Hospital management of abdominal trauma in Tehran, Iran: a review of 228 patients

Objective: Today, trauma is a major public health problem in some countries. Abdominal trauma is the source of significant mortality and morbidity with both blunt and penetrating injuries. We performed an epidemiological study of abdominal trauma (AT) in Tehran, Iran. We used all our sources to describe the epidemiology and outcome of patients with AT.Methods: This study was done in Tehran. The study population included trauma patients admitted to the emergency department of six general hospitals in Tehran during one year. The data were collected through a questionnaire that was completed by a trained physician at the trauma center. The statistical analysis was performed using the SPSS software (version 11.5 for Windows). The statistical analysis was conducted using the chi-square and P＜0.05 was accepted as being statistically significant.Results: Two hundred and twenty-eight (2.8%) out of 8 000 patients were referred to the above mentioned centers with abdominal trauma. One hundred and twenty-five (54.9%)of the patients were in their 2nd and 3rd decades of life and 189 (83%) of our patients were male. Road traffic accidents (RTA) were the leading cause of AT with 119 (52.2%) patients. Spleen was the commonly injured organ with 51 cases. Following the analysis of injury severity, 159 (69.7%) patients had mild injuries (ISS＜16) and 69 (30.3%) patients had severe injuries (ISS= 16). The overall mortality rate was 46 (20.2%).Conclusions: Blunt abdominal trauma is more common than penetrating abdominal trauma. Road traffic accidents and stab wound are the most common causes of blunt and penetrating trauma, respectively. Spleen is the most commonly injured organ in these patients. The mortality rate is higher in blunt trauma than penetrating one.     

胃黏膜相关淋巴组织淋巴瘤的临床病理分析

Objective To analyze the clinicopathological characteristics, immunophenotypes and immu-noglobulin heavy chain gene rearrangement of gastric mucosa-associated lymphoid tissue (MALT) lymphoma. Methods The clinical data of 35 patients with gastric MALT lymphoma who had been admitted to Southwest Hospital from January 1984 to June 2003 were retrospectively analyzed. The specimens of the gastric MALT lymphoma were obtained and their pathomorphological classifications were studied. The immunophenotypes and immunoglobulin heavy chain gene rearrangement were detected by immunohistochemistry and polymerase chain reaction, respectively. All the data were analyzed by chi-square test and Mann-Whitney U test. Results The main manifestations of patients with gastric MALT lymphoma were epigastric discomfort and abdominalgia. The percentage of patients with age≥40 accounted for 74% (26/35). The gastric MALT lyrnphoma has low differentia-tion in 32 patients, and high differentiation in 3 patients. There were 31 patients in Ⅰ E stage, 2 in Ⅱ E1 stage and 2 in Ⅳ stage. Pathological examination showed that gastric MALT lymphoma was composed of centrocyte-like cells and mononuclear-like B cells or lymphoplasma cells. Twenty-nine specimens were with lymphoepithelial lesions (83%), 2 with follicular colonization (6%), and 11 with plasma cells segregated beneath the surface epithelium (33%). All the 35 specimens were B cell immunotype. Restrictive expression of κ and λ light chains of the immunoglobulin was found in 19 cases, and Bcl-2 positive expression was found in 16 cases. High expres-sion of proliferating cell nuclear antigen was found in gastric MALT lymphoma with transformed blasts, which indicated that gastric MALT lymphoma with transformed blasts was more active in proliferation than those of low grade. A monoclonal pattern of immunoglobulin heavy chain gene rearrangement was detected in 33 patients (94%). Conclusions Digestive symptoms are the main manifestations of patients with gastric MALT lymphoma. The incidence of gastric MALT lymphoma increases with age. The final diagnosis of gastric MALT lymphoma depends on the pathological, immunohistochemical examination and laboratory test.     

