Segmental necrotizing glomerulonephritis: diagnostic, prognostic, and therapeutic significance

Renal biopsies from 50 patients with segmental necrotizing glomerulonephritis (SNGN) were divided into three groups on the basis of initial clinical information: (group A) Wegener's granulomatosis (WG)--14 patients; (group B) SNGN without renal vasculitis (RV)--21 patients; and (group C) SNGN with RV--15 patients. Renal biopsy findings did not distinguish the SNGN in WG from non-WG patients. However, focal endocapillary proliferation was more common in non-WG groups B (48%) and C (33%) than in WG (7%). In addition, GBM deposits of both IgG and C3 were present in 35% of biopsies in group B and 33% in group C in comparison to only 7% in WG. Glomerular fibrin deposition was common in all groups (54% group A, 70% group B, and 100% group C), suggesting that coagulation plays a role in the development of SNGN. Histologic parameters of severity and chronicity of the SNGN were inconsistent predictors of outcome, although an increased percentage of crescents in the non-WG groups correlated with a poorer prognosis. Chronic renal failure developed in 46% of group A patients, 65% group B, and 73% group C. After clinical follow-up, 15 patients had WG, 15 patients had documented or suspected systemic vasculitis (SV), and idiopathic SNGN was present in 20 patients. Sixty-six percent of patients with SV had RV, and 62% of biopsies with RV were from patients with SV. Chronic renal failure developed in 78% of patients with idiopathic SNGN and 57% patients with SV. These findings confirm that SNGN carries a poor prognosis, independent of its association with WG or SV. Fourteen of the 15 WG patients were treated with alkylating agents, and the development of chronic renal failure appeared to be related to delays in diagnosis and therapy. In the non-WG groups, presentation in acute renal failure with high serum creatinine and long duration of symptoms was predictive of development of chronic renal failure. Therapy in the non-WG patients consisted of alkylating agents (seven patients), steroids (20 patients), and dialysis only (seven patients). The seven non-WG patients treated with alkylating agents had clinical responses similar to WG patients, and cyclophosphamide therapy appeared to be most beneficial to patient outcome. Results of this retrospective study stress the importance of early diagnosis and, although based on small numbers of patients, suggest that aggressive chemotherapy should be recommended for SNGN, independent of its association with biopsy-proven WG or documented SV.     

ANCA-associated glomerulonephritis/systemic vasculitis in childhood: clinical features–outcome

Background

Antineutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis and systemic vasculitis (AAGNV) is uncommon in childhood.

Methods

This is a retrospective study of AAGNV cases diagnosed over a 13-year period in a tertiary pediatric nephrology department.

Results

Thirteen cases of AAGNV were identified: seven Wegener granulomatosis (WG) and six microscopic polyangiitis (MPA). Acute renal failure/nephrotic range proteinuria (NRP) was found in 77 % of the patients (4 with WG, all with MPA). Eleven (85 %) patients showed necrotizing glomerulonephritis (NGN), with ≥50 % crescents identified in nine patients (69 %) (4 with WG, 5 with MPA). Treatment with methylprednisolone, cyclophosphamide and plasma exchange resulted in extra-renal remission and antibody reduction in all patients and renal function improvement/stabilization in 77 % of the patients. Three patients, all without oliguria at presentation and few sclerotic lesions, had normal renal function at follow-up. Chronic kidney disease (CKD) stages 2 and 3–4 were observed in four (WG) and three (MPA) patients, respectively. Three patients (23 %) developed end stage renal disease: two were MPA patients with severe presentation (markedly impaired glomerular filtration rate, oliguria, NRP, crescentic NGN, glomerular sclerosis) and one was a WG patient with extensive interstitial fibrosis/tubular atrophy.

Conclusions

Severe renal involvement was more common in children with MPA than WG. Treatment with methylprednisolone, cyclophosphamide and plasma exchange induced extra-renal remission/serological response and renal function improvement/stabilization. Markedly decreased GFR, oliguria, NRP, and chronic glomerular lesions at presentation were predictors of poor outcome.     

Renal histology in Chinese patients with anti-myeloperoxidase autoantibody-positive Wegener's granulomatosis.

