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Ambiguous findings during external examination of a deceased in combination with dubious autopsy findings can raise doubts concerning the manner and cause of death. We report the case of a 35-year-old female deceased who had suffered from a borderline personality and depressive disorder with suicidal ideation. At the death scene, the body showed massive facial swelling accompanied by complete reddening of the skin of the face, with patchy skin abrasions on the forehead and neck, and purple bruise-like discolorations distributed symmetrically over both shoulders, elbows, hands, hips, knees, lower legs, and feet, raising the suspicion of underlying massive external blunt force injury. Police investigators strongly suspected sexual homicide. At autopsy, dissection in layers revealed massive subcutaneous hemorrhages as the cause of the reddish skin discolorations. Toxicological analyses showed fatal levels of lamotrigine with additional proof of zopiclone, zolpidem, diphenhydramine, O-desmethylvenlafaxine, pregabalin, tramadol, and modafinil in venous blood. Histologically, both the macroscopically impressive purple skin changes with underlying bleeding into the subcutaneous tissue and the skin abrasions were due to leukocytoclastic vasculitis, a form of acute hypersensitivity vasculitis that was a reaction to the multiple therapeutic drugs that the woman had taken shortly before death. The manner of death was classified as suicide, and sexual homicide was ruled out. [ABSTRACT FROM AUTHOR]

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Immunoglobulins protect against disease to a considerable extent by activating complement and stimulatory immunoglobulin crystallizable fragment receptors (Ig FcRs), and aggregating microbial pathogens. Yet IgG1, the predominant murine serum Ig isotype, cannot activate complement by the classical pathway, binds more avidly to an inhibitory than to stimulatory FcRs, and has limited ability to aggregate pathogens. In these regards, it resembles human IgG4 (ref. 4). We hypothesized that limited ability to activate effector mechanisms might protect against immune complex immunopathology. Here we show that IgG1-deficient (γ1−) mice, immunized with a potent antigen, develop lethal renal disease soon after they begin to produce antigen-specific antibody, whereas similarly immunized wild-type mice remain healthy. Surprisingly, renal disease in this model is complement and FcR independent and results from immune complex precipitation in glomerular capillaries, as in some cryoglobulinaemic humans. IgG3, which self-associates to form large immune complexes, accounts for more than 97% of the mouse Ig in this cryoglobulin; furthermore, glomerular disease develops when mice are injected with IgG3 anti-trinitrophenyl (TNP) monoclonal antibody followed by a TNP-labelled protein. Renal disease is prevented in both active and passive immunization models by antigen-specific IgG1; other isotypes are less potent at preventing disease. These observations demonstrate the adaptive significance of Ig isotypes that poorly activate effector mechanisms, reveal an immune-complex-dependent, complement- and FcR-independent nephrotoxic mechanism, and suggest that isotypes that poorly activate effector mechanisms may be useful for inhibiting immune complex immunopathology. [ABSTRACT FROM AUTHOR]

'Membranoproliferative' describes glomerular injury characterized by capillary wall thickening and mesangial expansion owing to increased matrix deposition and hypercellularity. The presence of immune deposits is indicative of membranoproliferative glomerulonephritis (MPGN). Historically, MPGN was further classified into three types according to the appearance and site of the electron-dense deposits seen by electron microscopy, but it is now recognized that many cases show only deposition of the complement component C3, owing to abnormal control of the alternative pathway of complement activation--these cases are now classified as C3 glomerulopathies. Not all cases of C3 glomerulopathy, however, show an MPGN pattern. C3 glomerulopathies include dense deposit disease, which shows dense osmiophilic deposits, and C3 glomerulonephritis, which shows isolated deposits, in many cases, the genetic mutations or autoantibodies responsible for C3 deposition have been identified. Some patients in whom complement control is abnormal will accumulate small amounts of immunoglobulin in their glomeruli and so, in everyday practice, the morphological diagnosis of 'glomerulonephritis with dominant C3' is useful for identifying patients who require investigation of the complement pathway. The recognition that many cases of MPGN are C3 glomerulopathies and that the underlying cause can often be identified in immunoglobulin-associated cases means that the diagnosis of idiopathic MPGN is now very uncommon. [ABSTRACT FROM AUTHOR]

Individual genome-wide association (GWA) studies and their meta-analyses represent two approaches for identifying genetic loci associated with complex diseases/traits. Inconsistent findings and non-replicability between individual GWA studies and meta-analyses are commonly observed, hence posing the critical question as to how to interpret their respective results properly. In this study, we performed a series of simulation studies to investigate and compare the statistical properties of the two approaches. Our results show that (1) as expected, meta-analysis of larger sample size is more powerful than individual GWA studies under the ideal setting of population homogeneity among individual studies; (2) under the realistic setting of heterogeneity among individual studies, detection of heterogeneity is usually difficult and meta-analysis (even with the random-effects model) may introduce elevated false positive and/or negative rates; (3) despite relatively small sample size, well-designed individual GWA study has the capacity to identify novel loci for complex traits; (4) replicability between meta-analysis and independent individual studies or between independent meta-analyses is limited, and thus inconsistent findings are not unexpected. [ABSTRACT FROM AUTHOR]

Infections by the viruses responsible for hepatitis B, C and D are accompanied by a number of immunopathological manifestations. A link between infection and autoimmunity is particularly well documented for the hepatitis C virus. Immunopathological manifestations range from production of autoantibodies to overt autoimmune disease, including thyroiditis and autoimmune hepatitis, and to immune-complex-mediated disorders, including cryoglobulinaemia, glomerulonephritis and vasculitis. Several of these manifestations improve with successful antiviral treatment, directly incriminating the virus in their pathogenesis. Mechanisms considered responsible for hepatitis virus-related immunopathology, including molecular mimicry, impairment of regulatory T cells and activation of B lymphocytes, will be examined in this review. [ABSTRACT FROM AUTHOR]