Human papillomavirus and p53 protein immunoreactivity in condylomata acuminatum and squamous cell carcinoma of penis

Aim: To determine the immunoreactive pattern of human papillomavirus (HPV) antigen and p53 protein in condylomata acuminatum (CA) and squamous cell carcinoma (SCC) of penis. Methods: Immunohistochemistry for HPV and p53 were performed in 40 specimens of formalin fixed, paraffin embedded tissues using a polyclonal (rabbit) antibody against HPV and a monoclonal (mouse) antibody against human p53 protein. Twenty one cases of CA and nineteen cases of SCC were examined. Results: HPV antigen was detected in all 21 CA and 2 penile SCC. p53 protein overexpression was observed in 12 of 19 (63%) SCC in which 6 cases were strong positive. Five of 21 CA (24%) showed low-grade p53 protein overexpression. Conclusion: CA is related to HPV infection and some cases show p53 protein low-grade overexpression. In contrast, p53 protein overexpression is common in penile SCC, which is seldom related to HPV infection.     

汞中毒相关肾小球疾病的临床病理分析

Objective To elucidate the clinical and pathological characteristics of patients with mercury poisoning-associated glomerulonephropathy. Methods Seven patients with mercury poisoning-associated glomerulonephropathy were enrolled in this study. The pattern of mercury exposure, feature of mercury toxicity, and clinicopathological presentation of the kidneys were investigated. Results They were all female, averaged (28.9 ±8.1) years old. Skin-whitening cream was the only cause of mercury poisoning. Proteinuria occurred 5 to 8 months after exposure. Serum mercury were 27.0 to 98.0 μg/L, and spot urinary mercury were 34.4 to 204.0 μg/L. The presentation of all the patients was mild to moderate edema with proteinuria and decreased serum albumin level. Five patients (5/7) were diagnosed as nephrotic syndrome. Six patients underwent renal biopsy: 3 cases with minimal change disease, 2 cases with membranous nephropathy and 1 case with focal segmental glomerular sclerosis. All the patients were administrated chelation therapy with sodium dimercaptopropanal sulfonate or sodium dimercaptosuccinic acid for 3 to 7 courses. They got complete remission by 3 to 5 weeks treatment. Conclusions Patients in this study with glomerulonephropathy induced by mercury poisoning are all from skin-whitening cream exposure. Mild to moderate edema and proteinuria are the common clinical pattern. Minimal change disease, membranous nephropathy and focal segmental glomerular sclerosis are found pathologically. Chelation therapy is effective.     

汞中毒相关肾小球疾病的临床病理分析

Objective To elucidate the clinical and pathological characteristics of patients with mercury poisoning-associated glomerulonephropathy. Methods Seven patients with mercury poisoning-associated glomerulonephropathy were enrolled in this study. The pattern of mercury exposure, feature of mercury toxicity, and clinicopathological presentation of the kidneys were investigated. Results They were all female, averaged (28.9 ±8.1) years old. Skin-whitening cream was the only cause of mercury poisoning. Proteinuria occurred 5 to 8 months after exposure. Serum mercury were 27.0 to 98.0 μg/L, and spot urinary mercury were 34.4 to 204.0 μg/L. The presentation of all the patients was mild to moderate edema with proteinuria and decreased serum albumin level. Five patients (5/7) were diagnosed as nephrotic syndrome. Six patients underwent renal biopsy: 3 cases with minimal change disease, 2 cases with membranous nephropathy and 1 case with focal segmental glomerular sclerosis. All the patients were administrated chelation therapy with sodium dimercaptopropanal sulfonate or sodium dimercaptosuccinic acid for 3 to 7 courses. They got complete remission by 3 to 5 weeks treatment. Conclusions Patients in this study with glomerulonephropathy induced by mercury poisoning are all from skin-whitening cream exposure. Mild to moderate edema and proteinuria are the common clinical pattern. Minimal change disease, membranous nephropathy and focal segmental glomerular sclerosis are found pathologically. Chelation therapy is effective.     

