Nephrotic syndrome as a paraneoplastic symptom of Hodgkin's disease in two children

A 13-year-old girl and a 3-year-old boy presented with the clinical symptoms of nephrotic syndrome. Both patients developed a nodular mass in the cervical region, which appeared to be due to Hodgkin's disease. The renal disease was a paraneoplastic phenomenon of Hodgkin's disease. Chemotherapy given for Hodgkin's disease also cured the nephrotic syndrome. A few years later one of the patients experienced a recurrent episode of nephrotic syndrome in conjunction with a recurrence of Hodgkin's disease but this was once again treated successfully. Nephrotic syndrome is a known but rare paraneoplastic syndrome accompanying Hodgkin's disease. It is thought that T cell dysfunction leads to a secretion of cytokines which alters the permeability of the glomerular basement membrane. This dysfunction is not only apparent during the period of active disease and this could clarify why paraneoplastic syndrome can exist or recur outside the period in which the malignancy is overt. The clear recognition of a paraneoplastic syndrome is important, as it is often only necessary to treat the malignancy.     

Effects of lymphoma on the peripheral nervous system.

Peripheral nervous system abnormalities occur in 5% of patients with lymphoma and have a wide differential diagnosis. Herpes zoster is the commonest cause. Vinca alkaloids are the only drugs used in lymphoma which commonly cause neuropathy. Compression or infiltration of nerve roots by lymphoma is a rare presenting feature but becomes more common with advanced disease. Radiation plexopathy does not usually develop until at least 6 months after irradiation and can be difficult to distinguish from neoplastic infiltration. Either multifocal infiltration of nerves or lymphoma-associated vasculitis may present as a peripheral neuropathy. The incidence of Guillain-Barré (GBS) syndrome, and possibly chronic idiopathic demyelinating polyradiculoneuropathy, appears to be increased in association with lymphoma, especially Hodgkin's disease. Subacute sensory neuronopathy and subacute lower motor neuronopathy have both been reported as paraneoplastic syndromes associated with Hodgkin's disease. Treatment of the underlying lymphoma is only rarely followed by recovery of the associated neuropathy.     

Cerebral deep vein thrombosis associated with rectal cancer

The authors report a case where the patient suffered from deep cerebral venous thrombosis, which developed beside cerebral metastases of a colorectal cancer. The pathogenesis and diagnosis of this disease are also discussed. This rare location of thrombosis is mainly due to hypercoagulable state seen in the use of oral contraceptive drugs, Beh?et syndrome, nephrotic syndrome, and as paraneoplastic syndrome in malignant diseases. Literature reports less than 50 cases of deep cerebral venous thrombosis, of which less than 10 are evoked by malignant disease. The symptoms of DCVT can mimic cerebral metastases in cancer patients. The course of disease is aggressive, the prognosis is poor. Even if the patients survive considerable neurological deficits may remain. Authors emphasize the importance of current modern diagnostic imaging methods in the diagnosis. The possibility of deep cerebral venous thrombosis must be taken into account if sudden neurological symptoms develop in a cancer patient.     

自身免疫性甲状腺疾病相关肾病临床分析

目的观察和研究自身免疫性甲状腺疾病相关肾病临床特点和发病机制。方法通过20例病人临床资料分析及文献复习,对临床表现和疗效进行综合分析。结果自身免疫性甲状腺疾病相关肾病发病率较高,应重视原发病的治疗,大多数病人需同时应用糖皮质激素和/或免疫抑制剂治疗才能取得良好疗效。结论临床上应重视自身免疫性甲状腺疾病相关肾病,对肾病综合征患者应注意检查甲状腺功能以免贻误治疗。     

Renal amyloidosis complicating Crohn's disease: report of two cases and review of literature

Secondary amyloidosis is caused by the extracellular store of the fragment AA of the circulatory protein in serum amyloid-A. It can complicate diseases such as family mediterranean fever, rheumatoid arthritis or Crohn's disease. Renal amyloidosis is a rare but serious complication of Crohn's disease. We report two cases of Crohn's disease associated with a nephrotic syndrome due to renal amyloidosis. It is important to recognize this complication, especially since Colchicine has been proposed as a possible treatment. So, the search for proteinuria seems to be important for an early diagnosis of renal amyloidosis in Crohn's disease.     