Until recently, membranoproliferative glomerulonephritis (MPGN) was clinically classified as either primary, idiopathic MPGN or as secondary MPGN when an underlying aetiology was identifiable. Primary MPGN was further classified into three types--type I, type II, and type III--based principally on the ultrastructural appearance and location of electron-dense deposits. Both the clinical and histopathologic schemes presented problems, however, as neither was based on disease pathogenesis. An improved understanding of the role of complement in the pathogenesis of MPGN has led to a proposed reclassification into immunoglobulin-mediated disease (driven by the classical complement pathway) and non-immunoglobulin-mediated disease (driven by the alternative complement pathway). This reclassification has led to improved diagnostic clinical algorithms and the emergence of a new grouping of diseases known as the C3 glomerulopathies, best represented by dense deposit disease and C3 glomerulonephritis. In this Review, we re-examine the previous and current classification schemes of MPGN, focusing on the role of complement. We survey current data about the pathogenesis of the C3 glomerulopathies, including familial studies and patient cohorts from the USA and Europe. In addition, we discuss the diagnosis, treatment, and prognosis of the C3 glomerulopathies. [ABSTRACT FROM AUTHOR]

Our previous study suggested involvement of alternative pathway activation of complement in ANCA-positive pauci-immune crescentic glomerulonephritis (CrGN). This study was to investigate the evidence of complement activation in renal biopsy specimens of patients with ANCA-negative pauci-immune CrGN. Renal biopsy specimens from 12 patients with ANCA-negative pauci-immune CrGN were used to detect the staining of membrane attack complex (MAC), C3d, C4d, mannose-binding lectin (MBL), factor B by immunohistochemistry, and/or immunofluorescence. Renal tissue from eight patients with minimal change disease (MCD) and renal tissue obtained from a normal kidney and the normal parts of a nephrectomized kidney due to renal carcinoma was used as disease and normal controls, respectively. MAC and C3d could be detected in the active renal lesions of cellular crescents in each of the 12 ANCA-negative CrGN patients but not or scarcely detected in patients with MCD and in normal renal tissue. The deposition of other complement components in the 12 patients was heterogeneous. Although none of them had C1q staining, eight of the 12 were C4d positive and six out of the eight were MBL positive. The remaining four only had evidence of the alternative pathway activation by positive staining of C3d, factor B, and MAC. The staining intensity of C4d and MBL was much higher in dialysis-dependent patients than that in dialysis-independent patients. Complement activation was involved in renal damage of human ANCA-negative pauci-immune CrGN but with heterogeneous activation pathways. Positive staining of C4d and MBL might be associated with poor renal outcome. [ABSTRACT FROM AUTHOR]

Abstract Introduction  Association between inherited deficiencies of the complement components and immune complex disease indicates the importance of the complement system in the handling of circulating immune complexes. High levels of circulating immune complexes are seen in pulmonary tuberculosis. This study is, therefore, aimed to look at the concentration of circulating immune complexes, the status of complement-mediated immune complex handling, and the extent of complement activation in untreated pulmonary tuberculosis compared to treated pulmonary tuberculosis patients and healthy controls. Results  High immune complex levels, decreased complement-mediated solubilization, and increased activation of the complement system were observed in untreated tuberculosis patients. Conclusions  The results obtained from the present study suggest that complement mediated solubilization is less in patients with tuberculosis, and this defective solubilization is likely to take part in a vicious cycle involving immune complex deposition and complement activation and, thus, may lead to disease progression depending on the nature of the defect. [ABSTRACT FROM AUTHOR]

A 6-year-old previously healthy Japanese girl was found to have dipstick 2+ proteinuria and a goiter based on the results of a routine school medical examination. Her serum free-thyroxine level was 4.98 ng/dL (normal range 0.95–1.74 ng/dL), thyroid-stimulating hormone (TSH) was less than 0.003 μU/mL (0.34–3.88 μU/mL), anti-microsomal (anti-thyroid-peroxidase) antibody was 1600 T (up to 100), anti-thyroglobulin antibody was 400 T (up to 100), and TSH-receptor antibody was 84% (up to ±10%). These results are consistent with a diagnosis of Graves’ disease. Electron microscopy examination of a renal biopsy specimen revealed electron-dense deposits located in the subepithelial spaces, and immunofluorescence microscopy examination demonstrated bright granular stainings of immunoglobulin G along the glomerular capillary walls. These findings are characteristic of membranous nephropathy. To investigate the relationship between the membranous nephropathy and Graves’ disease, we carried out a second immunofluorescence study, which revealed that the immunoglobulin G granular deposits corresponded to glomerular granular staining of thyroid-peroxidase, whereas staining for thyroglobulin was absent. It was therefore assumed that the deposition of immune complexes mediated by thyroid-peroxidase had caused the membranous nephropathy in this patient. This is the first report of membranous nephropathy associated with Graves’ disease in which deposits of thyroid-peroxidase, rather than thyroglobulin, have been confirmed in the kidney. [ABSTRACT FROM AUTHOR]

In infected individuals, hepatitis C virus (HCV) exists in various forms of circulating particles which role in virus persistence and in HCV resistance to IFN therapy is still debated. Here, the proportion of HCV bound to immunoglobulin was determined in plasma of 107 chronically infected patients harbouring different HCV genotypes and, for comparison, of six patients with acute HCV infection. The results showed that, in spite of wide individual variability, chronically HCV-infected patients exhibited an extremely high proportion of immune complexed (IC) virus regardless of plasma HCV load and infecting genotype. Moreover, no significant association was found between baseline proportion of IC HCV and response to IFN treatment. Plasma samples collected within 2 weeks of treatment from 20 patients revealed a significant decline of mean IC HCV values relative to baseline that clearly paralleled the decay of total HCV load. In acutely infected patients, circulating HCV was not IC or IC at very low levels only in patients developing chronic HCV infection. Collectively, these findings strengthen the possibility that IC virus could play a critical role in the pathogenesis of HCV infection. [ABSTRACT FROM AUTHOR]

We describe the case of a 78-year-old woman with peripheral neuropathy, neurogenic muscular atrophy, skin ulcers, arthritis and immune complex glomerulonephritis. Detection of mixed cryoglobulins in combination with typical clinical symptoms, and the exclusion of hepatitis C and other underlying diseases, led to the rare diagnosis of essential cryoglobulinaemic vasculitis type III. Because initial interventions with prednisolone, plasmapheresis and cyclophosphamide pulse therapy failed to induce remission, therapy with rituximab, a chimeric monoclonal antibody that reacts specifically with the CD20 antigen, was initiated. Rituximab was administered intravenously at a dose of 375 mg/m2 body surface. After five applications, the patient showed remission of clinical symptoms and complete normalisation of laboratory values. These results suggest that rituximab is an alternative therapeutical approach with strikingly good clinical outcome in patients with cryoglobulinaemic vasculitis type III. [ABSTRACT FROM AUTHOR]

