65 logM AR） compared with preoperative baselines（1.31±0.78 log MAR,P〈0.0001）,and in 53 eyes（79%） including 3 lines gain in 44 eyes（66%） at the last follow-up visit.OCT scans showed that central retinal thickness decreased from 580.0±270.0 μm preoperatively（n=67） to 179.7±84.7 μm postoperatively（n=58,P〈0.0001）.Total resolution of foveoschisis occurred in 41 eyes（61%）.Preoperative BCVA correlated well with postoperative BCVA,whereas other factors such as age,axial length,and refractive error were not correlated.The most common complications were cataract and full-thickness macular hole formation in 14 and 9 cases,respectively.CONCLUSION： Patients with progressive vision loss due to MF who were treated with vitrectomy with ILM peelingshow favourable outcomes.In most eyes,visual acuity and foveal structure remain stable during long-term observation.展开更多
Objectives To reveal etiologies of persistent isolated hematuria （PIH） through ultrastructural pathological examination, to disclose clinicopathological correlation in cases with PIH, and to summarize appropriate ma...Objectives To reveal etiologies of persistent isolated hematuria （PIH） through ultrastructural pathological examination, to disclose clinicopathological correlation in cases with PIH, and to summarize appropriate management of patients with PIH. Methods we retrospectively studied 155 P1H patients receiving renal biopsy between January, 2003 and December, 2008 in Peking Union Medical College Hospital. All the clinical data and follow-up result were analyzed. Results All subjects included 38 children and 117 adults, with mean age of 11.38±3.25 years for children and 35.17±8.44 years for adults. Thin basement membrane nephropathy （TBMN） was the most common pathology （55.3% of children and 49.6% of adults）, followed by IgA nephropathy （18.4% of children and 32.5% of adults, mainly grade 2-3） and mesangial proliferative glomerulonephritis （MsPGN） without IgA deposition （13.2% of children and 12.8% of adults）. Besides, A1port syndrome （2.6% of children） and membrane nephropathy （2.6% of children and 0.9% of adults） were demonstrated as other causes of PIH. Elevated mean arteral pressure or protein excretion rate, as well as episodic macrohematuria, indicated higher risk for MsPGN rather than TBMN. On the other hand, severity of microhematuria was irrelevant to pathological types of PIH. Totally, 86 patients were followed up and 37 cases therein stayed on track for long term （mean duration 41.11±28.92 months, range 8-113 months）. Most cases had benign clinical course except 3 cases with TBMN, 5 cases with IgA nephropathy, 1 case with MsPGN （without IgA deposition）, and 1 case with Alport syndrome, who developed hypertension or proteinuria. All of them were administered timely intervention. Conclusions Close follow-up should be required as the primary management for PIH. Equally important is careful monitoring for early identification of undesirable predictors; while renal biopsy and other timely intervention are warranted if there is hypertension, significant proteinuria or renal impairment.展开更多
