Do protection devices have a role in renal angioplasty and stent placement?

In renal atherosclerotic vascular disease there is often nofirm randomized trial data to guide clinical practice. Nephrologistsoften have to interpret trial data from other vascular territories.Vascular protection devices are now an issue in the carotidand cardiac territories. Should they have a role in renal atheroscleroticdisease? There is considerable literature on renal atheroembolic disease.The early paper of Thurlbeck and Castleman [1] showed that inpost-mortem renal histology analysis after aortic aneurysm repairfollowed by death there was an incidence of renal atheroembolicdisease of >70%. It also showed that there was a >20%incidence of atheroembolic disease in individuals with no interventionbut severe aortic atherosclerotic disease. Flory [2] showedthat this condition is related to cholesterol crystal embolizationto the renal vascular bed and suggested that the vessels occludedwere between 55 and 900 µm in diameter. There     

Renal transplantation in a patient with end stage renal disease due to cholesterol embolism

BACKGROUND: Renal failure due to cholesterol emboli is mostly irreversible. Therefore chronic renal replacement therapy is necessary. However, to the best of our knowledge no published experience exists with renal transplantation in patients with end-stage renal disease (ESRD) due to cholesterol embolization (CE). METHODS: Renal transplantation was performed in a 64-year-old man who suffered from ESRD due to CE after coronary angiography. Because our patient presented with a typical profile of cardiovascular risk factors effective long-term control of these risk factors before and after transplantation was a mandatory prerequisite before considering transplantation. RESULTS: After one rejection episode serum creatinine values have been stable and no major complications have occurred during a follow-up of 18 months. No signs of recurrent cholesterol emboli into the donated kidney were seen in renal biopsies performed due to graft rejection. CONCLUSION: Cholesterol embolization is an uncommon reason for ESRD and mainly occurs after invasive vascular procedures in patients with hyperlipidemia, arterial hypertension, and smoking. Because ESRD due to CE often is irreversible, chronic renal replacement therapy may be necessary. As demonstrated in our report, renal transplantation should be considered. However, in this setting effective long-term control of the underlying risk factors before and after renal transplantation has to be ensured.     

Cholesterol atheroembolic renal disease. Report of 3 cases with emphasis on diagnosis by skin biopsy and extended survival

Because antemortem diagnosis is difficult, renal failure due to cholesterol atheroembolism has, until recently, been regarded as a uniformly irreversible and generally fatal disease. Of late, recovery of renal function in several patients in whom the diagnosis was made by organ or other invasive biopsy has been reported. Three cases of cholesterol atheroembolic renal failure in which the diagnosis was made by simple, noninvasive biopsy of the skin in areas showing livedo reticularis are described. Two of the patients, including 1 who required dialysis for 2 months, had an extended survival with recovery of renal function.     

Clinical features and outcomes in children with antineutrophil cytoplasmic autoantibody-positive glomerulonephritis associated with propylthiouracil treatment.

A retrospective investigation was conducted by members of the Japanese Society for Pediatric Nephrology from 1990 to 1997 to define the clinical features and outcomes in children with antineutrophil cytoplasmic autoantibody (ANCA)-positive glomerulonephritis associated with propylthiouracil treatment. Seven Japanese pediatric patients who had myeloperoxidase-specific ANCA-positive biopsy-proven pauci-immune necrotizing crescentic glomerulonephritis associated with propylthiouracil administration were entered in the study. Three patients had nephritis alone, and four had nephritis and extrarenal organ system vasculitis. Females predominated, and the mean age at onset was 14 yr. Propylthiouracil was reduced or discontinued in all patients and was switched to methimazole in three patients. For the treatment of nephritis, five patients received corticosteroids; three had pulse methylprednisolone, one had plasma exchange, and one had plasma exchange and pulse methylprednisolone before initiating oral prednisolone. The remaining two patients received cyclophosphamide and corticosteroids, one of whom had pulse methylprednisolone before initiating oral prednisolone and cyclophosphamide. All patients achieved remission. In general, ANCA titers correlated with the response to treatment and disease activity, with some exceptions. No patient progressed to end-stage renal disease, renal dysfunction, or death during the follow-up period (58 +/- 25 mo; range, 32 to 108 mo). All but one patient remained euthyroid. In conclusion, this experience suggests that the clinical disease spectrum of ANCA-positive disease associated with propylthiouracil treatment is similar in pediatric and adult patients and that the overall prognosis may be better than that in the non-drug-induced ANCA-positive disease.     