肾盂鳞状细胞癌临床特点分析

目的 探讨肾盂鳞状细胞癌的诊治特点.方法 回顾性分析1991年10月至2009年5月收治8例肾盂鳞状细胞癌患者资料.临床表现血尿8例,腰痛7例,腹部包块1例.B超检查8例,IVU检查8例,CT检查4例.术前诊断为肿瘤3例,诊断为肾结石5例,结石术中发现肿瘤并经冰冻病理确诊2例.8例患者均经手术治疗,行根治性肾输尿管切除4例、单纯性肾切除3例、姑息性切除术1例.结果 8例病理诊断均为鳞状细胞癌.中分化6例,高分化和低分化各1例;pT1 1例,pT2 1例,pT3 3例,pT4 3例;淋巴结转移2例.获随访7例,失访1例.术后生存时间2～42个月,中位时间6个月,患者均死于肿瘤复发及转移.结论 肾盂鳞状细胞癌恶性程度高,常合并结石,术前诊断困难,确诊时多为中晚期,术后短期内易复发转移,预后极差.

Abstract：

Objective To review the diagnosis and treatment of squamous cell carcinoma of renal pelvis. Methods The clinical data from October 1991 to May 2009 of eight cases of squamous cell carcinoma of renal pelvis were reviewed and analyzed retrospectively. The symptoms of the patients were hematuria (eight cases), pain (seven cases) and abdominal mass (one case). All patients underwent B-ultrasound and IVU examination and four cases underwent CT scan. Three cases were diagnosed as having a tumor before surgery. Five cases were diagnosed as renal calculus, two of the five cases were diagnosed by intraoperative frozen section. Radical nephroureterectomy were performed in four cases, nephrectomy in three cases and palliative resection in one case. Results Histological classification revealed that six cases were moderately differentiated, one case was well differentiated and one case was poorly differentiated. Two cases had stage pT1/pT2 and six cases had stage pT3/pT4. 2 cases had regional lymph nodes metastasis. Seven cases were followed-up. All patients died of tumor recurrence or metastasis. The median tumor specific survive time was six months (range from two months to 42 months). Conclusions Squamous cell carcinoma of renal pelvis is often occurs concurrently with urolithiasis which could lead to difficulty in diagnose before operation. As the most of the patients were diagnosed with advanced stage disease, squamous cell carcinoma of renal pelvis tended to early recurrence and metastasis and the prognosis was very poor.     

An anterolateral thigh perforator chimeric flap combined with calcium sulfate impregnated with vancomycin for treatment of chronic fracture-related infection complicated with extensive soft-tissue defects北大核心CSCD

Objective To investigate the efficacy of an anterolateral femoral chimeric perforator flap combined with vancomycin-loaded calcium sulfate in the treatment of chronic infection after internal fixation of calf fracture with soft tissue defects. Methods Retrospectively analyzed were the data of 16 patients with chronic infection combined with extensive soft tissue defects after internal fixation of calf fracture who had been admitted to Department of Orthopedics, Shenzhen Hospital Affiliated to Peking University from September 2008 to November 2020. There were 11 males and 5 females, aged from 16 to 62 years (average, 37 years). Infection sites: the upper tibia in 4 cases, the middle and lower tibia in 10 cases, and the middle fibula in 2 cases. According to the Cierny-Mader classification, all patients were anatomical type III and by the host classification, there were 14 cases of type B and 2 cases of type C type. The areas of soft tissue defects ranged from 6 cm × 4 cm to 23 cm × 14 cm. All patients were treated by transplantation of an anterolateral thigh chimeric perforator flap combined with vancomycin-loaded calcium sulfate therapy. At the last follow-up, the curative efficacy was evaluated according to the Paley fracture union scoring. Results All patients were followed up for 8 to 24 months (mean, 16 months). Complete flap survival was achieved in 15 flaps and partial survival in one. According to the Paley fracture union scoring at the last follow-up, the curative efficacy was evaluated as excellent in 15 cases and as good in one. Both the grafted artificial bone and the tibia and fibula achieved bone union after 6 to 12 months (mean, 8.9 months). Infection with chronic sinus tract pus recurred in one case at post-operative one year. After re-debridement, the infection was controlled and the wound healed. The plate internal fixation was replaced by the unilateral or annular external fixator in 14 patients and retained in 2 patients. The lengths of the bone defects averaged 2.4 cm and the time for the external fixation 10.5 months (from 8 to 14 months). Conclusion In the treatment of chronic infection after internal fixation of calf fracture with extensive soft tissue defects, the efficacy of an anterolateral femoral chimeric perforator flap combined with vancomycin-loaded calcium sulfate is satisfactory, because the flap can fully cover the bone and soft tissue defects while the vancomycin-loaded calcium sulfate can effectively control the infection. © 2023 Chinese Journal of Orthopaedic Trauma. All rights reserved.     

