Primary antiphospholipid syndrome presenting as renal vein thrombosis and membranous nephropathy

Antiphospholipid syndrome is a multisystem auto-immune disorder characterized by thrombotic events and the presence of circulating antiphospholipid antibodies. Large vessel involvement in the form of thrombosis/stenosis and thrombotic microangiopathy is a commonly described renal finding. However, non-thrombotic glomerulopathies are increasingly being recognized in patients with antiphospholipid syndrome. We report a rare occurrence of both renal vein thrombosis and membranous nephropathy in a previously healthy adolescent male. Investigations revealed persistently positive antiphospholipid antibodies in the absence of an underlying systemic autoimmune disorder or malignancy. Our patient responded favourably to anti-proteinuric therapy and anticoagulation with complete resolution of proteinuria and a nearly occlusive thrombus.     

肾包膜下积液的外科治疗（附7例报告）

目的探讨肾包膜下积液的外科治疗。方法回顾性分析7例肾包膜下积液的病因、治疗经过。其中男性4例,女性3例。3例男性和1例女性患者由体外震波碎石（ESWL）引起,1例女性由输尿管镜检引起,其余2例无明显原因。同位素分肾功能检查均有患侧肾小球滤过率下降,2例有高血压和血管紧张素及醛固酮水平升高（Page肾）。7例均行肾包膜切除术,手术均顺利完成。结果随访6~24月（平均11月）,无1例肾包膜下积液复发。2例高血压患者术后4周血压逐渐降至正常,复查血中血管紧张素和醛固酮水平正常。5例患肾功能完全恢复,2例3月后患肾的肾小球滤过率较对侧下降15%和25%。结论对外伤和医源性肾包膜下积液或积血患者要严密观察,有肾性高血压或患肾功能减退者应积极外科治疗,防止发生不可逆性肾功能损害。腹腔镜肾纤维膜切除应为首选方法,严重患者行肾切除。     

Recurrent membranous nephropathy and leiomyosarcoma in the renal allograft of a lupus patient

The frequency of membranous lupus nephritis recurrence (World Health Organization (WHO) class V) in the allograft after renal transplantation is unknown, but it appears uncommon (only two reported cases in the literature). Despite the increased incidence of sarcomas in organ transplant recipients (compared to the general population), non-Kaposi's sarcoma is an uncommon malignancy, and primary tumor involvement of a renal allograft is a rare occurrence. Our patient is a 28 year old female with end-stage renal disease (ESRD) secondary to membranous lupus nephritis who received a living related transplant from her mother. At 26 months post-transplant, she presented with proteinuria and a rise in creatinine (Cr). Allograft biopsy was consistent with recurrent membranous nephropathy. Five weeks later, she was found to have a high-grade leiomyosarcoma originating within the allograft. We reviewed the literature on recurrent post-transplant membranous nephropathy and the possible role of the Epstein-Barr virus (EBV) infection in smooth muscle tumors occurring in organ transplant recipients. We also considered the association of membranous nephropathy and malignancy.     

Bucillamine-associated membranous nephropathy in a patient with rheumatoid arthritis

A case of bucillamine-associated membranous nephropathy in a patient with rheumatoid arthritis was reported. A 34 year-old woman was admitted to our hospital because of proteinuria in September, 1988. Rheumatoid arthritis had been diagnosed in April, 1988, and had been treated with bucillamine (disease modifying anti rheumatic drug) and amfenac (nonsteroidal antiinflammatory drug). The renal biopsy specimens showed Stage I membranous nephropathy, on both electron and immunofluorescence microscopy. Proteinuria decreased after the drugs were withdrawn despite continued RA symptoms. These results were consistent with drug induced nephropathy. Since bucillamine has some resemblances in its structure and pharmacologic action to D-penicillamine, which sometimes induces membranous nephropathy, it is thought that the nephropathy in this case was caused by an adverse reaction to bucillamine.     

后腹腔镜下肾包膜切除术和经皮穿刺引流术治疗特发性肾脏包膜下积液的临床分析

目的 探讨特发性肾包膜下积液的临床特点及治疗方法.方法 回顾性分析2006年至2013年间潍坊市中医院及潍坊市人民医院泌尿外科收治的特发性肾包膜下积液患者,共12例,总结其临床特点及诊治方法.结果 12例患者中,1例患者因无临床症状,肾实质受压迫不重,双肾功能良好,患者拒绝进一步治疗.7例患者肾实质受压明显,肾脏明显变形,患者接受腹腔镜下肾包膜切除术,患者随访半年至1年,疗效满意,均未复发.4例选择超声引导下穿刺置管引流治疗,1年内有2例复发,复发患者中1例患者在外院接受了腹腔镜肾包膜切除术,另外1例患者拒绝进一步治疗.结论 后腹腔镜肾脏包膜切除术较经皮穿刺引流术效果确切,复发率低,可以达到满意的治疗效果.     

Fibrillary glomerulonephritis complicated by membranous nephropathy in a patient with tuberculosis

Fibrillary glomerulonephritis (FGN) is a morphologically defined entity characterized by glomerular accumulations of non-branching, randomly arranged fibrils; these differ from amyloid fibrils by their larger size and lack of reactivity with Congo red and other amyloid-specific dyes. FGN is a rare disease and may mimic membranous nephropathy under routine light microscopy and immunohistochemistry. However, electron microscopy shows the fibrillary nature of these glomerular deposits. We report a rare case of membranous nephropathy complicated by fibrillary deposits in a 60-year-old man with a history of bone tuberculosis.     

Postpartum renal vein thrombosis with left retroaortic renal vein

An unusual case of thrombosis in a left retroaortic renal vein is presented. Noninvasive radiologic diagnosis is reviewed.     

Spontaneous renal subcapsular hematoma in a patient undergoing hemodialysis

We describe a patient on hemodialysis in whom a spontaneous renal subcapsular hematoma developed. The diagnosis was confirmed by computerized tomography and angiography, and the patient was treated conservatively. We recommend conservative management in such cases based on radiological findings that rule out underlying pathological changes.     

Chronic renal vein thrombosis in a renal allograft

Acute renal vein thrombosis in a renal allograft is uncommon and usually occurs in the post-transplant period. Chronic renal vein thrombosis can occur insidiously many years after transplant without significant deterioration in renal allograft function or symptoms.