BACKGROUND: Proteinase-3 antineutrophil cytoplasmic antibody (PR3-ANCA) was the serological marker for Wegener's granulomatosis (WG), while myeloperoxidase (MPO)-ANCA was the serological marker for microscopic polyangiitis (MPA). However, our previous study suggested that patients with MPO-ANCA positive WG were common in Chinese. This study aimed to analyse the renal histology of patients with MPO-ANCA positive WG. METHODS: Patients in our centre with WG were selected according to both the Chapel Hill Consensus Conference (CHCC) definition and American College of Rheumatology classification criteria. Patients with MPA were selected according to the CHCC definition. The renal histology was compared between patients with MPO-ANCA positive WG and with PR3-ANCA positive WG as well as patients with MPO-ANCA positive MPA. RESULTS: Sixty-one patients with WG had complete renal histological data, 39/61 with positive MPO-ANCA and 22/61 with positive PR3-ANCA. Among patients with crescents in glomeruli, those with MPO-ANCA had fewer cellular crescents and more fibrous crescents than those with PR3-ANCA (P < 0.01 and P < 0.05, respectively). Interstitial fibrosis and tubular atrophy were more prevalent and severe in patients with MPO-ANCA than in those with PR3-ANCA (P < 0.01 and P < 0.05, respectively). Compared with 44 patients with MPO-ANCA positive MPA, patients with MPO-ANCA positive WG had fewer glomeruli with crescents and more normal glomeruli (P < 0.01 and P < 0.01, respectively). CONCLUSION: Patients with MPO-ANCA positive WG are common in Chinese. In renal histology, chronic lesions were more severe and prevalent in patients with MPO-ANCA positive WG than in patients with PR3-ANCA positive WG. Glomerular lesions were less severe and less prevalent in patients with MPO-ANCA positive WG than in those with MPO-ANCA positive MPA.     

A case of exorbitism in association with Wegener's granulomatosis with renal involvement

Wegener's granulomatosis (WG) is a necrotizing granulomatous vasculitis involving the nose, paranasal sinuses, lungs, and kidneys. Ocular involvement can occur in about 50% of cases. There are very few reports of WG with orbital inflammation and exorbitism. We report a case of a female patient who presented with exorbitism related to orbital inflammation secondary to WG, with renal involvement. A 29-year-old woman with a previous history of recurrent pan-sinusitis presented with bilateral exophthalmos and renal failure with rapidly progressive glomerulonephritis. Computed tomography showed extensive bilateral soft tissue in the retro-orbital area. Immunologic tests showed the presence of type-C anti-neutrophil cytoplasmic antibodies and renal biopsy revealed pauci immune crescentic glomerulonephritis. The patient was treated with corticosteroids and pulses of cyclophosphamide followed by azathioprine and trimethoprim-sulfamethoxazole. After a follow-up of 10 months, the renal outcome was favorable with improvement of renal function but there was persistence of exorbitism and loss of visual function. Our case suggests that WG should be considered in the differential diagnosis of persistent bilateral exophthalmos. Prompt recognition of this early manifestation is important for the institution of early treatment.     

Hémorragie sous-arachnoïdienne anévrismale et maladie de Wegener : une association peu commune

Background

Wegener granulomatosis (WG) is an uncommon systemic necrotizing vasculitis that demonstrates renal and respiratory tropism. While the pathogenesis of WG remains controversial, autoimmune and inflammatory mechanisms are likely to be involved. The nervous system could be affected in up to 54% of cases. Although central nervous system involvement has been reported in 7-11% of cases, aneurysmal subarachnoid hemorrhage (SAH) occurrence is exceptional.

Methods

We describe the third reported case of WG-related aneurysmal SAH and then discuss the diagnosis and pathogenesis of WG along with the physiopathology of intracranial aneurysm in light of recent data reported in the literature.

Results

A 63-year-old woman with WG was referred to our neurosurgical department for aneurysmal SAH. The vasculitis diagnosis had been established 4 years earlier when she presented with chronic sinusitis, recurrent cystitis, and renal failure. The cerebral angiography revealed an anterior communicating artery dysplastic aneurysm. The neurosurgical management of the aneurysm was scheduled but delayed because the patient was experiencing a vasculitis flare-up. Immunosuppressive therapy and intravenous corticotherapy were given, with the patient's improvement, allowing neurosurgical clipping of the aneurysm.

Conclusions

Wegener granulomatosis-related aneurysmal SAH is an exceptional condition in neurovascular pathology. As inflammatory mechanisms are involved in the pathogenesis of aneurysm, the vasculitis flare-up could account for this SAH. The management of WG could benefit from anti-inflammatory therapy, as could the vasculitis-related SAH. SAH occurrence in patients with systemic vasculitis could indicate a vasculitis flare-up.     