残余胆囊结石与急性胰腺炎8例分析

Objective To study the residual gallbladder stones and the relationship between the onset of acute pancreatitis and its surgical treatment.Methods Retrospective analysis was made in 13 patients with residual gallbladder admitted tO the hospital from June 1998 to December 2007,of which 8 cases of residual gallbladder stones and acute pancreatitis were invostigated.Thirteen cases of patients had underovent cholecystectomy 2 to 9 years before,the first symptoms occurred before 1 month to 8 years.Other remnants of the gallbladder were diagnosed by B-ultrasound and CT,magnetic resonance cholangiopancreatography (MRCP).Results Two cases were confirmedas pigment stones;6 cases as cholesterol stones(size0.2 cm ～0.5 cm,an average of 3.2 cm);1 cases as anonlalous pancre-aticobiliary junction(anomalous pancreaticobiliary ductal union,APBDU);5 cases as anomal;8 cases(61.5%)as episode of acute pancreatitis.Au patients underwent resection of residual gallbladder,with line choledochal cyst excision and biliary-enteric Roux-en-Y internal drainage in 1 case.choledocholithotomy T-tube drainage in 3 cases.Postoperative follow-up of residual gallstone was done in 8 cases,no pancreatitis attack in 6 cases,pancreatitis attack in 2 cases.Significant difference was found in onset of acute pancreatitis (P=0.019,P<0.05).Five patients without stones in patients with postoperative all none pancreatitis attack.Conclusion Patients with residual gallbladder stones are prone to acute pancreatitis,and cholecystectomy for patients with residual treatment Can reduce the pancreatitis attack.     

残余胆囊结石与急性胰腺炎8例分析

Objective To study the residual gallbladder stones and the relationship between the onset of acute pancreatitis and its surgical treatment.Methods Retrospective analysis was made in 13 patients with residual gallbladder admitted tO the hospital from June 1998 to December 2007,of which 8 cases of residual gallbladder stones and acute pancreatitis were invostigated.Thirteen cases of patients had underovent cholecystectomy 2 to 9 years before,the first symptoms occurred before 1 month to 8 years.Other remnants of the gallbladder were diagnosed by B-ultrasound and CT,magnetic resonance cholangiopancreatography (MRCP).Results Two cases were confirmedas pigment stones;6 cases as cholesterol stones(size0.2 cm ～0.5 cm,an average of 3.2 cm);1 cases as anonlalous pancre-aticobiliary junction(anomalous pancreaticobiliary ductal union,APBDU);5 cases as anomal;8 cases(61.5%)as episode of acute pancreatitis.Au patients underwent resection of residual gallbladder,with line choledochal cyst excision and biliary-enteric Roux-en-Y internal drainage in 1 case.choledocholithotomy T-tube drainage in 3 cases.Postoperative follow-up of residual gallstone was done in 8 cases,no pancreatitis attack in 6 cases,pancreatitis attack in 2 cases.Significant difference was found in onset of acute pancreatitis (P=0.019,P<0.05).Five patients without stones in patients with postoperative all none pancreatitis attack.Conclusion Patients with residual gallbladder stones are prone to acute pancreatitis,and cholecystectomy for patients with residual treatment Can reduce the pancreatitis attack.     