Plasma exchange in Goodpasture's syndrome

The clinical course and levels of anti-glomerular basement membrane (GBM) antibody were compared in 20 patients with Goodpasture's syndrome treated with plasma exchange and immunosuppression (8 patients), immunosuppression alone (4 patients) or no specific therapy (8 patients). There was a more rapid fall in the level of anti-GBM antibody and pulmonary hemorrhage was less protracted in the 8 patients treated with plasma exchange and immunosuppression. In this group, 1 patient who presented with severe renal failure showed a marked improvement of renal function and there was no progression of disease in the 4 with milder renal involvement. 2 of the 4 patients treated with immunosuppression alone, and only 2 of the 8 patients who received no specific therapy, maintained normal renal function. In the group which received no specific therapy, 1 of the 6 patients who progressed to renal failure had mild renal involvement initially. There was a significant correlation between the level of anti-GBM antibody and the severity of the morphological changes seen at renal biopsy but not between the level of anti-GBM antibody and the severity of lung hemorrhage. The course and outcome of the disease in those patients not treated, or treated with immunosuppression alone, was better than that described in early reports of this disease, while those patients with plasma exchange and immunosuppression fared even better. An adequately stratified controlled trial of immunosuppression and plasma exchange versus immunosuppression alone is in order.     

Does radical nephrectomy with immunochemotherapy have any superiority over embolization alone in metastatic renal cell carcinoma? A preliminary report

INTRODUCTION: We evaluated the results and effects of radical nephrectomy followed by immunochemotherapy and embolization alone on the survival of patients with metastatic renal cell carcinoma. PATIENTS AND METHODS: The study included 20 patients with histologically confirmed renal cell carcinoma. Ten patients were in the combined therapy group and the other 10 patients who were unable to undergo nephrectomy because of poor performance status or unresectable tumor were in the embolization group. Radical nephrectomy was performed on patients with good performance status (WHO criteria 0-1). Immunochemotherapy (interferon alpha 2a and 5-fluorouracil) was started within 1 month after surgery. A dose of 9 x 10(6) U/day interferon alpha 2a was subcutaneously administered 3 times a week. A dose of 750 mg/m2 5-fluorouracil was administered intravenously during 4 h in the first 5 days of treatment. 5-Fluorouracil therapy was converted to weekly intervals after the first 12 days. Combined therapy was continued for 3 months. Ethanol was used for transarterial embolization. The main renal arteries and parasitic arteries of the tumor were embolized. RESULTS: There were no significant differences in age distribution, sex, affected side, tumor size and T stage between the groups. After completion of the combined therapy, 6 patients showed progression at the first control. Only 1 patient (10 %) had stable disease throughout the 10 months after combined therapy. One patient died of myocardial infarction on the 4th day in the embolization group. While progressive disease within the first 3 months was detected in 6 patients, the other 3 patients (30%) had stable disease for 14, 17 and 55 months, respectively. There was no complete response in any group and no patient was alive (died of renal cell carcinoma) at the time of the analysis of the study data. Whereas the median survival time was 11 months (1-80) (mean +/- SE: 22.2 +/- 9.1) in the combined group, this time was a median of 1 month (1-74) (mean +/- SE: 17.5 +/- 8.6) in the embolization group. There was no statistically significant difference in survival time between the groups (p > 0.05). CONCLUSION: In this preliminary report, the clinical findings in embolization-group patients were definitively worse than the nephrectomy plus immunochemotherapy-group patients. In spite of these differences, combination therapy using radical nephrectomy and immunochemotherapy could not show superiority to embolization alone, especially in terms of survival time.     

The prevalence of hyperlipidemia in renal transplant recipients. Associations with immunosuppressive and antihypertensive therapy