High proteinase 3-anti-neutrophil cytoplasmic antibody (ANCA) level measured by the capture enzyme-linked immunosorbent assay method is associated with decreased patient survival in ANCA-associated vasculitis with renal involvement

Wegener granulomatosis (WG) and microscopic polyangiitis (MP), diseases associated with antineutrophil cytoplasmic antibodies (ANCA), had an extremely poor prognosis before the introduction of cyclophosphamide and corticosteroids for their treatment. However, there is still reduced patient survival, and some studies have documented severe side effects of the immunosuppressants used. This 10-yr follow-up study assessed 117 consecutive patients with WG or MP with biopsy-confirmed renal involvement. The cumulative relative patient survival was lower: 0.664 for women and 0.648 for men. The causes of death (n = 64) were in most cases registered as associated with the vasculitic disease. Analysis of possible predictive factors for patient survival by multiple Cox regression analysis revealed that a very high level of proteinase 3 (PR3)-ANCA measured by the capture ELISA method, a diagnosis of MP, and older age were factors predicting poorer patient survival. High levels of B-thrombocytes at time of diagnosis were associated with a better prognosis. For patients surviving the first year, remission-sustaining therapy with azathioprine for longer than 12 mo was associated with improved patient survival. Thirty-nine patients developed end-stage renal failure. Elevated serum creatinine at time of diagnosis and a very high level of PR3-ANCA by capture ELISA were factors predicting a higher risk for renal failure during follow-up. The epitope on PR3 assessed by capture ELISA needs to be further analyzed and explored: it seemed to implicate poorer patient and renal survival in WG or MP with renal involvement.     

Clinical significance of autoantibodies against neutrophil cytoplasmic components in patients with renal disease

The diagnostic significance of anticytoplasmic autoantibodies (ANCA) was studied in 71 renal patients. The ANCA test was positive in 67% of patients with Wegener's granulomatosis (WG), in 35% of those with a simultaneous renal and respiratory tract disease but not diagnosed as WG and in 22% of patients with a renal disease associated with unspecific collagenosis/vasculitis. Among WG patients ANCA positivity clearly correlated with the presence of active renal disease. Interestingly, both ANCA-positive and -negative patients were encountered in the group with acute renal failure and acute extracapillary glomerulonephritis associated with diffuse pulmonary infiltrates. The diagnostic and clinical significance of the ANCA test in these cases remains for the present obscure. In the majority of the ANCA-positive renal patients with respiratory tract abnormalities, the antibodies showed diffuse cytoplasmic staining and were mostly of the IgG class, of both IgG and IgM classes in some cases and of IgG, IgM and IgA classes in 1 patient. In patients with unspecific vasculitis/collagenosis the level of ANCA was rather low, and the distribution of different isotypes resembled that of patients with respiratory symptoms. A certain isotype of ANCA or staining pattern did not mark out any clinicopathologic subgroup among the patients. Our findings indicate that the clinical picture of ANCA-positive patients varies considerably and the ANCA test may not be as specific a marker of WG as previously suggested.     

Outcomes of renal transplantation in recipients with Wegener’s granulomatosis

Shen J, Gill J, Shangguan M, Sampaio MS., Bunnapradist S. Outcomes of renal transplantation in recipients with Wegener’s granulomatosis.
Clin Transplant 2011: 25: 380–387. © 2010 John Wiley & Sons A/S. Abstract: Wegener’s granulomatosis (WG) is the leading cause of rapidly progressive glomerulonephritis‐induced end‐stage renal disease (ESRD). In this study, we compared transplant outcomes between recipients with ESRD caused by WG to recipients with ESRD secondary to other causes. Using OPTN/UNOS data from 1996 to 2007, 919 recipients with WG were identified. Post‐transplant outcomes included rates of delayed graft function, acute rejection within one‐yr post‐transplant, overall and death‐censored graft survival, and patient survival and were compared between recipients with ESRD secondary to WG versus ESRD from other causes. Recipients with ESRD because of WG had superior unadjusted and adjusted rates of graft loss, patient death, and functional graft loss (adjusted hazard ratio [HR] 0.711, 0.631, and 0.625 respectively, p < 0.001). When we compared the WG cohort to a non‐WG, non‐diabetic population, the HR for graft loss was still significant, but patient death and death‐censored graft loss were not. Subgroup analysis of recipients aged over 60 confirmed that WG recipients had better unadjusted outcomes. This study supports the notion that renal transplantation is an effective treatment option for patients with ESRD secondary to WG. They fare similarly, if not better, than other patients.     