Resection and reconstruction of the inferior vena cava for neoplasms

AIM: To evaluate the results of an aggressive surgical approach of resection and reconstruction of the inferior vena cava (IVC). METHODS: The approach to caval resection depends on the extent and location of tumor involvement. The supraand infra-hepatic portion of the IVC was dissected and taped. Left and right renal veins were also taped to control the bleeding. In 12 of the cases with partial tangential resection of the IVC, the flow was reduced to less than 40% so that the vein was primarily closed with a running suture. In 3 of the cases, the lumen of the vein was significantly reduced, requiring the use of a polytetrafluoroethylene (PTFE) patch. In 2 of the cases with segmental resection of the IVC, a PTFE prosthesis was used and in 1 case, the IVC was resected without reconstruction due to shunting the blood through the azygos and hemiazygos veins. RESULTS: The mean operation time was 266 min (230-310 min) with an average intraoperative blood loss of 300 mL (200-2000 mL). The patients stayed in intensive care unit for 1.8 d (1-3 d). Mean hospital stay was 9 d (7-15 d). Twelve patients (66.7%) had no complications and 6 patients (33.3%) had the following complications: acute bleeding in 2 patients; bile leak in 2 patients; intra abdominal abscess in 1 patient; pulmonary embolism in 2 patients; and partial thrombosis of the patch in 1 patient. General complications such as pneumonia, pleural effusion and cardiac arrest were observed in the same group of patients. In all but 1 case, the complications were transient and successfully controlled. The mortality rate was 11.1% (n = 2). One patient died due to cardiac arrest and pulmonary embolism in the operation room and the second one died 2 d after surgery due to coagulopathy. With a median follow-up of 24 mo, 5 (27.8%) patients died of tumor recurrence and 11 (61.1%) are still alive, but three of them have a recurrence on computed tomography. CONCLUSION: There are a variety of options for reconstruction after resection of the IVC that offers a higher resectable rate and better prognosis in selected cases.     

Clinical and pathological features of renal amyloidosis: An analysis of 32 patients in a single Chinese centre

Aim:   To summarize the clinical and pathological features of renal amyloidosis in order to achieve early diagnosis.
Methods:   The clinical and pathological data of 32 patients with renal amyloidosis, diagnosed by renal biopsy in one renal centre, were retrospectively analyzed. Immunohistochemistry of amyloid A protein and immunoglobulin light chains was further performed on the renal specimens for further classification.
Results:   Twenty-four out of the 32 patients (75%) were not considered to have renal amyloidosis by local physicians; 91.7% (22/24) of them had at least one of the following signs: bodyweight loss, organ enlargement and decreased blood pressure. Twenty-nine out of the 32 patients (90.6%) were over 40 years, 30 patients (93.8%) had nephrotic syndrome, and 21 patients (65.6%) were found to have monoclonal light chain in serum or urine by immunofixation. Six patients (18.8%) were negative by Congo red stain and were diagnosed as having early renal amyloidosis by electron microscopy. Twenty-eight patients were diagnosed as having AL amyloidosis, two were suspected of having AL amyloidosis, one had AA amyloidosis and the status of the remaining patient was undetermined.
Conclusion:   Renal amyloidosis is frequently neglected by local physicians in China. Middle-aged nephrotic patients with weight loss, organ enlargement and monoclonal light chains in serum or urine should be highly suspected of the disease. Renal biopsies, especially electron microscopy, play a crucial role in the early diagnosis of renal amyloidosis.     

