Thrombotic microangiopathy in adult Still's disease

Adult Still's disease (ASD) is a rare systemic disorder characterized by fever, arthralgia, cutaneous rash, and lymphadenopathy, with high polymorphonuclear leucocytosis and low glycosylated ferritinaemia. Kidney involvement has been reported rarely. We present a patient with ASD who developed haemolytic uraemic syndrome (HUS). The 42-year-old patient was admitted for unexplained fever related to ASD according to Yamaguchi's classification criteria. As Still's disease was resistant to prednisone, high-dose intravenous immunoglobulins (IV Ig) were administered. During the follow-up the patient developed acute renal failure and non-immune haemolytic anaemia with high levels of antiphospholipid antibodies (IgG anticardiolipin antibodies and anti-beta2 glycoprotein 1 antibodies). Renal biopsy disclosed thrombotic microangiopathy (TMA) with arteriolar and glomerular involvement. Treatment with steroids and intravenous IV Ig was reinitiated but renal function worsened towards end-stage renal failure. In this case, we suggest that antiphospholipid antibodies could have promoted arteriolar and glomerular TMA. HUS may be the cause of acute renal failure in Still's disease.     

Mechanisms of disease in Hantavirus infection: pathophysiology of hemorrhagic fever with renal syndrome.

Hemorrhagic fever with renal syndrome (HFRS) is an acute viral disease that occurs over wide areas of Europe and Asia. Hantaviruses are the cause of this syndrome. The hallmark of HFRS is the triad of fever, hemorrhage, and renal failure. In its severe form it is associated with significant mortality. The syndrome evolves through five phases: febrile, hypotensive, oliguric, diuretic, and convalescent. The central physiologic derangement in HFRS is vascular dysfunction, manifested by impaired vascular tone and increased vascular permeability. The systemic effects of this dysfunction account for the occurrence of hypotension and shock, while local effects are probably important in the development of renal failure. Shock in HFRS has distributive and oligemic features, while renal failure has features of acute tubular necrosis. Hemorrhage is a consequence of vascular injury and a deficit of functional platelets. Vascular and platelet dysfunction are both compounded by uremia. Disseminated intravascular coagulation contributes to hemorrhage in some patients. Although hantaviruses are infectious for endothelial cells and may cause direct injury, a large body of evidence suggests that immune mechanisms play an important role in the pathogenesis of HFRS.     

Acute renal failure likely due to acute nephritic syndrome associated with typhoid fever

A 45-year-old woman was admitted to our hospital because of high fever and malaise. She also presented abnormal urine findings including macrohematuria and proteinuria with transient renal insufficiency. Salmonella Typhi was isolated from her blood and stool culture, and then she was diagnosed as having typhoid fever. Salmonella enterica serotype typhi infection can be complicated by renal involvement, although rare. In Japan, few cases have been reported about acute nephritic syndrome in typhoid fever. Here, we report a case of endemic acquired typhoid fever associated with acute renal failure probably due to acute nephritic syndrome successfully treated with levofloxacin.     

Syndrome TAFRO et vascularite nécrosante cutanée : une association inédite

IntroductionTAFRO syndrome is a systemic inflammatory syndrome in the spectrum of Castleman's disease, associating thrombocytopenia, anasarca, fever, renal failure and/or reticulin myelofibrosis and organomegaly. Its association with necrotizing cutaneous vasculitis has not yet been reported.Case reportA 69-year-old woman presented with weight loss, fever, anasarca, organomegaly, lymphadenopathy, anuria and extensive necrotic livedo occurring after acute diarrhea. Biology showed anemia, thrombocytopenia, renal failure, hypergammaglobulinemia, a circulating B-lymphocyte clone, hypoparathyroidism and autoimmune hypothyroidism. The skin biopsy showed small vessel vasculitis with fibrinoid necrosis. Methylprednisolone infusions associated with tocilizumab were ineffective and the patient became anuric. Rituximab and plasma exchanges associated to corticosteroids allowed remission for 2 months. Combination of rituximab, cyclophosphamide and dexamethasone resulted in a prolonged remission.ConclusionWe report here the first case of severe cutaneous necrotizing vasculitis in a patient suffering from TAFRO syndrome. The possible resistance to tocilizumab should be known.     

Fever of unknown origin in childhood: difficulties in diagnosis.

We have described a child with systemic onset juvenile chronic arthritis who presented initially with fever of unknown origin. Treatment of a presumed infection led to a severe allergic response with Stevens-Johnson syndrome, renal failure and DIC. This reaction obscured the features of the underlying disease and delayed the diagnosis.     

Acute renal failure in Rocky Mountain spotted fever.