Glomerulonephritis secondary to infective endocarditis (IE) is an uncommon diagnosis and is usually associated with cardiac valvular infection by blood-culture-positive bacteria. We report a case of necrotizing glomerulonephritis associated with culture-positive endocarditis caused by Enterococcus faecalis. The patient presented with renal abnormalities and was further investigated by renal biopsy. He had immune complex-mediated necrotizing and crescentic glomerulonephritis with mesengial and capillary deposition of immunoglobulin M (Ig M), Ig G, and complement 3 (C3). He was treated with antibiotics, including ampicillin and gentamicin. In addition, steroid and cyclophosphamide were administered. The patient died of renal failure 48 days after hospital admission. In conclusion, glomerulonephritis caused by Enterococcus faecalis endocarditis is an immune-complex-mediated disease characterized by necrotizing and crescentic glomerular lesions that can be fatal despite aggressive antimicrobial and immunosuppressive therapy. [ABSTRACT FROM AUTHOR]

Postinfectious glomerulonephritis (PIGN) most commonly follows streptococcal infection. Antineutrophil cytoplasmic antibodies (ANCA) are characteristically negative in PIGN. We report on five cases who had positive atypical pANCA at presentation. The outcome was typical of other cases of PIGN with complete resolution of the glomerulonephritis in all five patients. Atypical pANCA occurs in a number of inflammatory conditions and antigenic targets may include proteins other than myeloperoxidase or proteinase 3. The presence of atypical p-ANCA does not indicate a poor prognosis. [ABSTRACT FROM AUTHOR]

Abstract  The objective of the study was to estimate association between maternal glomerulonephritis during pregnancy and structural birth defects, i.e. congenital abnormalities. The prevalence of maternal glomerulonephritis during the first trimester of pregnancy in cases with different congenital abnormalities was compared to that of matched controls without congenital abnormalities in the population-based Hungarian Case–Control Surveillance System of Congenital Abnormalities. Of 22,843 cases with congenital abnormalities, 309 (1.35%) had mothers with glomerulonephritis during pregnancy, compared to 479 (1.26%) of 38,151 controls (adjusted POR with 95% CI = 1.0, 0.9–1.2). Specified groups of congenital abnormalities were also assessed versus controls. Cases with isolated intestinal atresia/stenosis (adjusted POR with 95% CI: 6.8, 1.3–37.4) based on five cases were more likely to have mothers with prospectively and medically recorded glomerulonephritis. In conclusion a higher rate of congenital isolated intestinal atresia/stenosis may be associated with the maternal glomerulonephritis. However, this finding is considered only as signal and further studies are needed to confirm or reject this possible association. [ABSTRACT FROM AUTHOR]

We experienced two patients with IgG subclass deficiency who suffered from chronic glomerulonephritis (GN). Patient 1 was a 17-year-old girl with IgG subclass deficiency (combined deficiency of IgG2 and IgG4). Renal biopsy was performed when she was aged 16 years, and she was diagnosed with membranoproliferative GN. Patient 2 was a 16-year-old girl with IgG subclass deficiency (combined deficiency of IgG2, IgG3, and IgG4). Renal biopsy was performed when she was aged 15 years, and she was diagnosed with membranous nephropathy. We examined the glomerular deposition patterns of their IgG subclasses. Furthermore, we compared their clinical and laboratory findings with those of three patients with IgG subclass deficiency without GN. Patients with GN suffered infections more frequently than those without GN. The serum levels of IgG (especially IgG1) and IgM were higher in patients with GN than in those without GN. In patient 1 IgG1 and IgG3 were deposited in a mesangiocapillary pattern, but, in patient 2, only IgG1 was deposited in a capillary pattern. Thus, the different patterns of IgG subclass deficiency between the two patients may be responsible for their different glomerular pathologies. To the best of our knowledge, this is the first report of chronic GN in patients with IgG subclass deficiency. [ABSTRACT FROM AUTHOR]

IgA nephropathy is the most common form of primary glomerulonephritis. Variations in clinical manifestations indicate that a diagnosis of IgA nephropathy encompasses multiple disease subsets that cannot be distinguished on the basis of renal pathology or clinical variables alone. Familial forms of the disease have been reported throughout the world, but are probably under-recognized because associated urinary abnormalities are often intermittent in affected family members. IgA nephropathy has complex determination, with different genes probably causing disease in different patient subgroups. Of the many pathogenic mechanisms reported, defects in IgAl glycosylation that lead to formation of immune complexes have been consistently implicated. Here, we present the evidence for genetic contributions to the disease, review clinical patterns of familial disease, and summarize some of the most promising genetic studies conducted to date. Linkage-based approaches to the study of familial forms of the disease have identified significant or suggestive loci on chromosomes 6q22-23, 2q36, 4q26-3 1, 1 7q12-22 and 3p24-23, but no causal gene has yet been identified. Many interesting, but poorly replicated, genetic association studies have also been reported. We discuss recent developments in analytic tools that should enable genetic studies of sporadic forms of disease by the genome-wide association approach. [ABSTRACT FROM AUTHOR]

To investigate the association between HLA-DRB1* alleles and post-streptococcal acute glomerulonephritis (PSAGN) in Egyptian children, 32 unrelated patients with PSAGN and 380 healthy individuals from the same locality were typed for DRB1* alleles using the polymerase chain-reverse hybridization technique. Patients with PSAGN had significantly increased frequency of both DRB1* 03011 (46.9 vs. 19.2% in controls, P = 0.00025) and DRB1* 1105 (31.1 vs. 15.6% in controls, P = 0.0097) alleles. However, after correction of P values, only the difference for DRB1* 03011 allele remained significant ( Pc = 0.025). Their relative risks were significantly high (3.71, confidence interval [CI] = 1.8–7.8, and 3.57, CI = 1.4–8.9 respectively). No significant differences in the frequency of the two alleles were observed among patients with different grades of hypertension or proteinuria. In conclusion, DRB1* 03011, and possibly 1105, alleles confer susceptibility to PSAGN. However, the severity of the disease is not determined by these two alleles. [ABSTRACT FROM AUTHOR]

Background A 42-year-old man presenting with flank pain was found to have renal failure with severe hypocomplementemia and eosinophilia. Investigations Physical examination, laboratory testing, renal ultrasonography, and renal biopsies. Diagnosis Acute immune-complex-mediated tubulointerstitial nephritis. Management Immunosuppressive therapy with 1 mg/kg/day prednisone. [ABSTRACT FROM AUTHOR]