AL amyloidosis with renal involvement

Primary (AL amyloidosis) is a systemic disease characterized by an amyloid deposition process in many organs, with unsatisfactory survival of patients. The monoclonal light chains form the fibrils that deposit and accumulate in tissues. Renal involvement is very frequent in AL amyloidosis and could lead to development of nephrotic syndrome followed by the renal failure in many cases. Classic therapeutic combination melphalan and prednisone has been supplemented with drugs with different mechanisms of action in this group of patients: high-dose dexamethasone, high-dose dexamethasone with melphalan, combination of vincristine, doxorubicin, and dexamethasone or newly high-dose melphalan supported by peripheral blood stem cell transplantation. This progressive therapy leads to the better survival and prognosis in the majority of patients. Alternative therapeutic approaches include thalidomide (alone or in combination with cyclophosphamide), lenalidomide, iododoxorubicin, etanercept and rituximab. The development of immunotherapy is expected in the near future.     

Immunoglobulin heavy chain can be amyloidogenic: morphologic characterization including immunoelectron microscopy

It is not well recognized that monoclonal immunoglobulin heavy chains or their fragments can be amyloidogenic. Amyloidosis due to heavy chains, referred to as AH amyloidosis, is rare with only three cases previously reported. An additional case of AH amyloidosis is reported. To the best of our knowledge, this is the first case of IgM heavy chain amyloidosis. A 59-year-old man presented with nephrotic syndrome. Immunoelectrophoresis detected a monoclonal IgM lambda in his serum and free lambda light chains in his urine. A renal biopsy showed amyloidosis, in which the amyloid deposits stained only for mu heavy chain by immunofluorescent and immunoelectron microscopic studies. This case suggests that monoclonal immunoglobulin heavy chains can be amyloidogenic. Furthermore, this type of amyloidosis shares the same morphologic features with other types of amyloidosis and can involve the kidney to produce nephrotic syndrome.     

Leukocyte chemotactic factor 2: A novel renal amyloid protein

Renal amyloid deposits can often be seen in primary amyloidosis (immunoglobulin light chain disease) or in secondary forms such as reactive amyloidosis as well as in several hereditary forms where a variety of mutant proteins 'precipitate' as amyloid plaques. However, in rare cases, amyloidosis may be identified by renal biopsy, but no definitive diagnosis could be made. We have isolated amyloid fibrils from such a case in which the patient presented with nephrotic syndrome and subsequent azotemia requiring hemodialysis. Evaluation for amyloid deposition in other organ systems was negative and immunohistochemical analysis of the kidney deposits for known contributing proteins was unrevealing. Biochemical analysis of the fibrils identified a new amyloid subunit protein, leukocyte chemotactic factor 2, originally identified as a possible chemotactic and growth factor. A monoclonal antibody to this protein reacted specifically with the amyloid deposits in the glomeruli and interstitium by immunohistochemistry. This study emphasizes the importance of biochemical characterization of amyloid present in renal biopsies.     

Renal involvement in systemic amyloidosis--an Italian retrospective study on epidemiological and clinical data at diagnosis.

BACKGROUND: Few data are available on epidemiology and clinical picture of renal involvement in different forms of systemic amyloidosis. METHODS: Patients with biopsy-proven systemic amyloidosis diagnosed in Italy between January 1995 and December 2000 were selected from 49 Nephrology and Internal Medicine Units provided they showed signs characteristic of renal involvement. Clinical and laboratory information were collected by using a specific data form for diagnosis integrated by a questionnaire on diagnostic tools. Collected data were matched both with the Italian Registry of Renal Biopsies (IRRB) and the Registry of the Italian Society of Amyloidosis (SIA) in order to approximate the incidence of the disease. RESULTS: Of all patients, 373 were finally selected throughout Italy with an estimated mean incidence of renal amyloidosis of 2.1 per million population (p.m.p.) per year. Of those, 237 were affected from AL (primary) amyloidosis, 104 from AA (secondary) amyloidosis and 6 from AF (heredofamilial) forms. In 26 cases the type of amyloidosis remained undetermined. Among patients with AL, 36 presented an associated multiple myeloma (MM). Rheumatoid arthritis (RA) was the commonest underlying disease in AA. Median age ranged between 63 and 65 years in all groups. Males were prevalent in AL and females in AA. The main clinical features of renal involvement were represented by nephrotic syndrome and renal failure observed in 59 and 54% of cases, respectively. The presence of a lambda light chain, either in serum or urine was significantly associated to a more elevated urinary protein loss and to a reduced renal function. Patients with AA showed a worse renal function at presentation than patients with AL, possibly due to a late diagnosis and/or referral to nephrology units. Diagnosis was obtained by renal biopsy in 315 cases, by abdominal fat tissue (AFT) aspiration/biopsy in 156 patients and by other organ biopsies in 47 patients. Characterization of deposits was extremely variable among referring centres. CONCLUSIONS: Our results point to an increased incidence of renal amyloidosis observed in Italy over the period 1996-2000 with AL as the prevalent type. Characterization of amyloid deposits still remains the major diagnostic challenge of the disease. The institution of networks dedicated to rare diseases is strongly recommended in order to effectively afford this challenge.     