Review of clinical and pathologic data from ten fatal cases of Rocky Mountain spotted fever (RMSF) revealed the importance of acute renal failure in the clinical course and of multifocal perivascular interstitial nephritis as the principal pathologic lesion. In nine cases, Rickettsia rickettsii were demonstrated by immunofluorescence in the areas of vasculitis. Evidence was lacking for the role of disseminated intravascular coagulation, glomerulonephritis, or myoglobinuria in the pathogenesis of acute renal failure in these cases. Rickettsia-induced vascular injury led to acute renal failure by several mechanisms. Hypovolemia early in the course resulted in reversible, prerenal azotemia. Transient hypotension in midcourse produced acute tubular necrosis. In fulminant cases, preterminal circulatory collapse was associated with coma and oliguria. The interstitial nephritis could not be demonstrated conclusively to contribute to the acute renal failure.     

Anicteric leptospirosis-associated severe pulmonary hemorrhagic syndrome: a case series study

ABSTRACT:: In leptospirosis, severe pulmonary hemorrhagic syndrome has replaced Weil's disease as the main cause of mortality, with rates of up to 75%. Four men, all farmers, were admitted to the intensive care unit between August 2009 and July 2010 with a diagnosis of acute respiratory distress syndrome. All patients presented with fever, hemoptysis, bilateral pulmonary infiltrates in chest radiographs, and thrombocytopenia and had compatible epidemiological history with leptospirosis; 3 patients had anemia, 3 had renal failure, 2 had increased creatine kinase, whereas bilirubin was slightly increased in only 1 patient. Leptospirosis was diagnosed serologically in all cases. Empirical therapy with ceftriaxone was administered immediately to all patients, while implementation of ARDSnet protective mechanical ventilation approach combined with an early goal-directed hemodynamic approach led to a relatively low mortality rate (25%). Acute Physiology and Chronic Health Evaluation II, Simplified Acute Physiology Score II and Sepsis-Related Organ Failure Assessment scoring systems were unable to predict the outcome of the patients with leptospirosis-associated severe pulmonary hemorrhagic syndrome.     

Irreversible sequels of leptospirosis

Leptospirosis is a zoonotic feral nidal disease (synonyms: Weil-Vasilyev disease, waterborne fever) running as an acute febrile disease with evident intoxication, renal, hepatic, and central nervous system involvements, evolving hemorrhagic syndrome mainly with its severe complicated course and high mortality rates. The clinical features of leptospirosis have been little studied in patients with comorbidities. Its poor outcomes are generally due to the development of serious complications, such as infection-toxic shock, acute renal and hepatic failure, massive hemorrhagic syndrome, infectious myocarditis, etc. This communication describes a case of the disease with developed irreversible complications: involvement of the kidney and heart in 1 case and that of the kidney with a fatal outcome. Leptospirosis mortality is frequently associated with delayed diagnosis due to the misunderstanding of the clinical picture of this disease (particularly in its similarity to hemorrhagic fever with renal syndrome). The severer acute course of the infectious process in leptospirosis is burdened with the activation of the infection foci existing in the body or the exacerbation of somatic comorbidity, which substantiates the necessity of goal-oriented early individual, background pathology-depended correction of diagnostic, etiotropic, and pathogenetic therapy, rehabilitative measures. Comorbidities in patients with leptospirosis exert a significant impact on the development of its clinical form and the course of the infectious process manifesting itself as its worsening, the more frequent and more prolonged signs ofendogenous intoxication and multiple organ dysfunction, and a larger number of nonspecific complications in the structure of causes of deaths.     

Secondary amyloidosis with irreversible acute renal failure caused by visceral leishmaniasis in a patient with AIDS

We report a case of visceral leishmaniasis and acute renal failure in a white male patient, 28 years of age, infected with the human immunodeficiency virus (HIV). The clinical presentation of the patient was diarrheic syndrome of long evolution, fever, hepatosplenomegaly and pancytopenia, accompanied by nephrotic syndrome and irreversible acute renal failure. Renal biopsy showed glomerular AA amyloid deposits. This is the first case described in humans of secondary amyloidosis caused by visceral leishmaniasis.     