To determine the extent of persisting hyperlipidemia in renal transplant recipients receiving modern maintenance immunosuppressive and antihypertensive therapy we compared plasma levels of total and high-density lipoprotein and triglyceride in 275 renal transplant recipients with stable graft function with age- and sex-matched groups from the local general population (n = 4055). Total cholesterol and triglyceride were higher in transplanted patients in all age groups, but the difference was much more striking in women. Plasma levels of HDL cholesterol were similar or slightly lower in transplanted patients. Association with parameters of graft function, immunosuppressive therapy, and antihypertensive therapy were studied within the transplanted population using multiple regression. Total cholesterol was significantly and independently associated with age, sex, diuretic therapy, and urinary protein. In 127/134 (95%) of patients the diuretic was a loop diuretic. None of the other classes of antihypertensive drug was independently associated with serum cholesterol. The only variables significantly associated with HDL cholesterol were sex and the plasma creatinine. Plasma triglyceride was significantly and independently associated with both diuretic therapy and beta-blocker therapy and with age, urinary protein excretion, and plasma albumin. Plasma cholesterol, HDL cholesterol, and triglyceride levels were almost identical in patients receiving triple therapy (cyclosporine 3-5 mg/kg; prednisolone 7-10 mg o.d.; azathioprine 1-1.5 mg/kg) to those in patients receiving conventional immunosuppression (prednisolone 7-10 mg o.d.; azathioprine 2-2.5 mg/kg). Thus these results do not support the existence of a persisting long-term effect of cyclosporine on plasma cholesterol and triglyceride at these doses of the drug. The more striking abnormality of plasma cholesterol and triglyceride in females is unexplained but might be connected with greater sensitivity to low doses of corticosteroids.     

Clinical outcomes of renal cholesterol crystal embolization.

Cholesterol crystal embolization is an increasingly recognized disease, presenting with a wide clinical spectrum, usually occurring in elderly men who undergo an angiographic procedure or vascular surgery. We report three patients who developed systemic cholesterol embolic disease and varying degrees of renal failure after angiographic interventions of the coronaries.     

Cholesterol embolization in a renal graft

Cholesterol embolization into native kidneys has a dim prognosis for renal function and frequently leads to irreversible renal failure. Although uncommon, cholesterol embolization may also occur in renal allografts, particularly if either the recipient or the donor has prominent atherosclerosis. We report here on a case of a 65-yr-old man with cholesterol emboli in the renal allograft and delayed graft function. The recipient's arteria iliaca externa was a potential source because of heavy atherosclerosis. The patient was dialysis-dependent for two wk after transplantation. However, renal function improved, no cholesterol emboli were found in a second biopsy of the graft and serum creatinine is 260 micromol/L six months after the transplantation. In the case of primary renal non-function or dysfunction, cholesterol embolization must be considered in the differential diagnosis. If renal cholesterol embolization originates from the recipient, allograft survival is usually good. In contrast, if cholesterol embolization is of donor origin, graft dysfunction and subsequent graft loss are common. The reason for this difference may be the more extensive embolization developing in an atherosclerotic cadaver donor occurring during the organ procurement or the severe trauma leading to death.     

Successful Renal Transplantation in Factor H Autoantibody Associated HUS with CFHR1 and 3 Deficiency and CFH Variant G2850T

Factor H (CFH) autoantibodies are associated with atypical hemolytic uremic syndrome (aHUS). Peritransplantation plasma exchange therapy and intensification of immunosuppression, with adjuvant use of anti-CD20 monoclonal antibodies has recently been advocated for cases of CFH-autoantibody associated aHUS. In this report, we describe successful deceased donor renal transplantation in a case of CFH-autoantibody associated aHUS with combined CFHR1 and 3 deficiency in addition to the CFH sequence variant, (cG2850T, pGln950His). CFH-autoantibodies were detected 2 weeks prior to transplantation. Disease recurrence was not observed using basiliximab, an IL2-receptor antagonist and high-dose corticosteroids with mycophenolate mofetil. Adjuvant therapies such as Rituximab nor intensification of plasma therapy were employed. Consequently, careful consideration needs to be given to the use of additional immunosuppression in certain cases of CFH-autoantibody associated aHUS. Serial measurement of CFH-autoantibodies is required in the immediate pre- and posttransplantation period to further clarify their role as a factor in the recurrence of aHUS posttransplantation. Furthermore, delineation of the functional significance of CFH-autoantibodies is warranted in individual cases.     