Wegener's granulomatosis presenting as renal mass: a case for nephron-sparing surgery

Wegener's granulomatosis (WG) is a systemic disease well recognized by physicians. The upper and lower respiratory symptoms associated with the disease are easily recognized; however, its presentation as a solid renal mass is underappreciated. We present a case of a 61-year-old man who presented with a 5.2-cm renal mass diagnosed as WG after nephron-sparing surgery. The patient presented with mild symptoms suggestive of WG, but the antineutrophil cytoplasmic antibody test was negative. The renal mass and concerns about possible malignancy obscured the diagnosis of WG. This case illustrates that WG should be considered in the differential diagnosis of solid renal masses.     

Outcomes of renal failure and dialysis patients undergoing anatomic and reverse shoulder arthroplasty

BackgroundAs anatomic and reverse shoulder arthroplasty incidence increases and outcomes improve, those with severe pre-existing comorbidities may be indicated for surgery. Renal failure patients, including those on dialysis, may have systemic physiologic effects impacting their recovery after shoulder arthroplasty. We hypothesized that renal failure patients would have worse near-term outcomes and increased overall revision rates.MethodsAn institutional cohort of 1773 anatomic and reverse shoulder arthroplasty cases performed between July 2013 and May 2019 was retrospectively reviewed for the presence of renal failure as defined by the Elixhauser comorbidity index. This cohort was also reviewed for patients on preoperative dialysis. Near-term outcomes were compared, including inpatient length of stay (LOS), unplanned 90-day readmission, and discharge to a rehabilitation or skilled nursing facility (SNF). Revision status and indication were collected for all renal failure patients, including those having a minimum 1-year and 2-year clinical follow-up, and these outcomes were tested for comparative significance (P < .05) with chi-square tests.ResultsOne hundred fifty-nine patients were positive for renal failure at the time of surgery (62% reverses, P < .0001), 6 of which were already on dialysis. Sixty-two patients had minimum 1-year clinical follow-up (mean overall follow-up: 1.0 years). Dialysis patients experienced longer inpatient LOS relative to nondialysis renal failure patients (3.2 vs. 2.5 days, P = .0112), and renal failure patients overall had a longer LOS relative to non–renal failure patients (2.6 vs. 2.1 days, P < .0001). Ninety-day readmission rates and SNF/rehab utilization were higher in dialysis patients and renal failure patients overall. While readmissions trended toward significance, SNF rates reached statistical significance (17.0% vs. 10.5%, P = .0125). Postacute care utilization was related to stage of renal failure (P = .03442), inpatient LOS trended toward significance, and revision status, and unplanned 90-day readmission was not related to the stage of renal failure. Eleven revisions (6.9%) were observed in the renal failure cohort, more frequently in dialysis patients (50.0% vs. 5.2%, P < .0001). The overall 2-year survival on Kaplan-Meier analysis was 87%.ConclusionRenal failure patients (especially those on dialysis) have relatively poor 2-year survival rates, and although many still benefit from shoulder arthroplasty, they should be counseled preoperatively regarding elevated risks, including higher revision rates, postacute care utilization, possibly higher 90-day readmission rates, and longer inpatient LOS. Of note, complications observed in our cohort were primarily related to instability or subscapularis insufficiency rather than infection.     

Alteration of the Intestinal Environment by Lubiprostone Is Associated with Amelioration of Adenine-Induced CKD

