Minimal Change Glomerulopathy Research Articles

Disease spectrum of 9 310 cases of renal biopsy pathological diagnosis from a single center in China.

The pathological types of renal diseases have changed in the past decade, but it lacks large sample analysis in Hunan Province. This study aims to retrospectively analyze the composition characteristics of pathological spectrum of kidney disease in Hunan Province in recent 10 years, compare the changes of pathological types of kidney disease. We collected a total of 9 310 kidney biopsy cases from 2011 to 2020 registered in the kidney biopsy system of the Nephrology Institute of the Second Xiangya Hospital of Central South University. Patients were assigned into a 14-24 years old group, a 25-44 years old group, a 45-59 years old group, and a ≥60 years old group, and divided into 2 time periods, namely 2011-2015 and 2016-2020. Pathological types of renal disease were divided into primary glomerulonephritis (PGN), secondary glomerulonephritis (SGN), tubulo-interstitial nephritis (TIN), hereditary kidney disease, and other pathological types. PGN accounted for 66.93% cases. Among PGN, IgA nephropathy (IgAN) (42.83%), membranous nephropathy (MN) (19.16%), minimal change glomerulopathy (12.46%), and focal segmental glomerulosclerosis (FSGS) (14.97%) were the 4 most common pathological types. Compared with the group in the period of 2011-2015, IgAN (46.74% vs 37.33%) and MN (21.26% vs 16.18%) were increased significantly in years 2016-2020 (both P<0.001). SGN accounted for 24.49% cases, of which lupus nephritis (LN) (26.91%), hypertensive nephropathy (HT) (14.47%), diabetic nephropathy (DN) (12.25%), and henoch-schonlein purpuric nephritis (HSPN) (13.44%) were the 4 most common pathological types. LN (32.53%), HSPN (12.97%), hepatitis B virus associated nephritis (HBVN) (10.98%), HT (9.48%) were the most common SGN in the period of 2011-2015. In the period of 2016-2020, the top 4 pathological types of SGN were LN (23.21%), HT (17.75%), DN (15.84%), and HSPN (13.74%). LN was the most common type in young adults ≤44 years old. HT, DN, and monoclonal immunoglobulin associated renal disease (including amyloidosis nephropathy) became the main pathological types of SGN in middle-aged and elderly patients. IgAN remains the primary pathological type of kidney disease in China in the recent years. The patients with the age of 25-44 years old have the largest proportion of IgAN. With increases in age, the proportion of IgAN gradually decreases. MN increases to different degrees at various ages, while cases of HT, DN, and monoclonal immunoglobulin-related renal damage have increased. This single-center renal pathology spectrum can provide clinical evidence for diagnosis, prevention and epidemiological study.

New-onset and relapsing glomerular diseases related to COVID-19 vaccination.

COVID-19 vaccine, as one of the critical measures to control the pandemic, has been administered in nearly all countries. However, the new-onset and relapsing glomerular diseases associated with COVID-19 vaccination have become a new concern. Both mRNA vaccine and inactivated vaccine may cause new-onset and relapsing glomerular diseases; these diseases would occur after the first dose vaccination or the second dose. New-onset glomerular disease is mainly minimal change glomerulopathy, which is mostly sensitive to steroid, while relapsing cases have good prognosis, and some cases can be spontaneously remitted. The pathogenesis of these vaccine-associated diseases is possibly due to the humoral and cellular immune responses. In this article, we provide a general review of the new-onset and relapsing glomerular diseases related to COVID-19 vaccination, and make suggestions for patients with kidney diseases to receive COVID-19 vaccination.

Nephrotoxicity of Antimicrobials and Antibiotics.

Medication-induced nephrotoxicity remains one of the most common causes of acute kidney injury (AKI) among hospitalized patients. Within the extensive group of medications associated with AKI, antibiotics and other antimicrobials are well recognized triggers of structural and functional renal impairment. Clinical manifestations range from mild forms of tubular injury to significant deterioration of kidney function requiring acute renal replacement therapy. Several mechanisms are described, although the most frequent are acute interstitial nephritis, acute tubular necrosis, intratubular crystal deposition, and proximal/distal tubulopathy with electrolyte wasting abnormalities. General risk factors for antimicrobial-induced AKI include pre-existing chronic kidney disease, and concomitant use of medication with nephrotoxic potential. Prevention and early recognition of AKI represent the standard approach to mitigate AKI and avoid morbidity.