目的分析比较不同病理分期特发性膜性肾病(IMN)经环磷酰胺(CTX)静脉冲击联合口服糖皮质激素(GC)治疗的疗效情况,为临床治疗IMN提供依据。方法回顾性分析2017年1月—2022年12月某医学院附属和平医院及附属和济医院肾内科经肾穿刺活检确诊...目的分析比较不同病理分期特发性膜性肾病(IMN)经环磷酰胺(CTX)静脉冲击联合口服糖皮质激素(GC)治疗的疗效情况,为临床治疗IMN提供依据。方法回顾性分析2017年1月—2022年12月某医学院附属和平医院及附属和济医院肾内科经肾穿刺活检确诊为IMN且接受CTX静脉冲击联合口服GC治疗患者的临床资料及实验室资料。按照IMN病理分期进行分组,随访12个月,分析比较不同病理分期组IMN患者的基线资料及治疗后各时间点的疗效差异。结果共收集到符合纳入标准的80例IMN患者,其中Ⅰ期组0例,Ⅱ期组65例,Ⅲ期组15例,Ⅳ期组0例。基线资料分析结果:Ⅱ期组和Ⅲ期组在年龄、性别、血压、病程、就诊原因、24 h尿蛋白(24 h UTP)、血清总蛋白(TP)、血清白蛋白(SA)、血肌酐(Scr)、估算肾小球滤过率(eGFR)、血尿酸(UA)、甘油三酯(TG)、总胆固醇(TC)、补体C4、补体C3、免疫球蛋白G(IgG)等指标比较,差异无统计学意义(P>0.05)。CTX+GC治疗后疗效及疗效相关指标分析结果:Ⅱ期组和Ⅲ期组在完全缓解率、部分缓解率、无效率及总缓解率等指标比较,差异无统计学意义(P>0.05)。Ⅱ期组与Ⅲ期组接受治疗后24 h UTP不存在统计学差异(P>0.05);Ⅱ期组和Ⅲ期组的24 h UTP均对时间差异有统计学意义(P<0.05),提示治疗时间越长,24 h UTP有下降趋势。Ⅱ期组和Ⅲ期组接受治疗后SA存在统计学差异(P<0.05),治疗3个月时Ⅲ组SA 34.50(32.40,38.00)g/L,Ⅱ期组SA 31.50(25.95,35.10)g/L,Ⅲ期组高于Ⅱ期组,差异有统计学意义(P<0.05);Ⅱ期组和Ⅲ期组SA均对时间差异有统计学意义(P<0.05),提示治疗时间越长,SA有上升趋势。Ⅱ期组与Ⅲ期组患者接受治疗后Scr不存在统计学差异(P>0.05);Ⅱ期组和Ⅲ期组的Scr均对时间差异无统计学意义(P>0.05)。结论CTX静脉冲击联合口服GC治疗方案对不同病理分期IMN患者均有效,在治疗开始后1年内,随着治疗时间的延长,24 h UTP逐渐降低,SA逐渐升高,但不同病理分期IMN 24 h UTP在同一治疗时间点无统计学差异,其缓解率、Scr亦无统计学意义(P>0.05)。展开更多
目的探讨特发性膜性肾病(IMN)伴免疫球蛋白IgA沉积的临床表现及病理特点,为早期判断IMN病变严重程度提供参考。方法选择2020年1月—2022年12月在本院就诊并经肾脏穿刺活检诊断为IMN的105例患者作为研究对象,按肾小球内有无IgA沉积分为Ig...目的探讨特发性膜性肾病(IMN)伴免疫球蛋白IgA沉积的临床表现及病理特点,为早期判断IMN病变严重程度提供参考。方法选择2020年1月—2022年12月在本院就诊并经肾脏穿刺活检诊断为IMN的105例患者作为研究对象,按肾小球内有无IgA沉积分为IgA阳性组(22例)和IgA阴性组(83例)两组。比较两组的临床及病理资料,包括患者一般情况、临床表现、实验室检查指标、超声引导下肾穿刺活检病理资料等。结果105例IMN患者中男女之比约1.2︰1,平均发病年龄为(47.19±12.6)岁,临床首发症状以“水肿”为主,伴镜下血尿患者占比高,病理分期以膜性肾病Ⅱ期为主;IgA阴性组患者的24 h U-TP水平高于IgA阳性组,差异有统计学意义(P<0.05);IgA阳性组患者的补体C3指标均低于IgA阴性组,差异有统计学意义(P<0.05);IgA阳性组患者病理分期损伤较IgA阴性组更严重,差异有统计学意义(P<0.05);IgA阳性组患者的肾小球细胞内增生比例高于IgA阴性组,差异有统计学意义(P<0.05);IgA阴性组患者的肾小管萎缩、肾间质炎症细胞浸润比例高于IgA阳性组,差异有统计学意义(P<0.05);IgA阳性组患者的免疫荧光指标中PLA2R沉积比例低于IgA阴性组,差异有统计学意义(P<0.05)。结论105例IMN患者中男女之比约1.2︰1,平均发病年龄为(47.19±12.6)岁,临床首发症状以“水肿”为主,伴镜下血尿患者占比高,病理分期以膜性肾病Ⅱ期为主;IgA阴性组患者的24h U-TP水平高于IgA阳性组,差异有统计学意义(P<0.05);IgA阳性组患者的补体C3指标均低于IgA阴性组,差异有统计学意义(P<0.05);IgA阳性组患者病理分期损伤较IgA阴性组更严重,差异有统计学意义(P<0.05);IgA阳性组患者的肾小球细胞内增生比例高于IgA阴性组,差异有统计学意义(P<0.05);IgA阴性组患者的肾小管萎缩、肾间质炎症细胞浸润比例高于IgA阳性组,差异有统计学意义(P<0.05);IgA阳性组患者的免疫荧光指标中PLA2R沉积比例低于IgA阴性组,差异有统计学意义(P<0.05)。展开更多