辽宁省首次发现的新布尼亚病毒其培养特性的研究

摘要 目的 自2009年起我省从发热伴粒细胞、血小板减少的患者血液中,分离出一种能够引起人类患有类似肾综合征出血热（hemorrhagic fever with renal syndrome,HFRS）疾病的病毒。采用逆转录的方法扩增病毒核酸（RT-PCR）。选择有地区代表性的病毒株采用23对引物扩增病毒全部基因序列,将获得的全部氨基酸序列经BLAST检索,与GenBank中已知的汉坦病毒属、白蛉热病毒属基因序列排列做比对,确定了我省新分离出的病毒株属于布尼亚科新型病毒。2010年3月17日由中国疾病预防控制中心病毒病预防控制所李德新老师在《新英格兰医学杂志》首次向世界公布我国新分离的病毒株被正式命名为：发热伴血小板减少综合征布尼亚病毒（SFTSV）,简称 “新布尼亚病毒”,并确定了该病毒为“蜱咬病”的元凶。为叙述方便以下简称“新病毒”。方法 收集发病一周以内发热伴粒细胞、血小板减少的患者血液首先做汉坦病毒（Hantavirus,HV）血清学检测,然后将样本采用多种细胞培养的方法进行病毒分离。结果 从我省发热伴出血征候群血液样本分离出新型病毒12株。结论 我省东部山区及丘陵地带有布尼亚新病毒流行,其临床症状及体征与肾综合征出血热极为相似。但体外细胞培养及核苷酸序列却存在着明显的差别。 关键词 新布尼亚病毒 RT-PCR 细胞培养     

Acute human immunodeficiency virus infection temporally associated with rhabdomyolysis, acute renal failure, and nephrosis

A previously healthy 29-year-old homosexual man presented with a 4-day history of fever, malaise, sore throat, and bleeding gums. Rhabdomyolysis, acute renal failure, and nephrotic range proteinuria were also present. The patient was found to have acute human immunodeficiency virus (HIV) infection confirmed by the presence of HIV antigen in his serum and subsequent evolution of an HIV antibody profile typical of acute seroconversion. A kidney biopsy revealed acute tubular necrosis and mesangioproliferative glomerulonephritis, with tubuloreticular inclusions. In the presence of otherwise unexplained acute renal failure, rhabdomyolysis, or new onset nephrotic syndrome, acute HIV infection should be considered in the differential diagnosis.     

Membranous nephropathy and crescentic glomerulonephritis

The most frequent causes of glomerular diseases whose main clinical syndrome are nephrotic syndrome and acute renal failure may have several causes: acute tubular necrosis, thrombosis of renal veins, acute tubulointerstitial nephritis. Infrequently, the association between primary glomerular disease (membranous nephropathy and others) and crescentic glomerulonephritis can cause this clinical picture. We describe a young woman without systemic disease with nephrotic syndrome and acute renal failure secondary to membranous nephropathy and superimposed crescentic glomerulonephritis. She received steroids and cyclophosphamide with stabilization of renal function after two months of follow-up.     

Acute hantavirus infection or renal transplant rejection

Hantaviruses belong to the so-called emerging pathogens that are transmitted to humans by infected rodents and their excreta. In Central Europe, hantavirus infections usually occur in a mild to moderate form of hemorrhagic fever with renal syndrome. In contrast to the mostly benign or even asymptomatic course of hantavirus infections in previously healthy individuals, the acute hantavirus infection in kidney transplant recipients represents an exceptional situation regarding diagnosis and therapy. We describe the case of a 44-year-old kidney transplant recipient with acute renal transplant failure associated with acute hantavirus infection.     

Nonsteroidal antiinflammatory drugs and the kidney

Nonsteroidal antiinflammatory drugs (NSAIDs) have potentially important renal adverse effects. With regard to renal adverse effects there is no indication of significant differences between conventional NSAIDs and selective COX-2 inhibitors. Their nephrotoxicity has been well documented. Many of the renal abnormalities that are encountered as a result of NSAIDs use can be attributed to the inhibition of prostaglandins synthesis. The release of prostaglandins is particulary importent in high-risk patients, including patients with severe heart disease, liver disease, preexisting renal disease, elderly and patients with volume depletion. The common complication of NSAID use is retention of sodium and edema formation due to increased reabsorption of sodium and water in the loop of Henle and hyperkalemie due to diminished renin secretion. Nonsteroidal antiiflammatory drugs can induce two different forms of acute renal failure. Decreased prostaglandin synthesis can lead to reversible renal ischemia and hemodynamically-mediated acute renal failure. Second form of acute renal failure is acute interstitial nephritis. This type of interstitial nephritis is often accompanied by nephrotic syndrome due to minimal change disease. Nephrotic syndrome after NSAIDs treatments may be also associated with membranous nephropathy. Another complication of NSAIDs treatment is modest rise of systemic blood pressure in some hypertensive patients due to increase in renal and systemic vascular resistence. In patients consuming excessive amount of NSAIDs over a prolonged period of years papillary necrosis can occur. Exposure to large quantities of NSAIDs can probably induce in some patients chronic renal insufficiency.     