Renal disease concurrent with a Mycoplasma pneumoniae infection is uncommon. In this report we describe the clinical outcome of a 6-year-old patient who presented with a rapidly progressive glomerulonephritis that required dialysis. A kidney biopsy was performed, and the results revealed membranoprolipherative glomerulonephritis. The IgM serology was positive, and M. pneumoniae DNA was detected in a renal biopsy sample using a nested-PCR assay. The outcome was good. [ABSTRACT FROM AUTHOR]

Genome scans for osteoarthritis (OA) have yielded inconsistent results. The absence of replication of linkage might be due to the lack of power of individual studies. A meta-analysis of published data was performed to assess evidence for linkage of OA across genome scan studies. Three OA whole-genome scans including 893 families with 3,000 affected individuals were used for genome scan meta-analysis (GSMA). A total of five bins lie above 95% confidence level ( P=0.05) and one bin is above 99% confidence level ( P=0.01) in OA GSMA; bins 7.6 (7q34–7q36.3, P sumrnk =0.0035), 11.3 (11p12–11q13.4), 6.3 (6p21.1–6q15), 2.8 (2q31.1–2q34) and 15.3 (15q21.3–15q26.1). The highest summed bin was bins 7.6. In conclusion, the OA GSMA has shown that chromosome 7q34–7q36.3 have the highest summed rank and four additional loci with significant summed ranks across studies. [ABSTRACT FROM AUTHOR]

Although it has been reported that high-dose immunoglobulin has beneficial effects in chronic glomerulonephritis, it is not known whether it is effective in the treatment of idiopathic nephrotic syndrome. We have investigated the effects of intraperitoneal immunoglobulin in adriamycin-induced nephrotic syndrome. Adriamycin (2 mg kg−1 per dose) was given intravenously to sixteen Wistar albino rats (eight control and eight treatment rats) on day 1 and at week 3. At week 5 intraperitoneal immunoglobulin (1 g kg−1 per dose) was given to the treatment group on two consecutive days whereas the control group received intraperitoneal saline solution. In both treatment and control groups urinary protein excretion was significantly elevated after administration of adriamycin ( P=0.018). Urinary protein excretion, serum albumin, and triglyceride levels in the two groups were not significantly different after 5, 8, 12, and 16 weeks. Serum creatinine levels were higher and creatinine clearance was significantly lower in the control group in week 16 ( P=0.001 and P=0.049, respectively). Glomerular sclerosis index was significantly lower in the treatment group ( P=0.012). Although intraperitoneal high-dose immunoglobulin did not reverse biochemical results, it is encouraging that glomerular sclerosis index was significantly lower in the treatment group. [ABSTRACT FROM AUTHOR]

Acute proliferative glomerulonephritis is characterized by glomerular hypercellularity that can be caused by many different etiologies and pathogenetic mechanisms. A balance between cell birth by mitosis and cell death by apoptosis is crucial. In this study, apoptosis and the regenerative activity (Ki67/apoptosis index) were investigated in acute proliferative glomerulonephritis. Thirty-five children with biopsy-proven acute proliferative glomerulonephritis and five controls with MCD were studied retrospectively. According to the clinical outcome, patients were divided into 2 groups: group 1 ( n =21) were patients with normal renal functions at follow-up; group 2 ( n =8) were patients with end-stage renal failure or those who died. Immunohistochemical staining of proliferating cells (Ki67) was done. In situ end labeling of DNA was used to evaluate apoptosis. Glomerular cell apoptosis was 45% in the patients with acute proliferative glomerulonephritis and 3% in controls ( p <0.001). Apoptotic cells were identified in the tubulointerstitial compartment with higher and heavier immunostaining in patients than controls ( p =0.001). Tubular proliferative index (= tubular proliferation/tubular apoptosis ratio) was significantly higher in group 1 patients than in group 2 patients (2.03±2% versus 0.32±0.6%, p =0.002). Tubulointerstitial regenerative ratio (=tubular proliferation/interstitial proliferation ratio) was significantly higher in controls than in patients (3.4±1.9 versus 1.52±0.8, p =0.01). In addition, it was significantly increased in group 1 patients when compared with those in group 2 patients (1.89±0.8 versus 0.73±0.2, p =0.001). Since 17 patients presented with postinfectious proliferative glomerulonephritis, which is known to exhibit better course, we also evaluated those parameters in patients with postinfectious proliferative glomerulonephritis separately. We found statistically significant differences only in the tubulointerstitial regenerative ratio, which was higher in postinfectious cases when compared with those in other cases [1.60 interquartile range (IQR) 1.54 versus 1.22 IQR 1.26, respectively, p =0.003]. In conclusion, tubular proliferative index and tubulointerstitial regenerative ratio might be useful parameters for predicting final functional outcome in acute proliferative glomerulonephritis. Further studies, however, are still needed to clarify the importance of these histopathological parameters. [ABSTRACT FROM AUTHOR]

Since an active isoform of plasma hemopexin (Hx) has been proposed to be a potential effector molecule in minimal change disease (MCD), we tested plasma and urine samples from subjects with MCD in relapse ( n =18) or in remission ( n =23) (after treatment with prednisolone) for presence or activity of Hx. For comparison, plasma or urine from proteinuric subjects with focal and segmental glomerulosclerosis (FSGS, n =11), membranoproliferative glomerulonephritis (MPGN, n =9), IgA nephropathy ( n =5) or healthy control donors ( n =10), were incorporated into the study. Electrophoresis and Western blotting methods were used for evaluation of the Hx status, whereas protease activity of Hx was tested upon kidney tissue in vitro according to standard methods. The results show (1) a decreased mean titer of plasma Hx exclusively in MCD relapse subjects as compared with MCD in remission (0.21±0.14 mg/ml vs 0.44±0.06 mg/ml; p <0.01). Mean Hx titers in other proteinuric subjects ranged from 0.38±0.05 mg/ml to 0.40± 0.06 mg/ml, whereas, the mean titer of healthy controls was 0.59±0.03 mg Hx/ml; (2) an increased Hx activity (expressed in arbitrary units) exclusively in plasma from MCD relapse subjects (3.3±0.72 vs 1.16±0.56, MCD remission; p <0.01); (3) different Western blot patterns in MCD relapse vs remission plasma; (4) reduced stainability or virtual absence of the 80-kD Hx band in blots of urine from MCD relapse in contrast to urine samples from other proteinuric subjects with FSGS, MPGN, or IgA nephropathy. It is concluded that Hx in MCD relapse subjects may exist in an altered isoform, showing enhanced protease activity as compared with subjects in remission, subjects with other forms of primary glomerulopathy, or healthy control individuals. [ABSTRACT FROM AUTHOR]

