scholarly journals Antineutrophil Cytoplasmic Autoantibody—Associated Glomerulonephritis in Children

2001 ◽  
Vol 12 (7) ◽  
pp. 1493-1500 ◽  
Author(s):  
MOTOSHI HATTORI ◽  
HIDEAKI KURAYAMA ◽  
YASUSHI KOITABASHI

Abstract. A retrospective investigation was conducted by members of the Japanese Society for Pediatric Nephrology from 1990 to 1997 to define the clinical features and outcome of antineutrophil cytoplasmic autoantibody (ANCA)-associated glomerulonephritis in children. Thirty-four ANCA-seropositive Japanese pediatric patients with biopsy-proven pauci-immune necrotizing crescentic glomerulonephritis were identified. Of these, 3 cases associated with Wegener's granulomatosis were excluded because of the small sample size. Among the 31 patients studied, 10 had a diagnosis of necrotizing crescentic glomerulonephritis alone and 21 had microscopic polyangiitis. Females predominated (87%), and the median age at onset was 12 yr. Twenty-six patients received treatment with cyclophosphamide and corticosteroids, and five patients received treatment with corticosteroids alone; 84% of patients achieved remission, and 39% of responders relapsed in a median of 24 mo. ANCA titers correlated with response to treatment and disease activity, with some exceptions. Patients were followed for a median of 42 mo (range, 3 to 96 mo). Nine of 31 patients (29.0%) progressed to end-stage renal disease, 6 (19.4%) had reduced renal function, and 15 (48.4%) had normal renal function at the last observation. One patient (3.2%) died from cytomegalovirus infection 3 mo after initiation of therapy. Life-table analysis showed 75% renal survival at 39 mo. Patients who subsequently developed end-stage renal disease (n= 9) had significantly higher average peak serum creatinine levels and more chronic pathologic lesions at diagnosis compared with patients with favorable renal outcome (n= 15). In conclusion, our clinical experience suggests that the clinical disease spectrum of ANCA-associated glomerulonephritis is similar in pediatric and adult patients, but there is a female predominance in children.

Author(s):  
Patrick Nachman ◽  
Richard J. Glassock

The term crescentic glomerulonephritis (CrGN) refers to a diverse collection of disorders of widely different etiology and pathogenesis having in common the development of extensive proliferation of cells within Bowman's space (Couser, 1988; Glassock et al., 1995; Nachman et al., 1998; Pusey and Rees, 1998; Morgan et al., 2006; Lionaki, et al., 2007). The resulting accumulation of cells gives rise to a ‘crescent’ enveloping the glomerular tuft itself. Polymerization of fibrinogen in Bowman's space due to passage of fibrinogen through gaps in the capillary wall, the elaboration of procoagulant factors by infiltrating monocytes and impaired fibrinolysis all contribute to the pathogenesis of the crescent (Couser, 1988, Glassock et al., 1995). Usually 〉50% of glomeruli are involved with crescentic lesions. Such patients also frequently manifest rapid and progressive deterioration of renal function leading to the clinical syndrome of rapidly progressive glomerulonephritis. Early and aggressive treatment can often delay or prevent the development of end-stage renal disease (ESRD). See Table 10.1 for an etiologic and pathogenetic classification of CrGN.


1985 ◽  
Vol 5 (1) ◽  
pp. 33-35 ◽  
Author(s):  
Karel Matousovic ◽  
Josef Moravek Stefan ◽  
Vitko Vladimir Prat ◽  
Milena Horcickova