The accumulation of uremic toxins is involved in the progression of CKD. Various uremic toxins are derived from gut microbiota, and an imbalance of gut microbiota or dysbiosis is related to renal failure. However, the pathophysiologic mechanisms underlying the relationship between the gut microbiota and renal failure are still obscure. Using an adenine-induced renal failure mouse model, we evaluated the effects of the ClC-2 chloride channel activator lubiprostone (commonly used for the treatment of constipation) on CKD. Oral administration of lubiprostone (500 µg/kg per day) changed the fecal and intestinal properties in mice with renal failure. Additionally, lubiprostone treatment reduced the elevated BUN and protected against tubulointerstitial damage, renal fibrosis, and inflammation. Gut microbiome analysis of 16S rRNA genes in the renal failure mice showed that lubiprostone treatment altered their microbial composition, especially the recovery of the levels of the Lactobacillaceae family and Prevotella genus, which were significantly reduced in the renal failure mice. Furthermore, capillary electrophoresis–mass spectrometry-based metabolome analysis showed that lubiprostone treatment decreased the plasma level of uremic toxins, such as indoxyl sulfate and hippurate, which are derived from gut microbiota, and a more recently discovered uremic toxin, trans-aconitate. These results suggest that lubiprostone ameliorates the progression of CKD and the accumulation of uremic toxins by improving the gut microbiota and intestinal environment.     

De novo glomerulonephritis in patients during remission from Wegener's granulomatosis.

In a cohort of 20 consecutive patients with Wegener's granulomatosis and biopsy-proven pauci-immune crescentic glomerulonephritis three patients were in remission, but developed again a nephritic sediment without signs of systemic disease or positive ANCA titers. The second renal biopsy showed de novo mesangial IgA deposits 6, 17 and 28 months following admission for systemic disease and institution of immunosuppressive treatment. All patients were male, HLA-DR-2 positive and exhibited repeated upper respiratory tract infections. A fourth patient was admitted in end-stage renal failure with high titers of C-ANCA of the IgG isotype and proteinase 3 ab without clinical evidence of systemic manifestations of WG. Renal biopsy showed chronic sclerosing GN with marked IgA deposits. De novo development of IgA-GN is observed in a remarkable proportion of patients with WG and must be distinguished from exacerbation of the systemic disease.     

Renal histology in ANCA-associated vasculitis: differences between diagnostic and serologic subgroups.

BACKGROUND: Differences in renal histopathology between microscopic polyangiitis (MPA) and Wegener's granulomatosis (WG), and between anti-neutrophil cytoplasm autoantibody (ANCA) test results in patients with ANCA-associated vasculitis may provide insight into the differences in pathogenesis and raise the opportunity of classifying the vasculitides more accurately. The possible differences in histopathology are investigated in this study. METHODS: We report an analysis of 173 patients with renal disease in microscopic polyangiitis or Wegener's granulomatosis. A total of 173 renal biopsies, performed at diagnosis, were scored by two observers separately, using a previously standardized protocol. Consensus on each biopsy was achieved during a central review. RESULTS: Normal glomeruli were more common in WG than in MPA (P < 0.001). Glomerulosclerosis was more prominent in MPA than in WG (P=0.003). Interstitial fibrosis (P < 0.001), tubular atrophy (P < 0.001), and tubular casts (P=0.005) were more frequently present and more severe in MPA than in WG. Presence of glomerulosclerosis was more extensive in patients with myeloperoxidase (MPO)-ANCA than with proteinase 3 (PR3)-ANCA (P=0.022). Interstitial fibrosis (P=0.008), tubular necrosis (P=0.030), tubular atrophy (P=0.013), and intra-epithelial infiltrates (P=0.006) were more frequently present and more severe in MPO-ANCA than in PR3-ANCA. CONCLUSIONS: Glomerulonephritis in relation to MPA has more characteristics of chronic injury at the time of presentation than glomerulonephritis in relation to WG. This difference may be due to a delayed establishment of diagnosis in patients with MPA compared to patients with WG. Both active and chronic lesions are more abundantly present in MPO-ANCA-positive patients than in patients with PR3-ANCA-positivity, which suggests that the pathogenesis of renal disease in these ANCA subsets could be different. Our results also suggest that ANCA test results may be useful in classifying ANCA-associated vasculitides.     

Use of EuroSCORE as a predictor of morbidity after cardiac surgery

Objective

To evaluate the use of the EuroSCORE as a predictor of postoperative morbidity after cardiac surgery.

Methods

We retrospectively analyzed the charts of 900 patients operated on and admitted to the intensive care unit postoperatively at the Royal Portuguese Hospital of Recife. We included all patients with complete medical records, excluding those who died during surgery, underwent transplantation or correction of congenital heart disease. We evaluated the development of respiratory infection, cerebrovascular accident, and dialysis-dependent renal failure, and the EuroSCORE was compared in terms of the three complications using the Mann-Whitney test. The calibration model for predicting the morbidities being studied was evaluated using the test set of Homer-Lemeshow goodness. The accuracy of the model was assessed using the area under the ROC curve (AUROC).