A 79-year-old woman was admitted to our hospital because of leg edema due to a nephrotic syndrome. Urinary and serum immunoelectrophoresis showed positive for the lambda type of Bence Jones protein. A bone marrow aspiration test revealed mild plasmacytosis (6.4% of the total cells). These findings confirmed her diagnosis of monoclonal gammopathy of undetermined significance (MGUS). Her renal biopsy specimen revealed mild mesangial cell proliferation and an increase in the mesangial matrix. Immunofluorescence studies showed positive staining for IgG, IgA, C3, and kappa and lambda light chains in the capillary wall and mesangium area. Electron microscopy showed that the electron deposits in the thickened basement membrane were formed by randomly arranged 16- to 18-nm nonbranching fibrils. A Congo red stain for amyloid was negative. These findings corresponded with the diagnosis of fibrillary glomerulonephritis. Therefore, this case showed a rare combination of fibrillary glomerulonephritis and MGUS. [ABSTRACT FROM AUTHOR]

AimsThe aims of this study were to: Investigate retinal changes associated with type 2 membranoproliferative glomerulonephritis (type 2 MPGN).Assess whether there was a relationship between the severity of the kidney disease and ophthalmoscopically visible fundal changes.Find out if renal transplantation was linked with visual deterioration.Decide if patients with type 2 MPGN need to be referred for ophthalmological assessment.MethodsThe patients were identified from pathology department records as having renal biopsy proven type 2 MPGN from January 1981 to October 2003. Patients were invited to attend for ophthalmic assessment.ResultsA total of 26 biopsy proven cases of type 2 MPGN were identified. One patient lost vision from a possible occult choroidal neovascular membrane. The extent of ocular involvement did not consistently appear to be related to the severity of the renal involvement, although there was a relationship between the presence of ocular lesions and the duration of the disease. The longer the disease was present the more likely the patients were to have ophthalmoscopically visible fundal changes. Renal transplantation did not appear to have a detrimental effect on vision with the possible exception of one patient.ConclusionReferral of type 2 MPGN patients by the renal physician for an initial ophthalmological assessment may be beneficial so that symptoms of choroidal neovascularization can be explained and patients advised to seek urgent help if distortion of central vision occurs. Renal transplantation does not appear to be a risk factor for the development of choroidal neovascular membranes.Eye (2005) 19, 985–989. doi:10.1038/sj.eye.6701697; published online 17 September 2004 [ABSTRACT FROM AUTHOR]

Systemic lupus erythematosus, which predominantly affects young women, is frequently complicated by renal involvement. The presence of acute glomerulonephritis significantly adds to morbidity and mortality. Cyclophosphamide has become the mainstay of treatment in patients with proliferative forms of lupus nephritis. However, adverse events such as severe infections and infertility have spurred the search for novel treatment regimens and agents. Sequential therapy has significantly reduced adverse events. In several pilot studies, mycophenolate mofetil (MMF) has emerged as a promising therapeutic approach for both the induction and maintenance phase in patients with lupus nephritis, delivering equal efficacy and a better adverse effect profile; however, these studies had a limited power, and a large, multicentre and probably multinational clinical trial will be needed to discern the optimal therapeutic approach. On the basis of the currently available literature, sequential therapy with cyclophosphamide induction followed by azathioprine or MMF maintenance can be recommended for most patients. In selected populations, induction with MMF is a reasonable option to reduce adverse events. [ABSTRACT FROM AUTHOR]

On April 25, 2003, a 62-year-old Japanese man had been admitted to a hospital because of heavy proteinuria and elevated serum creatinine level, and purpura on the lower extremities. On May 15, 2003, he was referred to our hospital for evaluation and treatment. Serum immunoglobulin and complements were within normal ranges. Immune serology was negative for antinuclear antibody, antiglomerular basement membrane antibody, and antineutrophil cytoplasmic antibodies. Histological examination of a percutaneous renal biopsy specimen revealed that all of the glomeruli had severe crescent formation without deposits of immunoreactants. A diagnosis of antineutrophil cytoplasmic antibody-negative pauci-immune crescentic glomerulonephritis was made. The patient was treated with one cycle of steroid pulse therapy (1000 mg methylprednisolone daily, given on 3 consecutive days), and subsequently with prednisolone (60 mg/day). Despite this treatment, renal failure progressed rapidly and hemodialysis was started 1 month after the acute presentation. On May 30, 2003, he suddenly developed massive hematochezia. A technetium-targeted red-blood-cell scan suggested bleeding in the small intestine. On June 11, he presented with massive melena. A bleeding ulcer was found in the third part of the duodenum, and was treated successfully with endoscopy, using a heater probe. On June 19, he presented with massive hematochezia again. Mesenteric angiography revealed active bleeding from the iliac branch of the superior mesenteric artery. He was treated with continuous intraarterial vasopressin infusion by a catheter seated in the branch artery. The majority of patients with pauci-immune crescentic glomerulonephritis, one of the most common causes of rapidly progressive glomerulonephritis, have glomerular disease as part of a systemic vasculitis. Massive gastrointestinal bleeding, although rare, should be considered one of the serious complications in these patients. [ABSTRACT FROM AUTHOR]

We report on a 65-year-old Japanese woman with Takayasu arteritis who developed severe proteinuria, hypertension, and renal dysfunction. Renal angiography demonstrated moderate irregular narrowing of both renal arteries. Renal biopsy showed glomerulonephropathy mimicking membranoproliferative glomerulonephritis (MPGN) with glomerular capillary wall thickening (“double contour”) accompanied by mesangial cell proliferation and moderate increase of mesangial matrix without deposits of C3. Electron microscopy showed no subendothelial deposit and no circumferential mesangial interposition (CMI), and these findings are different from MPGN. In this report we present a case of Takayasu arteritis associated with glomerulonephropathy mimicking MPGN. [ABSTRACT FROM AUTHOR]