Incidence and features of dual anti-GBM-positive and ANCA-positive patients

Aims: Goodpasture's syndrome, glomerulonephritis and pulmonary haemorrhage, may be due to a variety of causes. Rarely, patients with Goodpasture's syndrome present with both anti‐glomerular basement membrane (GBM) and antineutrophil cytoplasmic antibody (ANCA). The aim of this report was to determine the incidence, clinical features, management and outcomes of patients presenting with concurrent ANCA and anti‐GBM disease in Auckland. Methods: Potential patients were identified by an electronic search of serology for ANCA and anti‐GBM antibody, diagnostic renal biopsy, or in‐hospital admissions using ICD9 and ICD10 codes between 1998 and 2008. A retrospective case‐note review of all potential cases was performed. Results: Six cases were identified: two women and four men. The incidence was estimated at 0.47 cases per million people per year. The mean age of presentation was 59 years (range 25–85 years). One patient was a smoker and two patients were ex‐smokers. All subjects were anaemic, had haemoptysis and an abnormal chest X‐ray at presentation. The mean creatinine at presentation was 225 µmol/L (range 126–406 µmol/L); all patients had haematuria and proteinuria. All patients received corticosteroids and cyclophosphamide. Two patients were not plasma exchanged and died. Four patients received plasma exchange and are alive. One patient had a clinical relapse 6 years after their initial presentation and is on renal replacement therapy. Conclusion: Concurrent ANCA and anti‐GBM disease is rare. The mortality rate is high. Aggressive immunosuppression with steroids, cyclophosphamide and plasma exchange can induce remission and preserve renal function. Long‐term monitoring for relapses should occur.     

Profound persistent eosinophilia in a patient with spontaneous renal atheroembolic disease.

We describe an elderly women who died of renal failure secondary to spontaneous renal atheroembolic disease. The sole clinical clue to this diagnosis was a profound eosinophilia up to 19,100/mm3 and a relative eosinophil count of 80%. Renal atheroembolic disease should be a prominent consideration in any patient with both renal insufficiency and peripheral eosinophilia.     

Plasma exchange in patients with rapidly progressive idiopathic IgA nephropathy: a report of two cases and review of literature

Primary IgA nephropathy is generally considered an indolent disease, but progression to chronic renal failure is not uncommon, and a rapidly progressive course is observed in some cases, especially when extensive fibrocellular crescents are present. The therapeutic benefit of immunosuppression and plasma exchange remains controversial. We described two patients with primary IgA nephropathy and rapidly progressive renal failure. Both patients showed extensive glomerulosclerosis and crescent formation in their renal biopsies. Corticosteroid and immunosuppressive therapy failed to control the progression of the disease, and plasma exchanges were performed. In both cases, the serum creatinine and creatinine clearance initially improved with plasma exchange and the rapid progression of renal failure was apparently halted. In one patient, the serum creatinine rose when treatment was discontinued and fell again when plasma exchange was recommenced. Nevertheless, the long-term benefit of plasma exchange in crescentic IgA nephropathy was unsatisfactory as the renal function continued to deteriorate in the following 12 months despite an initial stabilization.     

Corticosteroid treatment of kidney disease in a patient with familial lecithin-cholesterol acyltransferase deficiency

Familial lecithin-cholesterol acyltransferase (LCAT) deficiency (FLD) is a rare genetic disorder of lipid metabolism, characterised by low plasma HDL cholesterol, proteinuria, haemolytic anaemia and corneal opacities. Usually renal disease progresses during the third decade of life to renal failure; however the pathogenesis of renal disease is not well understood. In this study we describe treatment of renal disease in two siblings with FLD. The proband WX at the age of 31 years presented proteinuria and ankle oedema during her third pregnancy. Diagnosis of FLD was based on a renal biopsy with characteristic serpiginous fibrillar deposits under electron microscopy, markedly decreased HDL cholesterol, esterified cholesterol levels and LCAT activity, confirmed by molecular analysis. After 3 years her proteinuria increased and she received an ACE inhibitor to which she responded well. During further increases of proteinuria she additionally received methylprednisolone and her proteinuria decreased. This long-term observation indicates the efficacy of corticosteroids and renin−angiotensin−aldosterone system blockers in the treatment of proteinuria in patients with FLD. The results suggest the role of inflammatory processes as well as dyslipidemia in the pathogenesis of glomerular disorders in LCAT-deficient patients.     