文摘BACKGROUND: Budd-Chiari syndrome (BCS) refers to posthepatic portal vein hypertension and/or inferior vena cava hypertension syndrome caused by obstruction of the blood flow at the portal cardinal hepatic vein and/or posterior hepatic inferior vena cava. The main surgical treatments of BCS include operations on pathological lesioned membrane, shunt, and combined operations. There are more than ten treatments available and reports on their therapeutic effects vary. As to operations on lesioned membrane, there are Kimura's finger rupture, balloon dilatation and membrane removal. With reference to our experience, the clinical value of membrane resection at normal temperature and under direct vision is discussed. METHODS: A total of 292 patients with BCS undergoing membrane resection at normal temperature and under direct vision from June 1996 to June 2005 were retrospectively analyzed. RESULTS: The short-term therapeutic effect in 256 patients was satisfactory and the effective rate was 87.7% (256/292). Within a week, ascitic fluid disappeared, the liver shrank and edema of the lower extremities was greatly relieved or even disappeared. Perioperative death occurred in 14 patients (4.8%). Of these, 3 had acute heart failure (one during the operation, one after 6 hours and one 7 days later). Six patients had thoracic cavity bleeding within 12 hours after the operation, 3 had acute respiratory distress syndrome (ARDS), 2 had disseminated intravascular coagulation (DIC), and I had pulmonary embolism. 158 patients were followed up for 6 months to 12 years, and 12 (7.6%) had recurrences. CONCLUSIONS: After membrane resection at normal temperature and under direct vision, hemodynamics was found to be close to normal, damage was slight, effectiveness was evident and the recurrence rate low. So this method is effective in treating BCS.
文摘Purpose:To investigate the pathological changes of epiretinal membranes(ERM)and internal limiting membrane (ILM) removed during idiopathic macular hole surgery.Methods:Ten consecutive patients with a unilateral idiopathic macular hole underwent pars plana vitrectomy(PPV) with the surgical removal of the ERMs overlying the hole and ILM surrounding the hole. The pathological features of the excised tissues were examined under the microscope. Results:According to the morphological changes, four ERMs showed cellular elements which looked like glia cells, macrophages, plasma cells, lymphocytes and fibroblast cells. Two of the ILM appeared as transparent membranes without cellular elements. The other eight ILM showed cellular elements on the transparent membranes.Conclusion: Our study supports the hypothesis that the tangential traction of vitreous and proliferative cellular elements on the inner surface of ILM causes idiopathic macular holes. Removal of the posterior cortical vitreous, ILM and proliferative cellular tissue is a valid treatment for IMH.