急性HBV感染合并肾功能衰竭及多器官损害1例

急性乙型肝炎并发肾功能衰竭十分少见,其肝外表现通常为血清病样表现,包括发热、皮疹和多动脉炎。我们报道1位男性34岁患者,诊断为急性HBV感染,临床表现为肝脏和肝外多器官的损害,以肾功能衰竭为突出的表现,推测是由循环免疫复合物沉积在肾小球血管壁,导致膜性肾小球肾炎而引起的。患者接受了血液透析和持续性血液滤过治疗、常规的护肝和对症治疗及恩替卡韦抗病毒治疗,最后痊愈。     

Leptospirosis in the tropics and in travelers

Leptospirosis, caused by spirochetes of the genus Leptospira, has increasingly been recognized to affect travelers and residents in tropical settings. A zoonotic disease, leptospirosis is transmitted to humans through environmental surface waters contaminated by the urine of chronically infected mammals. Outcome of infection varies, ranging from acute febrile illness (including selfresolving undifferentiated fever) to aseptic meningitis to a fulminant syndrome of jaundice, oliguric renal failure, pulmonary hemorrhage, and refractory shock. Hospitalized cases have mortality rates as high as 25%. A recent clinical trial showed that third-generation cephalosporin is as effective as doxycycline and penicillin in the treatment of acute disease. Doxycycline is effective in preventing leptospirosis in travelers. No protective vaccine is currently available.     

Nonoliguric acute renal failure complicated with tumor lysis syndrome in chronic myelocytic leukemia in lymphoid crisis

A case of nonoliguric acute renal failure complicated with tumor lysis syndrome is described. The patient is a 14-year-old boy who was diagnosed chronic myelocytic leukemia 17 months ago. On lymphoid crisis, he received vindesine-prednisolone therapy and acute renal failure occurred. Urine output was kept enough volume (2,500-4,000 ml/day), but blood urea nitrogen and serum creatinine levels rose and hyperkalemia, hyperphosphatemia and hypocalcemia were observed. Tumor lysis syndrome in patients with chronic myelocytic leukemia is rare, and acute renal failure with tumor lysis syndrome is oliguric or anuric in most patients. At therapy of lymphoproliferative disease, nonoliguric acute renal failure may occur. Physicians who treat patients with lymphoproliferative disease should pay attention to blood urea nitrogen and serum creatinine levels even if urine output is satisfactory.     

以急性胰腺炎为主要特征的肾综合征出血热3例

肾综合征出血热以急性胰腺炎为主要特征表现较少,表现特殊,易造成误诊.本3例患者临床表现均以腹痛为主,查血尿淀粉酶明显增高,CT及彩超胰腺肿大,易误诊为"急性胰腺炎".后经感染科会诊结合流血病学,临床特征与实验室检查才得以确诊.临床医生需开阔思路,在临床诊治过程中遇到类似病例不排外为肾综合征出血热.     

Acute renal failure in a case of nephrotic syndrome secondary to focal and segmental glomerulosclerosis

Nephrotic syndrome is infrequently complicated with appearance of acute renal failure and minimal change disease is the glomerulopathy more usually involved. Pathogenesis is unclear and three possible mechanisms it has been proposed to explain the decrease of glomerular filtration rate: a severe reduction of glomerular permeability, the presence of acute tubular necrosis or an increased intrarenal pressure related with interstitial oedema. Here we present a 36 years-old-male with a nephrotic syndrome caused by focal and segmental glomerulosclerosis who developed an anuric acute renal failure. Renal function did not change despite oedema removal with haemodialysis and only after corticosteroid and cyclophosphamide therapy introduction we observed a rapid recovery of urinary output and resolution of acute renal failure. Renal biopsy did not show signs of tubular damage or obstruction with proteins nor significant interstitial oedema. Therefore, in this case we think acute renal failure was caused by a severe reduction in glomerular ultrafiltration rate and steroids were the effective treatment that allowed recovery of renal function.     

Akutes Nierenversagen

The acute renal failure is characterized by a rapid deterioration of the renal function. In addition to the usual prerenal, intrinsic and postrenal causes of an acute renal failure distinct causes have to be considered for oncological patients. Factors imminent to the malignant disease, e. g. paraneoplastic syndromes or retroperitoneal bulks can account for an acute renal failure. Paraproteins as produced by a multiple myeloma are other possible causes for renal dysfunction. For some anticancer drugs nephrotoxicity is a potential side effect, in particular for cisplatin, methotrexate, ifosfamide and melphalan. A hemolytic uremic syndrome may be induced by mitomycin and gemcitabine. Extensive surgery can be associated with rhabdomyolysis and myoglobinuria and results in renal impairment. Treatment of a chemosensitive neoplasia with a highly effective regimen may result in a tumor lysis syndrome with hyperuricemia, hyperkalemia, hyperphosphatemia and hypocalcemia.