Results

The model showed good calibration in predicting respiratory infection, acute renal failure and stroke (P=0.285, P=0.789, P=0.45, respectively), with good accuracy for respiratory infection (AUROC=0.710 and P<0.001) and dialysis-dependent renal failure (AUROC=0.834 and P<0.001), but no accuracy to predict stroke (AUROC=0.519). The high-risk patients were more likely to develop respiratory infection (OR=9.05, P<0.001) and dialysis-dependent renal failure (OR=39.6, P<0.001). The probability of developing respiratory infection and dialysis-dependent renal failure was less than 10% with EuroSCORE up to 7 and more than 70% with EuroSCORE greater than 15.

Conclusion

EuroSCORE proved to be a good predictor of major postoperative morbidity in cardiac surgery: respiratory and dialysis-dependent renal failure.     

Identification of patients best suited for combined liver[ndash ]kidney transplantation: Part II

Liver-kidney transplantation (LKT) should be reserved for those recipients with primary disease affecting both organs. However, increasing transplant list waiting times have increased the development and duration of acute renal failure before liver transplantation. Furthermore, the need for posttransplant calcineurin inhibitors can render healing from acute renal failure difficult. Because of the increasing requests for and controversy over the topic of a kidney with a liver transplant (OLT) when complete failure of the kidney is not known, the following article will review the impact of renal failure on liver transplant outcome, treatment of peri-OLT renal failure, rejection rates after LKT, survival after LKT, and information on renal histology and progression of disease into the beginnings of an algorithm for making a decision about combined LKT. (Liver Transpl 2002;8:193-211.)     

ANCA in haemodialysis patients

The prevalence of positive ANCA as well as the prevalence ofPR-3 and MPO antibodies were examined in a cross-sectional sampleof 1277 haemodialysis patients from 16 German haemodialysiscentres. We found 32 patients positive for c-ANCA (median titre1:40; range 1:20–1:320) and 65 for p-ANCA (1:80; 1:20–1:1280).Twenty-two percent of the c-ANCA-positive and 31% of the p-ANCA-positivepatients had PR-3 and MPO antibodies by ELISA respectively.Clinical evidence of vasculitis was found in 11 of 32 c-ANCA-positiveand 19 of 65 p-ANCA-positive patients. Of the 11 c-ANCA-positive,four had a known diagnosis of Wegener's granulo-matosis (WG);WG was recognized after the test in a further five patientsand two had renal limited RPGN. Of the 19 p-ANCA-positive patients,three had a clinical diagnosis of microscopic polyarteritis(MP), MP was newly diagnosed in a further 12, WG in one andrenal limited RPGN in three. The patients had not received cyclophosphamide(the diagnosis had been non-specified ‘systemic disease’).Thus false-positive ANCA, as defined by absence of vasculitis,was found in 5% of dialysis patients versus 0% in patients withpreterminal renal failure {n=152) or blood donors (n=150). Patientswith vasculitis tended to have higher c-ANCA and p-ANCA titresrespectively, but there was a considerable overlap. Titres werenot higher in patients symptomatic at the time of examination(6 of 11 c-ANCA and 10 of 19 p-ANCA), but PR-3 and MPO ELISAwere positive in all but two. Thus c-ANCA were false positive(i.e. not associated with clinical vasculitis) in 66% and p-ANCAin 71%. In some patients ANCA was positive, but ELISA negative. We conclude that (i) in dialysis patients c-ANCA and p-ANCAare frequently present in the absence of vasculitis; (ii) specificitycan be improved, but sensitivity is lost, by using PR-3 andMPO ELISA respectively; (iii) serology permits the recognitionof WG or MP in cases where the diagnosis has been missed. Itis recommended that all patients with unknown primary renaldisease admitted to renal replacement therapy be examined usingboth ANCA-IF and PR-3/MPO ELISA.     

Acute tubulointerstitial nephritis after wasp stings

A 61-year-old Caucasian man presented with acute renal failure after multiple wasp stings. The patient required dialysis support temporarily. Work-up failed to show rhabdomyolysis or hemolysis and a kidney biopsy revealed acute allergic interstitial nephritis. The patient's renal function recovered completely after a short course of steroid therapy. Acute renal failure after wasp stings is typically caused by acute tubular necrosis in the setting of hemolysis or rhabdomyolysis. Compared with previously reported cases of acute renal failure associated with bee stings, our patient is unique in that his renal failure was caused by a hypersensitivity reaction apparently to the wasp venom. © 2001 by the National Kidney Foundation, Inc.     