基金Supported by the National Key Basic Research Program of China(No.2013CB967503)
文摘AIM： To report the long-term surgical outcomes of pathologic myopic foveoschisis（MF） following vitrectomy.METHODS： We performed a retrospective case series analysis of 50 consecutive patients diagnosed with MF who experienced vision loss due to progression of foveoschisis.The 50 patients（67 eyes） were treated in our hospital with vitrectomy with internal limiting membrane（ILM） peeling from December 2004 to September 2010.Best corrected visual acuity（BCVA）,refractive error,optical coherence tomography（OCT）,and routine examination results were analysed.The changes of BCVA,foveal anatomical features on OCT scan,and complications were the main outcome measures.RESULTS： The mean follow-up duration was 42±17mo（range 24 to 93mo）.BCVA improved significantly postoperatively（0.76±0.65 logM AR） compared with preoperative baselines（1.31±0.78 log MAR,P〈0.0001）,and in 53 eyes（79%） including 3 lines gain in 44 eyes（66%） at the last follow-up visit.OCT scans showed that central retinal thickness decreased from 580.0±270.0 μm preoperatively（n=67） to 179.7±84.7 μm postoperatively（n=58,P〈0.0001）.Total resolution of foveoschisis occurred in 41 eyes（61%）.Preoperative BCVA correlated well with postoperative BCVA,whereas other factors such as age,axial length,and refractive error were not correlated.The most common complications were cataract and full-thickness macular hole formation in 14 and 9 cases,respectively.CONCLUSION： Patients with progressive vision loss due to MF who were treated with vitrectomy with ILM peelingshow favourable outcomes.In most eyes,visual acuity and foveal structure remain stable during long-term observation.
文摘Objectives To reveal etiologies of persistent isolated hematuria （PIH） through ultrastructural pathological examination, to disclose clinicopathological correlation in cases with PIH, and to summarize appropriate management of patients with PIH. Methods we retrospectively studied 155 P1H patients receiving renal biopsy between January, 2003 and December, 2008 in Peking Union Medical College Hospital. All the clinical data and follow-up result were analyzed. Results All subjects included 38 children and 117 adults, with mean age of 11.38±3.25 years for children and 35.17±8.44 years for adults. Thin basement membrane nephropathy （TBMN） was the most common pathology （55.3% of children and 49.6% of adults）, followed by IgA nephropathy （18.4% of children and 32.5% of adults, mainly grade 2-3） and mesangial proliferative glomerulonephritis （MsPGN） without IgA deposition （13.2% of children and 12.8% of adults）. Besides, A1port syndrome （2.6% of children） and membrane nephropathy （2.6% of children and 0.9% of adults） were demonstrated as other causes of PIH. Elevated mean arteral