Renal amyloidosis secondary to tuberculosis of cecum

Renal amyloidosis can occur as a primary or secondary, systemic or localized disorder. It is defined as a chronic infiltrative disorder characterized by impaired organ function caused by extracellular insoluble protein fibrils. Although colonic tuberculosis is not uncommon, the occurrence of reactive renal amyloidosis in such patients is not as prevalent. We report a single case of renal amyloidosis in a patient with tuberculosis of the cecum who presented with nephrotic syndrome.     

Current treatment of AL amyloidosis

Systemic AL amyloidosis is a rare complication of monoclonal gammopathies. Renal manifestations are frequent, mostly characterized by heavy proteinuria, with nephrotic syndrome and renal failure in more than half of the patients at diagnosis. Without treatment, median survival does not exceed 12 months. Amyloid heart disease and diffusion of amyloid deposits are associated with reduced survival. Treatment of systemic AL amyloidosis has been profoundly modified with the introduction of international criteria for the definition of organ involvement and hematologic response, and with the use of sensitive tests for the measurement of serum-free light chain levels. Melphalan plus dexamethasone is now established as the gold standard for first line treatment of systemic AL, with similar efficacy and reduced treatment-related mortality compared to high-dose therapy. Modern chemotherapy regimens, based on the use of novel agents such as bortezomib and lenalidomide, might further improve patient survival.     

Castleman病肾损害的临床病理分析

目的 探讨Castleman病肾损害的临床病理特点。 方法 对10例Castleman病合并肾损害患者的临床病理资料进行分析。全部病例均接受了淋巴结和肾组织的病理检查,肾活检组织分别进行了光镜、免疫荧光和电镜检查。 结果 10例均为男性,平均年龄（49±14）岁。多数患者有水肿;全部患者均有蛋白尿,尿蛋白量（24 h）为（2.79±3.56）g,其中1例为肾病综合征（NS）;8例有血尿;6例并发急性肾功能不全;4例有高血压。多数有发热、乏力、纳差、体质量下降等。其他异常有贫血、血小板减少、浆膜腔积液、红细胞沉降率增块、高γ球蛋白血症、补体降低、C反应蛋白（CRP）升高、肝脾肿大、甲状腺功能低下等。2例符合POEMS综合征,1例有干燥综合征。10例均表现全身多处淋巴结肿大,以颈部、腋窝及腹股沟淋巴结肿大最常见。淋巴结活检病理类型分别为浆细胞型4例,透明血管型3例,混合型3例。肾活检病理诊断分别为血栓性微血管病5例、新月体性肾小球肾炎2例、肾淀粉样变1例、肾小球微小病变1例、慢性肾小管间质肾病1例。所有病例经过免疫抑制剂或COP方案治疗,病情均明显缓解,淋巴结缩小,蛋白尿减少或转阴,大部分病例肾功能恢复正常。 结论 Castleman病肾损害的临床和病理表现具有多样性,急性肾功能不全发生率较高,常并发全身多系统损害,肾脏病理以血栓性微血管病较多见。对于伴有全身多系统异常的肾脏病患者,有必要进行淋巴结影像学检查和淋巴结活检。     