Dual Paraneoplastic Syndromes: Small Cell Lung Carcinoma-related Oncogenic Osteomalacia,and Syndrome of Inappropriate Antidiuretic Hormone Secretion: Report of a Case and Review of the Literature

Acquired isolated renal phosphate wasting associated with a tumor, known as oncogenic osteomalacia or tumor-induced osteomalacia, is a rare paraneoplastic syndrome caused by overproduction of fibroblast growth factor 23. Oncogenic osteomalacia is usually associated with benign mesenchymal tumors. Syndrome of inappropriate antidiuretic hormone secretion (SIADH), on the other hand, is a common paraneoplastic syndrome caused by small cell carcinoma (SCC). Concomitant oncogenic osteomalacia and SIADH associated with SCC is very rare with only 4 other cases reported in the literature. The authors report a case of small cell lung cancer (SCLC)-related renal wasting hypophosphatemia and concurrent SIADH, and review the literature reporting 9 other cases of SCC associated with oncogenic osteomalacia. Almost half of reported cases of renal phosphate wasting associated with SCC concomitantly presented with SIADH. These cases had initial serum phosphorus level lower and survival periods shorter than those without SIADH. This rare combination of a dual paraneoplastic syndrome and low serum phosphorus may be a poor prognostic sign. In addition, both renal phosphate wasting and SIADH usually occur in a short period of time before identification of SCC. Therefore, renal wasting hypophosphatemia with concomitant SIADH/hyponatremia should prompt a search for SCC rather than a benign mesenchymal tumor.     

Nephrotic syndrome in the Africans and Indians of South Africa. A ten-year study

The frequency of nephrotic syndrome was 0.2 % of all medical admission records. A prospective study of 180 nephrotic patients was begun in 1966 and ended in 1976 and the clinical, biochemical and renal histological changes were studied. Unlike other studies of nephrotic syndrome in West and East Africa, in which malaria is believed to play an important aetiological part, this study in Durban was done in a malaria-free area. Most of the patients were between the ages of 12 and 30 years. The aetiology of the nephrotic syndrome was undetermined in 94% of the African patients and in 87% of the Indian patients. The most common histological pattern in both racial groups was proliferative glomerulonephritis, followed by membranous glomerulonephritis. Minimal change on light microscopy was rare. This has important implications from the therapeutic aspect because African patients suffering from nephrotic syndrome will not as a rule respond to steroids or cyclophosphamide therapy. An initial diastolic blood pressure ? 110 mm Hg and a low serum complement were more common in proliferative glomerulonephritis. The serum gamma-globulin, though low, was raised compared with the normal serum gammaglobulin, in the white population. A difference in the serum lipoprotein patient between the two races was observed.     

小儿肾病综合征的家庭护理对策及护理干预分析

目的研究并分析小儿肾病综合征的家庭护理对策及护理干预效果。方法将我院2009年12月-2012年6月期间共收治的80例小儿肾病综合征患者随机分为观察组和对照组两组,每组分别由40例小儿肾病综合征患者组成,给予两组小儿患者常规治疗,即激素、饮食、利尿剂、调整血脂、控制血压、纠正水电解质紊乱等。对照组的小儿患者在上述常规治疗的基础上给予常规的肾病综合征护理,而观察组的小儿患者则在常规护理的基础上外加家庭护理干预,将两组小儿患者的临床疗效进行对比与分析。结果观察组的小儿患者无论在疾病的治愈率还是在基本的复发率等方面均明显优于对照组的小儿患者,差异具有显著的统计学意义(P<0.05)。结论家庭护理干预对于小儿肾病综合征的患者而言十分重要,其不仅能够有效的提高疾病的治愈率,还能够明显降低疾病的复发率,护理疗效十分显著,值得推广和借鉴。     

Pierson综合征的研究进展

Pierson综合征是编码层黏连蛋白β2的基因LAMB2（laminin beta 2）突变导致的常染色体隐性遗传性病,以先天性肾病综合征、早发性肾功能衰竭和双侧小角膜为典型特征。常伴有弥漫性系膜硬化、其他眼部异常以及神经系统异常,发病后通常会迅速发展至肾衰竭。Pierson综合征临床上较为少见,难以依据临床表型进行诊断,容易漏诊和误诊,需要与其他可以引起肾脏异常伴或不伴肾外异常的疾病相鉴别。目前Pierson综合征尚无特定疗法,治疗方法主要是针对性的支持治疗,但预后较差。综述该综合征的研究进展,提高临床医生对Pierson综合征的认识。     

Acrokeratosis paraneoplastica of Bazex in association with a metastatic neuroendocrine tumour.

Bazex syndrome is a rare cutaneous paraneoplastic phenomenon not previously reported in association with neuroendocrine tumours. We describe a patient who responded well to treatment with octreotide.     