pressure or protein excretion rate, as well as episodic macrohematuria, indicated higher risk for MsPGN rather than TBMN. On the other hand, severity of microhematuria was irrelevant to pathological types of PIH. Totally, 86 patients were followed up and 37 cases therein stayed on track for long term （mean duration 41.11±28.92 months, range 8-113 months）. Most cases had benign clinical course except 3 cases with TBMN, 5 cases with IgA nephropathy, 1 case with MsPGN （without IgA deposition）, and 1 case with Alport syndrome, who developed hypertension or proteinuria. All of them were administered timely intervention. Conclusions Close follow-up should be required as the primary management for PIH. Equally important is careful monitoring for early identification of undesirable predictors; while renal biopsy and other timely intervention are warranted if there is hypertension, significant proteinuria or renal impairment.
文摘目的分析比较不同病理分期特发性膜性肾病(IMN)经环磷酰胺(CTX)静脉冲击联合口服糖皮质激素(GC)治疗的疗效情况,为临床治疗IMN提供依据。方法回顾性分析2017年1月—2022年12月某医学院附属和平医院及附属和济医院肾内科经肾穿刺活检确诊为IMN且接受CTX静脉冲击联合口服GC治疗患者的临床资料及实验室资料。按照IMN病理分期进行分组,随访12个月,分析比较不同病理分期组IMN患者的基线资料及治疗后各时间点的疗效差异。结果共收集到符合纳入标准的80例IMN患者,其中Ⅰ期组0例,Ⅱ期组65例,Ⅲ期组15例,Ⅳ期组0例。基线资料分析结果:Ⅱ期组和Ⅲ期组在年龄、性别、血压、病程、就诊原因、24 h尿蛋白(24 h UTP)、血清总蛋白(TP)、血清白蛋白(SA)、血肌酐(Scr)、估算肾小球滤过率(eGFR)、血尿酸(UA)、甘油三酯(TG)、总胆固醇(TC)、补体C4、补体C3、免疫球蛋白G(IgG)等指标比较,差异无统计学意义(P>0.05)。CTX+GC治疗后疗效及疗效相关指标分析结果:Ⅱ期组和Ⅲ期组在完全缓解率、部分缓解率、无效率及总缓解率等指标比较,差异无统计学意义(P>0.05)。Ⅱ期组与Ⅲ期组接受治疗后24 h UTP不存在统计学差异(P>0.05);Ⅱ期组和Ⅲ期组的24 h UTP均对时间差异有统计学意义(P<0.05),提示治疗时间越长,24 h UTP有下降趋势。Ⅱ期组和Ⅲ期组接受治疗后SA存在统计学差异(P<0.05),治疗3个月时Ⅲ组SA 34.50(32.40,38.00)g/L,Ⅱ期组SA 31.50(25.95,35.10)g/L,Ⅲ期组高于Ⅱ期组,差异有统计学意义(P<0.05);Ⅱ期组和Ⅲ期组SA均对时间差异有统计学意义(P<0.05),提示治疗时间越长,SA有上升趋势。Ⅱ期组与Ⅲ期组患者接受治疗后Scr不存在统计学差异(P>0.05);Ⅱ期组和Ⅲ期组的Scr均对时间差异无统计学意义(P>0.05)。结论CTX静脉冲击联合口服GC治疗方案对不同病理分期IMN患者均有效,在治疗开始后1年内,随着治疗时间的延长,24 h UTP逐渐降低,SA逐渐升高,但不同病理分期IMN 24 h UTP在同一治疗时间点无统计学差异,其缓解率、Scr亦无统计学意义(P>0.05)。
文摘目的探讨特发性膜性肾病(IMN)伴免疫球蛋白IgA沉积的临床表现及病理特点,为早期判断IMN病变严重程度提供参考。方法选择2020年1月—2022年12月在本院就诊并经肾脏穿刺活检诊断为IMN的105例患者作为研究对象,按肾小球内有无IgA沉积分为IgA阳性组(22例)和IgA阴性组(83例)两组。比较两组的临床及病理资料,包括患者一般情况、临床表现、实验室检查指标、超声引导下肾穿刺活检病理资料等。结果105例IMN患者中男女之比约1.2︰1,平均发病年龄为(47.19±12.6)岁,临床首发症状以“水肿”为主,伴镜下血尿患者占比高,病理分期以膜性肾病Ⅱ期为主;IgA阴性组患者的24 h U-TP水平高于IgA阳性组,差异有统计学意义(P<0.05);IgA阳性组患者的补体C3指标均低于IgA阴性组,差异有统计学意义(P<0.05);IgA阳性组患者病理分期损伤较IgA阴性组更严重,差异有统计学意义(P<0.05);IgA阳性组患者的肾小球细胞内增生比例高于IgA阴性组,差异有统计学意义(P<0.05);IgA阴性组患者的肾小管萎缩、肾间质炎症细胞浸润比例高于IgA阳性组,差异有统计学意义(P<0.05);IgA阳性组患者的免疫荧光指标中PLA2R沉积比例低于IgA阴性组,差异有统计学意义(P<0.05)。结论105例IMN患者中男女之比约1.2︰1,平均发病年龄为(47.19±12.6)岁,临床首发症状以“水肿”为主,伴镜下血尿患者占比高,病理分期以膜性肾病Ⅱ期为主;IgA阴性组患者的24h U-TP水平高于IgA阳性组,差异有统计学意义(P<0.05);IgA阳性组患者的补体C3指标均低于IgA阴性组,差异有统计学意义(P<0.05);IgA阳性组患者病理分期损伤较IgA阴性组更严重,差异有统计学意义(P<0.05);IgA阳性组患者的肾小球细胞内增生比例高于IgA阴性组,差异有统计学意义(P<0.05);IgA阴性组患者的肾小管萎缩、肾间质炎症细胞浸润比例高于IgA阳性组,差异有统计学意义(P<0.05);IgA阳性组患者的免疫荧光指标中PLA2R沉积比例低于IgA阴性组,差异有统计学意义(P<0.05)。