Background. Matrix metalloproteinase (MMP)-9, a member of the MMP family with specificity towards type IV collagen, is implicated in the turnover of the extracellular matrix in the kidney. To elucidate its physiological and pathophysiological significance, we examined the expression and localization of MMP-9 in the normal kidney and the changes in these features during the course of antiglomerular basement membrane (GBM) glomerulonephritis induced in WKY rats, along with the changes in these features of tissue inhibitor of metalloproteinase 1 (TIMP-1) and MMP-2. Methods. The expression of MMP-9, TIMP-1 and MMP-2 mRNA was quantified by ribonuclease protection assay, and the gelatinolytic activities of MMP-9 and MMP-2 were evaluated by gelatin zymography. The localization of MMP-9 was visualized by immunohistochemistry and immunofluorescence microscopy. Results. The ribonuclease protection assay indicated the almost exclusive expression of MMP-9 mRNA in the glomerulus of normal kidneys. Immunohistochemistry and double-label immunofluorescence microscopy showed that MMP-9 was localized in the mesangial cells. During the course of anti-GBM glomerulonephritis, the expression of MMP-9 mRNA in glomeruli increased on day 1, peaked on days 3 to 7, and then decreased on day 14. The change in MMP-9 mRNA expression was accompanied by parallel changes in the gelatinolytic activity of the active form of MMP-9, TIMP-1 mRNA expression, and MMP-9 immunoreactivity in mesangial cells. In contrast, glomerular MMP-2 mRNA expression and its activity increased after the decline of MMP-9. Conclusions. MMP-9 mRNA was predominantly expressed in the glomerulus in normal rat kidneys and MMP-9 was present in the mesangium. The MMP-9 mRNA expression increased in the glomerulus 3 to 7 days after the induction of anti-GBM glomerulonephritis in WKY rats, in parallel with the development of abnormal glomerular histology and injury, suggesting a role of MMP-9 in proteolysis of the GBM during glomerulonephritis. MMP-2 may participate in the later phase of the nephritis. [ABSTRACT FROM AUTHOR]

AIM: To determine factors predicting relapse and poor outcome in patients with type I autoimmune hepatitis (AIH).METHODS: Patients with AIH were retrospectively recruited. Definitions—remission: AST/ALT<2 ULN; relapse: AST/ALT≥ 2 ULN; poor outcome: cirrhosis complications, transplantation (OLTx), and death; abnormal transaminases: AST/ALT>ULN but within the remission range; abnormal transaminases index (ATI): number of occasions AST/ALT abnormal/years of remission. Liver biopsies were assessed by Ishak system, and additional score given for portal and parenchymal plasma cells. Data are presented as median (range).RESULTS: Seventy-one patients were identified. Twenty (28%) had cirrhosis at presentation, 14 (20%) developed it during follow-up of 52 months (18–336). Of the 14, four had histological confirmation, and the remainder had clinical/radiological evidence of cirrhosis. Factors independently associated with cirrhosis development were inability to have consistently normal transaminases during remission, OR 19.3 (95% CI 2.2–40),p= 0.002. Treatment was discontinued in 40/69 patients of whom 30 (75%) relapsed within 2 months (1–23), culminating in one death. Factors independently associated with relapse were: time to initial remission, OR 5.5, 95% CI 1.3–22,p= 0.01; failure to have consistently normal transaminases during remission OR 11.8, 95% CI 1.3–100,p= 0.02; and portal plasma cell score (PPCS) OR 10.6 (95% CI 1.0–107),p= 0.04. Time to remission≥ 5 months, PPCS≥ 3 and ATI≥ 2 was associated with>90% probability of relapse (PPV 100%). Fifteen percent had a poor outcome. Independent predictors of poor outcome were: globulins at onset OR 3.4 (95% CI 1.1–10.1),p= 0.02 and cirrhosis development, OR 23 (95% CI 1.7–307),p= 0.CONCLUSIONS: Seventy percent of patients with AIH relapse upon drug cessation. Time to remission≥ 5 months, ATI≥ 2 and PPCS≥ 3 were associated with>90% probability of relapse. Factors predicting poor outcome were globulins at onset and cirrhosis development. [ABSTRACT FROM AUTHOR]

In recent years a peculiar type of chronic pancreatitis with underlying autoimmunity has been described. Lymphoplasmacytic infiltration and fibrosis on histology and elevated IgG levels or detected autoantibodies on laboratory data support the concept of autoimmune chronic pancreatitis (AIP). Pancreatic imaging reveals a rare association of diffuse enlargement of the pancreas and irregular narrowing of the main pancreatic duct, which is unique and specific to AIP. Although AIP is not a common disease, it is increasingly being recognized as knowledge of this entity builds up. Clinically it is very important to be aware of this disease because AIP can clinically disguise as pancreaticobiliary malignancies, ordinary chronic, or acute pancreatitis. Above all, AIP is a very attractive disease to clinicians in terms of its dramatic response to oral steroid therapy in contrast to ordinary chronic pancreatitis. This review discusses the clinical, laboratory, histologic, and imaging findings that are seen in patients with AIP, especially focusing on the diagnosis. [ABSTRACT FROM AUTHOR]

The infiltration of leukocytes into the glomeruli is a major factor in inflammatory glomerular damage in acute poststreptococcal glomerulonephritis (APSGN). Chemokines participate in leukocyte infiltration. The aim of the present study was to investigate the role of monocyte chemoattractant protein-1 (CCL2/MCP-1) and interleukin-8 (CXL8/IL-8) in APSGN with special emphasis on their role in the clinical course of renal disease. Twenty-one children with APSGN were studied. Serum and urinary CCL2/MCP-1 and CXL8/IL-8 levels were measured by ELISA. The relationships between urinary chemokines and the degree of proteinuria were investigated. Serum and urinary CCL2/MCP-1 levels were significantly higher in the acute phase than in the resolution phase and in controls ( P <0.05). Urinary CCL2/MCP-1 levels in the control group were significantly lower than in both the acute and resolution phases ( P =0.01 and P =0.001, respectively). In the acute phase, urinary CCL2/MCP-1 correlated with the extent of proteinuria ( r =0.58, P =0.006) but not with serum CCL2/MCP-1 levels ( r =0.21, P =0.36). Urinary and serum CXL8/IL-8 levels were significantly elevated in the acute phase compared with the resolution phase and controls ( P <0.05). A consistent increase in urinary CCL2/MCP-1 was found in the acute phase of patients with APSGN, and this correlates with the degree of proteinuria. Our results emphasize the important role of locally produced chemokines in immune-mediated glomerular injury. [ABSTRACT FROM AUTHOR]