Resolution of primary amyloidosis by melphalan and prednisolone: a case report

We here report a case of a 50-year-old man who showed histologically evident resolution of primary amyloidosis by melphalan and prednisolone. The patient was admitted to our hospital for further evaluation of nephrotic syndrome and remarkable hepatomegaly with refractory ascites, on September 11, 1998. Laboratory tests at presentation showed nephrotic syndrome with slight renal impairment and elevation of the enzymes of the biliary system. Monoclonal light chains were not detected in the serum or urine by immunoelectrophoresis. A renal biopsy revealed global deposition of amyloid in all glomeruli, interstitium and blood vessels. Immunofluorescence staining was positive for kappa light chains. Liver biopsy specimens showed extensive deposition of amyloid along sinusoid walls. Bone marrow aspiration contained 7% plasma cells but no clusters or abnormal cells. Based on these findings, systemic AL- (amyloid light chain) amyloidosis was diagnosed, and the treatment with combinations of melphalan and prednisolone was started from October 1998 at intervals of 4-6 weeks. Renal impairment progressed, resulting in the initiation of maintenance hemodialysis in February 1999. Reinfusion of ascitic fluid into the hemodialysis circuit had been performed from March 1999 for refractory ascites, and ascites disappeared in July 1999. Furthermore, urinary output increased after 14 courses of chemotherapy. Renal function gradually ameliorated with a concomitant reduction in the enzymes of biliary system, and finally hemodialysis was discontinued in April 2001. Sixteen courses of chemotherapy were administered by April 2001. Proteinuria was negative in August 2001. A second renal biopsy was performed on November 20, 2001, which showed markedly decreased amyloid deposition and a proliferation of mesangial cells and increase in matrix in various degrees. We report a case of a patient with primary amyloidosis who was successfully treated by melphalan and prednisolone, resulting in marked resolution of renal amyloidosis.     

肾脏淀粉样变性的临床病理分析

目的：探讨肾脏淀粉样变性病（amyloidosis,AL）的临床病理特点、诊断及鉴别诊断。方法回顾性分析9例肾脏淀粉样变性病患者的临床表现,并对其肾活检组织进行光镜、特殊染色、免疫荧光、免疫组化及超微结构观察。同时对患者进行随访获取预后信息。结果9例肾脏淀粉样变性病患者的临床上主要表现为肾病综合征伴进行性肾功能不全并逐渐恶化。肾活检组织光镜观察,早期淀粉样变光镜表现轻微,可有系膜轻度增生或基底膜空泡变性及轻度增厚,PASM染色可见节段性睫毛状结构;后期,肾小球系膜区明显增宽、基底膜增厚,毛细血管腔闭塞,呈无细胞结节硬化状态。免疫荧光表现不一,部分病例全部阴性,部分表现免疫球蛋白及补体沿系膜区或毛细血管壁不同程度的沉积。刚果红染色呈砖红色,甲基紫染色呈紫红色。免疫组化显示,5例来源于轻链λ,1例来源于轻链κ,全阴性者有3例。电镜观察下,肾小球毛细血管基底膜部分节段无明显病变,部分节段不规则增厚,肾小球基底膜外侧和（或）内皮下可见淀粉样纤维沉积;系膜轻、中度无细胞性增生,其内可见不规则排列淀粉样纤维沉积,直径8~10 nm,无分支,僵硬,排列紊乱。足突大部分融合,肾间质纤维化,其内亦可见淀粉样纤维沉积。结论肾脏淀粉样变性多见于中老年,根据光镜的典型病理学改变,并结合刚果红和甲基紫染色,可明确诊断。电镜检查是早期诊断肾淀粉样变的重要手段。