Inflammatory Demyelinating Neuropathies

The primary goal of therapy in patients with the Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is improved strength and functional ability. Improvement in pain, sensory loss, gait disorder, and autonomic instability are other goals of therapy. Patients with very mild symptoms that do not interfere with activities of daily living can be observed for deterioration without treatment. For GBS, standard care includes plasma exchange (PE) or human immune globulin (HIG), both of which have similar efficacy. Supportive care in the intensive care unit may be needed for those patients with severe bulbar or respiratory weakness. We treat most patients with PE, usually performing an exchange every other day for a total of five exchanges. We use HIG in children, if there are antiglycolipid antibodies (eg, anti-GM1 or anti-GQ1b) or if there is a contraindication to PE, such as hemodynamic instability; severe renal, hepatic, or cardiac disease; or poor venous access. For CIDP, there are no guidelines concerning the initial choice of therapy. Corticosteroids, HIG, and PE have all been shown to be effective in prospective, randomized controlled trials, and comparison trials have shown equal efficacy among these three immunomodulating therapies. The choice of therapy depends on several factors including disease severity, concomitant illnesses, side-effect profile, potential drug interactions, venous access, age-related risks, and cost of treatment. In patients with moderate to severe symptoms, treatment with corticosteroids or HIG should be used. We usually use high-dose, intermittent methylprednisolone as the initial drug of choice. We believe intermittent corticosteroids are better than HIG because of their good safety profile, low cost, ease of administration (can be given intravenously or by mouth), and proven efficacy. If there is a major contraindication to corticosteroids, then HIG is offered. PE is less well tolerated and is primarily used as a third choice and only for a few weeks to months to induce initial improvement. Once symptoms are improving, the dose of corticosteroids or HIG should be tapered with the goal of eventual discontinuation depending on patient response. Patients who do not respond to initial therapy, experience adverse effects from the initial immunomodulating agent, or require chronic treatment can be treated with another first-line agent or one of several second-line agents.     

The evaluation of corticosteroid therapy in conjunction with plasma exchange in the treatment of renal cholesterol embolic disease. A report of 5 cases

In this report, we describe 5 patients with cholesterol atheroembolic renal failure. In 3 of the 5 patients, combined therapy with corticosteroids and plasma exchange was performed. These 3 patients survived, with 2 showing an improvement in renal function. The 2 remaining patients died of multifactorial causes. The literature on therapy for cholesterol atheroembolic renal failure is reviewed and the efficacy of combined therapy by use of corticosteroids and plasma exchange is evaluated.     

Cholesterol atheroembolic disease in kidney allografts--case report and review of the literature

Cholesterol atheroembolic renal disease is a rare cause of renal allograft dysfunction. Two recipients of cadaveric kidney transplantats from the same donor are discussed with presumed graft failure due to cholesterol emboli of donor origin. A review of the literature summarizes the reported cases in renal transplant recipients. While cholesterol embolization of presumed donor origin seems to have a poor renal outcome, cholesterol emboli originating in the recipient have a more favorable prognosis. As donors and recipients of increasing age or prominent atherosclerosis are accepted for transplantation, cholesterol atheroembolic renal disease may become more prevalent and should be considered in patients with renal allograft dysfunction.     

Eosinophilia in the diagnosis of atheroembolic renal disease

We report 6 patients with eosinophilia and atheroembolic renal disease. Histologic examination of biopsy of kidney or skin revealed extensive atheroembolic in the vasculature. However, no evidence of vascular or tubulointerstitial inflammation was observed. Eosinophil count ranged from 540 to 2,000 cells/mm3. Upon review of the literature, 80% (29 out of 36) of patients with adequately reported total and differential leukocyte counts had eosinophilia in association with atheroembolic disease. In contrast, review of the clinical records of 40 consecutive patients with acute renal failure seen during an 18-month period uncovered only 1 case of eosinophilia (2.5%). This latter patient was established as having acute interstitial nephritis. Thus eosinophilia appears to be a helpful diagnostic clue to the presence of atheroembolic renal disease.     

Cholesterol atheroembolic renal failure after arteriography. Report of a case diagnosed by renal biopsy

A case of atheroembolic renal failure diagnosed by renal biopsy was presented. A 69-year-old man was referred because of progressive renal failure two months after major angiography for occlusive arterial disease of lower limbs. The physical examination on admission revealed an uncontrollable hypertension. The laboratory findings showed elevated serum creatinine (7.5 mg/dl) and eosinophilia (1022/mm3) with normal urinalysis findings. Renal biopsy disclosed a occlusive lesion of the arcuate artery which contained cholesterol clefts and foam cells, and showed ischemic renal parenchymal changes. These findings were compatible with cholesterol atheroembolic renal disease. In spite of the aggressive medical treatment, renal function had deteriorated progressively and the patient has been on regular hemodialysis. Atheroembolic renal failure after arteriography have been reported recently, but the case diagnosed by renal biopsy has been rare. Since there is no therapeutic way to reverse this type of renal failure, strict selection of patients for the angiographic examination and use of flexible catheter might be mandatory. Subacute course of renal failure after angiography and eosinophilia seem to be the important diagnostic clues for this disorder.