Human T-cell leukemia/lymphoma virus type 1 (HTLV-1) persists despite a vigorous virus-specific host immune response, and causes adult T-cell leukemia and lymphoma in approximately 2% of infected individuals. Here we report that HTLV-1 has evolved a genetic function to restrict its own replication by a novel post-transcriptional mechanism. The HTLV-1-encoded p30II is a nuclear-resident protein that binds to, and retains in the nucleus, the doubly spliced mRNA encoding the Tax and Rex proteins. Because Tex and Rex are positive regulators of viral gene expression, their inhibition by p30II reduces virion production. p30II inhibits virus expression by reducing Tax and Rex protein expression. [ABSTRACT FROM AUTHOR]

Two cases of hypertrophic pachymeningitis (HP) associated with pulmonary silicosis in tunnel workers are described. In both cases the myeloperoxidase antineutrophil cytoplasmic autoantibody (MPO-ANCA) was positive. Two patients with pulmonary silicosis developed headache and neurological disturbance, and a diagnosis of HP was made. In both cases the serum CRP level and the MPO-ANCA titer were elevated. Corticosteroid therapy produced a rapid improvement in all the clinical and laboratory parameters. Although an association has been noted between exposure to silica dust and ANCA-associated vasculitis, particularly glomerulonephritis, central nervous system involvement is rare. However, there have been some recent reports of HP cases that were positive for ANCA, and the association between HP and vasculitis has been discussed in the medical literature. HP may be one feature of multiorgan involvement in ANCA-associated disease, and the association between silica dust exposure and HP should be considered, as with other forms of ANCA-associated vasculitis. [ABSTRACT FROM AUTHOR]

Retinoids, derivatives of vitamin A, inhibit mesangial cell proliferation, glomerular inflammation, and extracellular matrix deposition in acute anti-Thy1.1 glomerulonephritis (Thy-GN) of the rat. We examined a model, chronic mesangioproliferative Thy-GN (MoAb 1-22-3), which is more akin to human disease. Treatment started on day 23 when Thy-GN had already been established. Nonnephritic control and Thy-GN rats were treated orally for 67 days with vehicle or with two doses of either the retinoic acid receptor α-specific agonist AGN 195183 (RARα agonist) or the retinoid X receptor specific agonist AGN 194204 (RXR agonist). Doses of either the RARα or the RXR agonist significantly reduced albuminuria and normalized blood pressure during the course of treatment. The glomerulosclerosis index, glomerular cell and interstitial cell counts, and area of the interstitial space were significantly lower in nephritic rats treated with the RARα agonist or RXR agonist than with vehicle. The RARα and RXR agonist significantly reduced the infiltration of the glomerulus by macrophages. The increase in glomerular TGFβ1 and prepro-ET1 gene expression in vehicle-treated nephritic rats was significantly attenuated by RARα or RXR agonists. Glomerular expression of RXRα and RARα receptor mRNA was significantly greater in vehicle-treated nephritic rats than in nonnephritic controls. Treatment with RARα or RXR agonists tended to normalize retinoid-receptor gene expression. Our data indicate that both RARα agonists and RXR agonists reduce renal damage in rats with established chronic glomerulonephritis. Receptor-specific retinoids may provide a novel therapeutic approach for the treatment of chronic glomerulonephritis. [ABSTRACT FROM AUTHOR]

We present a girl with type I membranoproliferative glomerulonephritis (MPGN) diagnosed by the third renal biopsy. The first renal biopsy was performed at age 11.2 years after microscopic hematuria (which was revealed by school urinary screening) had persisted for 3 months, along with a low level of serum C3. Pathological examination of the biopsied specimen revealed endocapillary proliferative glomerulonephritis with multiple humps. The serum C3 level increased to within the normal range 2 months after the first renal biopsy, and the microscopic hematuria disappeared at age 12.3. However, microscopic hematuria, proteinuria, and the low serum complement level reappeared at age 12.8. Pathological examination of a further renal biopsy that was performed at age 13.2 revealed focal MPGN with humps. Prednisolone therapy was subsequently initiated. Fluvastatin was added to her treatment regime when she developed hypercholesterolemia at age 13.6 and was continued even after normal cholesterol levels were reestablished. Pathological examination of the third renal biopsy, which was performed at age 15.2, revealed type I MPGN with humps. Serum C3 normalized 6 months after the cessation of prednisolone at age 15.9. It is clinically important that patients with nontypical acute glomerulonephritis should be observed over a long period and repeated renal biopsies should be performed. [ABSTRACT FROM AUTHOR]

Unlabelled: Henoch-Schönlein purpura (HSP) is a form of systemic vasculitis characterised by vascular wall deposits of predominally IgA typically involving small vessels in skin, gut and glomeruli and associated with purpura, colic, haematuria and arthralgia or arthritis. HSP nephritis (HSPN) leads to chronic renal failure in up to 20% of paediatric patients after 20 years of follow-up in selected series. The risk is related to the initial clinical presentation and the percentage of glomeruli presenting with epithelial crescents. The pathogenesis of HSPN might be related to an increased production of abnormally glycosylated IgA, which is not sufficiently cleared by the liver and leads to the formation of IgA macromolecules, accumulating in the circulation with subsequent deposition in vessel walls and in the glomerular mesangium. HSPN is related to IgA nephropathy. These two diseases can be encountered consecutively in the same patient, have been described in identical twins and bear similar pathological and biological abnormalities. No consensus about treatment has been reached up to now. Recent studies indicate that early treatment with methylprednisolone or a combination of steroids and cytotoxic drugs might prevent evolution to chronic renal failure.Conclusion: Despite numerous studies, the pathogeny of Henoch-Schönlein nephritis remains incompletely elucidated and controlled therapeutic trials are still needed. [ABSTRACT FROM AUTHOR]

Schistosomiasis or bilharzia is a parasitic disease found in tropical countries. Most infections are subclinical but may progress to chronic form characterized most frequently by the presence of liver involvement and portal hypertension. We report a patient that presented chronic polyarthritis with positive rheumatoid factor. During investigation, increased liver enzymes, negative hepatitis serologies and signs of portal hypertension on an ultrasound examination raised suspicion of S. mansoni infection. We will discuss pathophysiology and clinical manifestations of S. mansoni infection with special attention to articular involvement. [ABSTRACT FROM AUTHOR]

The convalescence phase of severe meningococcal sepsis is complicated by immune complex reactions with arthritis being the commonest. No standard guidelines exist for management of such complications, but non-steroidal anti-inflammatory drugs and steroids have been used with varying success. We report excellent response to intravenous immunoglobulin in a child with immune complex reaction following meningococcal sepsis. [ABSTRACT FROM AUTHOR]