We investigated the pharmacokinetics of non-metabolized cefotaxime in 10 patients undergoing CAPD. The elimination half-life after IV administration of I g cefotaxime was 3.1 ± 1.3 hr, i.e. two to three times longer than in individuals with normal renal function but similar to patients with severe renal insufficiency. An average of 2.18% of the dose was recovered in the effluent. The halflife of I g cefotaxime administered in the dialysis solutions was 1.4 ± 0.8 hr. This difference between the half-lives after intraperitoneal and intravenous administration indicates a faster transport through the peritoneal membrane. Intraperitoneally administered cefotaxime -250 mg four times daily, was effective in the treatment of peritonitis in three CAPD patients. Since its introduction in 1976, CAPD has become an effective therapy for end-stage renal disease. The most serious complication is peritonitis and effective treatment is essential. Cefotaxime, a new broad-spectrurn cephalosporin, is active against most gramnegative and gram-positive organisms. It possesses no nephrotoxicity and may be useful in the treatment of peritonitis and other infections in patients on CAPD. This study was done to evaluate the pharmacokinetics of cefotaxime administered intravenously and intraperitoneally during CAPD.


Author(s):  
Lorraine Harper ◽  
David Jayne

The goals of treatment in renal vasculitis are to stop vasculitic activity and recover renal function. Subsequent strategies are required to prevent vasculitis returning and to address longer-term co-morbidities caused by tissue damage, drug toxicity, and increased cardiovascular and malignancy risk.Cyclophosphamide and high-dose glucocorticoids remain the standard induction therapy with alternative immunosuppressives, such as azathioprine, to prevent relapse. Plasma exchange improves renal recovery in severe presentations. Refractory disease resulting from a failure of induction or remission maintenance therapy requires alternative agents and rituximab has been particularly effective. Replacement of cyclophosphamide by rituximab for remission induction is supported by recent evidence. Methotrexate is effective in non-renal vasculitis but difficult to use in patients with renal impairment. Mycophenolate mofetil seems to be effective but there is less long-term evidence.Drug toxicity contributes to co-morbidity and mortality and has led to newer regimens with reduced cyclophosphamide exposure. Glucocorticoid toxicity remains a major problem with controversy over the rapidity with which glucocorticoids can be reduced or withdrawn.Disease relapse occurs in about 50% of patients. Early detection is less likely to lead to an adverse affect on outcomes. Rates of cardiovascular disease and malignancy are higher than in control populations but strategies to reduce their risk, apart from cyclophosphamide-sparing regimens, have not been developed. Thromboembolic events occur in 10% and may be linked to the recently identified autoantibodies to plasminogen and tissue plasminogen activator.Renal impairment at diagnosis is a strong predictor of patient survival and renal outcome. Other predictors include patient age, antineutrophil cytoplasmic antibody subtype, disease extent and response to therapy. Chronic kidney disease can stabilize for many years but the risks of end-stage renal disease are increased by acute kidney injury at presentation or renal relapse. Renal transplantation is successful with similar outcomes to other causes of end-stage renal disease.


2009 ◽  
Vol 75 (9) ◽  
pp. 790-793 ◽  
Author(s):  
Christopher R. Schneider ◽  
William Cobb ◽  
Shivani Patel ◽  
David Cull ◽  
Cass Anna ◽  
...  

Little research has been performed in regards to the morbidity and outcomes associated with elective general surgery performed on patients with end stage renal disease (ESRD). With minimal data about the severity of disease in these patients, we sought to quantify the differences in the ESRD patient undergoing elective surgical procedures compared with matched controls. A review of all ESRD patients undergoing elective surgical procedures at a University Medical Center between 2001 and 2005 was performed. Outcomes included length of hospital stay, 1 year morbidity, and mortality. These patients were then compared with a control group with normal renal function matched 2:1. Fifty-two consecutive ESRD patients undergoing elective general surgery procedures were compared with 104 matched controls. The ESRD group experienced more complications (25 vs 16, P = 0.05) and had a larger number of overall complications compared with the controls (33 vs 19, P < 0.05). Length of stay was significantly longer in the ESRD group as well (8 vs 2.65 days, P < 0.0001). Incidence of death (4%) in the ESRD group was increased as well. Patients with ESRD require longer hospital stays and have an increased overall incidence and frequency of complications than patients with normal renal function undergoing elective general surgery procedures. The significantly increased morbidity should be considered when evaluating expected outcomes.


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