RNA expression as a prognostic tool in idiopathic nephrotic syndrome

Approximately 90% of children with nephrotic syndrome have idiopathic nephrotic syndrome. Idiopathic nephrotic syndrome includes three histologic types: minimal change disease, mesangial proliferation and focal segmental glomerulosclerosis. These diseases have similar clinical presentation but different prognosis. The aim of this review is to summarize the genetic knowledge related to idiopathic nephrotic syndrome, follow the progression of these diseases and to offer a survey of the gene expression pattern changes and their functional classification. Different types of RNA expression analysis methods, such as the northern-blot assay, the ribonuclease protection assay, the RNA in situ hybridization, the quantitative RT-PCR and the RNA expression microarray technology are discussed. Previous studies emphasize the importance of the following gene groups in the idiopathic nephrotic syndrome: genes involved the DNA synthesis and repair, growth factors, extracellular matrix proteins, extracellular ligand receptors, extracellular signal transduction, metabolic and transport process and immune regulation are frequently dys-expressed in idiopathic nephrotic syndrome. With the development and spread of the microarray technology these genes can be used as a compliment to the conventional diagnostic method.     

Guillain-Barré syndrome in patients treated for Hodgkin disease

INTRODUCTION: A 14-year-old boy treated with Hodgkin's disease developed muscular weakness and pain, hypotonia, abolished deep tendon reflexes. Examination of the cerebrospinal fluid showed albuminocytologic dissociation, the characteristic finding of Guillain-Barré syndrome. After high dose steroid treatment complete recovery occurred. DISCUSSION: Paraneoplastic neuropathies may develop in patients with cancer, Guillain-Barré syndrome occurs in patients with active Hodgkin's disease. CONCLUSIONS: Progressive neurological signs may indicate the presence of a malignancy, or in case of chemotherapy shows the activity of the tumour.     

Paraneoplastic syndromes

As the lengthy but nonetheless incomplete review suggests, paraneoplastic syndromes are protean in their manifestations and, for the most part, poorly understood. Indeed, some of the more common abnormalities in cancer patients that might be considered paraneoplastic--such as anorexia-cachexia syndrome or unexplained fever--have not been discussed because they are so poorly understood. Most of the syndromes reviewed are either clearly paraneoplastic or strongly associated with cancer. Their clinical importance does not lie in the number of patients affected; it is a small minority. Instead, the syndromes may occasionally be helpful in the diagnosis of cancer or in monitoring response to cancer therapy. They may also be confused with the effects of metastatic disease. In some patients, amelioration of the syndromes can reverse the patient's dominant symptoms and thus provide significant clinical palliation. In a more general context, studies of etiologic mechanisms in paraneoplastic syndromes may offer insights into a variety of unexplained abnormalities in cancer patients. The best-understood syndromes result from tumor production of biologically active substances or, to a lesser extent, from autoimmune phenomena. These would appear to be probable mechanisms in many recognized paraneoplastic syndromes of uncertain etiology and perhaps in some heretofore unrecognized paraneoplastic syndromes. Finally, paraneoplastic syndromes could also hold clues to the neoplastic process. Better understanding of the ways in which tumors regulate remote effects--such as release of TGFs--may ultimately enhance our knowledge of tumor growth itself.     

雷公藤多苷与环磷酰胺治疗肾病综合征的对比研究

目的：探究肾病综合征患者采用雷公藤多苷与环磷酰胺综合治疗的治疗效果和安全性。方法：选取2012年4月-2013年10月收治的74例肾病综合征患者进行治疗,随机分组,实验组44例患者采用雷公藤多苷的治疗,而对照组30患者仅使用环磷酰胺的治疗,对患者的治疗效果进行观察和评价。结果：实验组患者治疗的总有效率为88.64%,4例出现有不良反应,发生率为9.1%,24小时尿蛋白和血浆蛋白情况得到明显缓解;对照组患者治疗的总有效率为60.0%,10例出现有不良反应,发生率为33.3%。有显著的效果差异,有统计学意义（P〈0.05）。结论：肾病综合征患者选择雷公藤多苷治疗,可使肾功能得到较好的恢复,缓解身体症状,延缓病情的发展,且安全性较高,值得推荐     

肾病综合征与糖尿病肾病的血脂代谢特点分析

目的分析比较原发性肾病综合征和糖尿病肾病患者血脂异常的特点。方法选择2008年12月～2010年12月在我院肾病内科以及内分泌科住院的70名原发性肾病综合征患者和70例糖尿病肾脏疾病患者,比较两组患者各血脂指标是否存在差异。结果原发性肾病综合征患者血脂异常的发生率为98.6%,高于糖尿病肾病患者90.0%的血脂异常发生率,且差异具有统计学意义;其中肾功能正常时,肾病综合征患者TC、HDL和LDL水平均明显高于糖尿病肾病患者,在肾功能下降的人群之中,TC、LDL的差异虽仍有统计学意义,但在数值上差距减小,HDL的差异并没有统计学意义。结论TC和LDL在两组患者中的差异较为明显,在临床上可以考虑作为鉴别原发性肾病综合征和糖尿病肾病的血脂方面指标。     