Wilms’ tumour suppressor gene 1 ( WT1) encodes a transcription factor required for normal development of the genitourinary system. In the kidney, mutations in WT1 can cause diffuse mesangial sclerosis or focal segmental glomerulosclerosis. Here, we report on a girl with a mutation in WT1, who developed membranoproliferative glomerulonephritis (MPGN) 3 years after completion of treatment for Wilms’ tumour. This finding extends the spectrum of glomerular disease seen with WT1 mutations and could have implications for the screening of children with MPGN. [ABSTRACT FROM AUTHOR]

Acute post-streptococcal glomerulonephritis (APSGN) is rare in children under 2 years of age. This is related in part to the disease patterns of group A streptococcus (GAS) and in part to impaired immunogenicity in infants. We report the case of a 14-month-old child with APSGN following GAS pharyngitis. This case illustrates that APSGN needs to be considered in the evaluation of both gross and microscopic hematuria in this age group. We review the literature of both GAS and APSGN and discuss the pathogenesis and epidemiologic reasons for this association. [ABSTRACT FROM AUTHOR]

Renal manifestations associated with infective endocarditis (IE) may present with different clinical patterns, and the most common renal histopathological finding is diffuse proliferative and exudative type of glomerulonephritis, leading to hematuria and/or proteinuria. Renal failure due to crescentic glomerulonephritis (CGN) in children with IE is a very rare condition. We report here a 6-year-old boy, who had a history of cardiac surgery for pulmonary atresia and ventricular septal defect, presenting with the clinical findings of IE and hematuria associated with renal failure due to CGN. He was treated with a combination of intravenous (IV) methylprednisolone pulses and appropriate antibiotics, but also received one dose of IV cyclophosphamide. Complete serological, biochemical, and clinical improvement was achieved after 2 months of follow-up. Antibiotic therapy is the essential part of the treatment of IE-associated glomerulonephritis; however, this case also highlights the importance of aggressive immunosuppressive therapy to suppress the immunological process related with infection in this life-threatening condition leading to renal failure. [ABSTRACT FROM AUTHOR]

There have been few reports on immune complex-mediated glomerulonephritis associated with chronic infection from long-term central venous catheterization in adulthood. We report here on a 13-year-old boy with nephritis who exhibited glomerulonephritis that had been induced by the long-term use of central venous catheters, and its resolution after extraction of the central venous catheter. A diagnosis of glomerulonephritis associated with chronic infection caused by long-term central venous catheterization was made, based on the absence of clinical findings after removal of the catheter, hypocomplementemia, pathology findings resembling membranoproliferative glomerulonephritis, and detection of Staphylococcus epidermidis from culture of the removed catheter culture. For clinicians using long-term central venous access for parenteral feeding, rapid catheter exchange is necessary for patients with fever of unknown origin. [ABSTRACT FROM AUTHOR]

Immune complex and complement systems play an important role in membranoproliferative glomerulonephritis (MPGN). X-linked agammaglobulinemia (XLA) is a primary immunodeficiency characterized by severe hypogammaglobulinemia. We report the case of an XLA patient who developed MPGN during an intravenous immunoglobulin (IVIG) treatment. In this patient, the serum IgG level was maintained at more than 400 mg/dl of regular IVIG administration (2.5 g/dose/month). The patient presented with microscopic hematuria, proteinuria (U-pro/Cr: 4.0–4.2) and low serum complement levels (C3: 57.8 mg/dl) 3 years after IVIG treatment and was diagnosed histopathologically as having MPGN type III. Both hematuria and proteinuria significantly improved, and the serum complement level returned to a normal level following methylprednisolone pulse therapy. To our knowledge, this is the first case report of MPGN associated with XLA. Although it is unclear how MPGN occurred in this XLA patient, we suggest that residual humoral immunity in the patient could be associated with the development of MPGN. [ABSTRACT FROM AUTHOR]

We report a case of rapidly-progressive glomerulonephritis complicating Behcet’s disease (BD). A 44-year-old male has suffered from recurrent oral ulcers and retinal vasculitis developed 2 years ago. He complained of abdominal pain and papulopustular skin lesions. Multiple ulcers were seen on the colon on colonoscopy. Routine renal work-up revealed heavy proteinuria and hematuria. Renal biopsy demonstrated crescentic glomerulonephritis. Most symptoms improved after steroid therapy, except for urinary abnormalities. At this point, intravenous monthly cyclophosphamide pulse therapy was undergone. After the sixth pulse therapy, proteinuria and hematuria were dramatically improved and renal function was well preserved. [ABSTRACT FROM AUTHOR]

Selective IgA deficiency associated with glomerulonephritis is rare and no previous reports in childhood have been made of the association of IgA deficiency and membranous glomerulonephritis (MGN). We report a 5-year-old boy with selective IgA deficiency and MGN. He presented with nephrotic syndrome. Percutaneous renal needle biopsy showed diffuse global thickening on light microscopy and heavy IgG and moderate C3 deposits were found on immunofluorescence. Electron microscopy detected extensive global subepithelial deposition of electron-dense material with frequent intramembranous extension and spike formation. The pathological diagnosis was diffuse MGN stage 1. Oral prednisolone (1 mg kg-1 day-1), angiotensin-converting enzyme inhibitors (ACEI), and angiotensin II receptor blocker (ARB) were given resulting in reduction of proteinuria. The prednisolone dose was gradually tapered and discontinued after 2 months. At present the patient has been in complete remission for 10 months despite the discontinuance of prednisolone. In conclusion, our treatment with corticosteroid, ACEI and ARB reduced proteinuria and was effective for our case with selective IgA deficiency and MGN. [ABSTRACT FROM AUTHOR]

Reversible posterior leukoencephalopathy syndrome (RPLS) is a recently described disorder with typical radiological findings of bilateral grey and white matter abnormalities in the posterior regions of the cerebral hemispheres. The majority of patients with RPLS are adults and it is rare in children. In this report, two patients with RPLS are presented. In the first patient the primary diagnosis was acute post-streptococcal glomerulonephritis, a known cause of RPLS both in adults and in children. The second patient had Henoch Schönlein purpura.Conclusion:These patients are presented to highlight the importance of reversible posterior leukoencephalopathy syndrome. As the spectrum of associated diseases is diverse, paediatricians must be aware of this syndrome in order to initiate appropriate management. [ABSTRACT FROM AUTHOR]