A tubulointerstitial nephritis antigen gene defect causes childhood-onset chronic renal failure

Tubulointerstitial nephritis antigen (TIN-ag), which has been localized to the renal tubular basement membrane, is a target antigen in some forms of TIN. Physiologically, TIN-ag is thought to be important in maintaining the structure of renal tubular basement membrane. Here we describe a child with chronic renal failure showing a human TIN-ag gene (hTIN-ag) deletion. Immunohistochemical examination using an antihuman TIN-ag monoclonal antibody showed attenuation or lack of TIN-ag staining along the renal tubular basement membrane, whereas nephrocystin staining was normal in renal tubules. Polymerase chain reaction detected no amplification band corresponding to hTIN-ag in this patient. Testing for a deletion in this gene showed nearly complete deletion. By using array-comparative genomic hybridization method, large deletion of a gene mapped on chromosome 6p11-6p12 was demonstrated, corresponding to the locus where hTIN-ag is located. Therefore, an hTIN-ag defect may be a potent cause of end-stage renal failure in childhood.     

Significance of past history of renal failure for the detection of high-risk individuals for cardiovascular and end-stage renal disease: analysis of data from a nationwide health checkup

Background

Clinical information regarding risk factors may be helpful in the detection of various diseases. This cross-sectional study examined the characteristics of subjects with past history of renal failure, and assessed whether this information would be useful for the efficient detection of high-risk individuals for chronic kidney disease (CKD) and cardiovascular disease (CVD) at health checkup.

Methods

This study utilized data from a nationwide health checkup, ??The Specific Health Check and Guidance in Japan,?? and data for 250,130 adult subjects were analyzed. Subjects with self-reported history of renal failure and receiving dialysis therapy were defined as having a history of renal failure.

Results

Among total participants, there were 1,400 (0.6%) with a history of renal failure. The prevalence of a history of renal failure was higher in subjects with CKD than in those without CKD (1.5 vs. 0.3%, P?2) and a higher prevalence of CKD (50.5%) and CVD (31.9%), compared with subjects with hypertension, diabetes or metabolic syndrome. Multivariate logistic regression analysis showed an independent association between a history of CVD and renal failure (odds ratio 3.68, 95% confidence interval 3.26?C4.15), after adjustment for confounding factors.

Conclusions

A history of renal failure was strongly associated with advanced CKD and CVD. Information regarding history of renal failure could be utilized to efficiently detect high-risk individuals at health checkup.     

Fenoldopam prophylaxis of postoperative acute renal failure in high-risk cardiac surgery patients

Background

Acute renal failure requiring replacement therapy occurs in 1% to 2% of patients who have undergone cardiac surgery with cardiopulmonary bypass and is associated with a very high mortality rate. The aim of this study was to determine if prophylactic treatment with fenoldopam mesylate of patients at high risk of postoperative acute renal failure reduced the incidence of this event.

Methods

This was a multicenter, prospective, cohort study in which 108 patients at high risk of postoperative acute renal failure and undergoing cardiac surgery with cardiopulmonary bypass were treated with fenoldopam mesylate (0.08 μg · kg⁻¹ · min⁻¹) starting at the induction of anesthesia and throughout at least the next 24 hours. A homogeneous control group of 108 patients was created using a propensity-score analysis.

Results

Fenoldopam prophylaxis was significantly associated with a reduction in acute renal failure incidence (from 22% to 11%, p = 0.028), a less pronounced creatinine clearance decrease (p = 0.05), and a lower mortality rate (6.5% versus 15.7%, p = 0.03) by the univariate analysis, but these results were not confirmed by a multivariable analysis. Within the subgroup of patients who suffered a postoperative low output syndrome, fenoldopam prophylaxis was an independent protective factor for postoperative renal failure (odds ratio, 0.14; 95% confidence interval, 0.03 to 0.7; p = 0.017).

Conclusions

Given the limitations of a nonrandomized prospective trial, our results support the hypothesis that fenoldopam may reduce the risk of acute renal failure in patients in whom endogenous and exogenous cathecolamines action may induce a renal vascular constrictive condition.