肾病综合征患者抑郁状态及其相关因素分析

[目的]了解肾病综合征患者抑郁现况及影响因素。[方法]采用Zung自评抑郁量表(SDS)对180例肾病综合征患者进行调查。[结果]抑郁症患病率为46.5%。影响患者抑郁得分值的因素有文化程度、经济状况、医保情况、激素效果、治疗时间、复发情况、并发症。[结论]对肾病综合征患者应及时采取针对性的措施进行心理状况干预,以改善患者的心理状态,提高患者的生活质量。     

环孢素A治疗难治性肾病综合征的临床观察

目的：探讨环孢素A用于治疗难治性肾病综合征的临床疗效与价值。方法：选取2010年1月-2013年12月本院儿科确诊的难治性肾病综合征患者共46例,对所有患者使用环孢素A进行治疗,并对其治疗后每3个月实验室检查指标、症状改善及复发人数等情况进行记录。结果：治疗后,患者各项检查指标均显著优于治疗前,比较差异统计学意义（P〈0.05）;治疗后不同病理类型疾病缓解率分别为75.0%,91.7%,88.9%,治疗效果显著;患者中,有2例微小病变患者、1例系膜增生性肾小球肾炎患者使用半年环孢素A后无效,病情迁延不愈,调整剂量后再次使用环孢素A,疾病均得到一定控制,疗效较好。结论：使用环孢素A对难治性肾病综合征进行治疗疗效显著,可发挥免疫抑制作用,在疾病治疗中抑制自身免疫反应,有效减少炎性反应。短期疗效显著,不良反应较少,安全系数较高。可在短时间内缓解患者症状,有效改善蛋白尿且长期预后较好。用药时严格检测药物血药浓度及肝肾功能可提高用药安全性,降低不良反应发生率。     

Paraneoplastic hormonal syndromes

We can define paraneoplastic syndromes as a combination of effects occurring far from the original location of the tumour and independently from the local repercussion of its metastases. Paraneoplastic hormonal syndromes depend on the secretion of hormonal peptides or their precursors, cytokines and, more rarely, thyroidal hormones and Vitamin D, which act in an endocrine, paracrine or autocrine way. Sometimes, paraneoplastic syndromes can be more serious than the consequences of the primary tumour itself and can precede, develop in parallel, or follow the manifestations of this tumour. It is important to recognise a paraneoplastic hormonal syndrome for several reasons, amongst which we would draw attention to three: 1) It can lead to the diagnosis of a previously undetected, underlying malign or benign neoplasia; 2) It can dominate the clinical picture and thus lead to errors with respect to the origin and type of primary tumour; and 3) It can follow the clinical course of the underlying tumour and thus be useful for monitoring its evolution. The molecular mechanisms responsible for the development of these syndromes are not well-known, but it is believed that they might be inherent to the mutations responsible for the primary tumour or depend on epigenetic factors such as methylation. In this review, we consider the following paraneoplastic hormonal syndromes: malign hypercalcaemia, hyponatraemia (inappropiate secretion of the antidiuretic hormone), ectopic Cushing's syndrome, ectopic acromegaly, hypoglycaemia due to tumours different from those of the islet cells and paraneoplastic gynaecomastia; we make a brief final reference to other hormones (calcitonin, somatostatin, and VIP).     

肾病综合征患者D-二聚体的检测

目的 探讨肾病综合征患者D-二聚体的检测及临床意义.方法 将笔者所在医院2009年2～6月收治的32例肾病综合征患者的D-二聚体检测资料作为观察组,同期健康者的D-二聚体检测资料作为对照组,对两组进行比较分析.结果 肾病综合征患者的D-二聚体水平明显高于对照组,随着尿蛋白量的增加而增加.结论 D-二聚体检测可以反映肾病综合征患者体内的血凝状态,有利于预防深静脉血栓等并发症的发生.     

C反应蛋白检测在评估儿童原发性肾病综合征病情及预后的价值

目的分析C反应蛋白检测在评估儿童原发性肾病综合征（PNS）病情及预后的价值,为PNS的诊断和治疗提供理论依据。方法对150例PNS患儿和150例正常儿童治疗前和治疗后血清CPR进行检测,并比较治疗过程中CPR的变化情况。结果肾炎型肾病CRP均明显高于单纯型肾病和对照组,差异有明显的统计学意义（t=7.34;11.23 ,P〈0.05）,单纯型肾病组CRP明显高于对照组,差异有明显的统计学意义（t=7.34,P〈0.05）,治疗后肾炎型肾病CRP均明显高于单纯型肾病和对照组,差异有明显的统计学意义（t=M4;5.23,P〈0.05）,而单纯型肾病CRP与对照组比较均无明显的统计学差异意义（t=1.34;P〉0.05）。结论CRP在判断PNS患儿病情和预后具有重要的临床价值。