Thymoma-associated myasthenia gravis and LGI1-encephalitis, with nephrotic syndrome post-thymectomy

Thymoma is associated with a wide spectrum of autoimmune paraneoplastic syndromes, though it is uncommon for multiple paraneoplastic syndromes to be present in a single individual. We report a rare case of an elderly gentleman who was found to have thymoma-associated myasthenia gravis and LGI1-encephalitis with myokymia, who presented with nephrotic syndrome (minimal change glomerulopathy) after thymectomy. The latter two paraneoplastic syndromes had manifested when prednisolone was tapered down to low dose. This case serves to remind neurologists that apart from paraneoplastic neurological manifestations, thymoma may also be associated with renal disease. Nephropathy in myasthenia patients with thymoma should be properly evaluated, as it is treatable with immunotherapy, and it may even occur post-thymectomy.

Mutations in INF2 may be associated with renal histology other than focal segmental glomerulosclerosis

In 2010, INF2 mutations were associated with autosomal-dominant focal segmental glomerulosclerosis (FSGS), clinically presenting with moderate proteinuria in adolescence. However, in the meantime, cases with more severe clinical courses have been described, including progression to end-stage renal disease (ESRD) during childhood. INF2 mutations in patients with isolated FSGS are clustered in exons 2 to 4, encoding the diaphanous inhibitory domain, involved in the regulation of the podocyte actin cytoskeleton. We report a family with 14 affected individuals (autosomal-dominant mode of inheritance), most of whom presented with nephrotic-range proteinuria, hypertension, and progressive renal failure. Four members received a kidney transplant without disease recurrence. Two patients underwent renal biopsy with the result of minimal-change glomerulopathy and IgA nephropathy respectively. We performed mutational analysis of ACTN4, CD2AP, COQ6, INF2, LAMB2, NPHS1, NPHS2, PLCE1, TRPC6, and WT1 in the index patient by next-generation sequencing. Additionally, in 6 affected and 2 unaffected family members target diagnostics were performed. We identified a novel heterozygous mutation c.490G>C (p.(Ala164Pro) in exon 3 of the INF2 gene in the index patient and 6 additionally examined affected family members. In silico analysis predicted it as "probably damaging". Additionally, three patients and 2 unaffected relatives harbored a novel heterozygous variant in ACTN4 (c.1149C>G, p.(Ile383Met)) with uncertain pathogenicity. Mutations in INF2 are associated with familial proteinuric diseases - irrespective of the presence of FSGS and in the case of rapid disease progression. Therefore, mutational analysis should be considered in patients with renal histology other than FSGS and severe renal phenotype.

Long-Term Outcome of Steroid-Resistant Nephrotic Syndrome in Children.

We investigated the value of genetic, histopathologic, and early treatment response information in prognosing long-term renal outcome in children with primary steroid-resistant nephrotic syndrome. From the PodoNet Registry, we obtained longitudinal clinical information for 1354 patients (disease onset at >3 months and <20 years of age): 612 had documented responsiveness to intensified immunosuppression (IIS), 1155 had kidney biopsy results, and 212 had an established genetic diagnosis. We assessed risk factors for ESRD using multivariate Cox regression models. Complete and partial remission of proteinuria within 12 months of disease onset occurred in 24.5% and 16.5% of children, respectively, with the highest remission rates achieved with calcineurin inhibitor-based protocols. Ten-year ESRD-free survival rates were 43%, 94%, and 72% in children with IIS resistance, complete remission, and partial remission, respectively; 27% in children with a genetic diagnosis; and 79% and 52% in children with histopathologic findings of minimal change glomerulopathy and FSGS, respectively. Five-year ESRD-free survival rate was 21% for diffuse mesangial sclerosis. IIS responsiveness, presence of a genetic diagnosis, and FSGS or diffuse mesangial sclerosis on initial biopsy as well as age, serum albumin concentration, and CKD stage at onset affected ESRD risk. Our findings suggest that responsiveness to initial IIS and detection of a hereditary podocytopathy are prognostic indicators of favorable and poor long-term outcome, respectively, in children with steroid-resistant nephrotic syndrome. Children with multidrug-resistant sporadic disease show better renal survival than those with genetic disease. Furthermore, histopathologic findings may retain prognostic relevance when a genetic diagnosis is established.

The Evolving Role of Rituximab in Adult Minimal Change Glomerulopathy

Background: Minimal-change glomerulopathy is defined histologically by the presence of normal glomeruli on light microscopy and diffuse podocyte effacement on electron microscopy. Although effective in children, corticosteroid treatment in adults is more variable and time to response can be prolonged. Data to support rituximab use in adults with corticosteroid-dependent or resistant minimal-change glomerulopathy are limited. Here, we describe the clinical course of adults with corticosteroid-dependent or -resistant minimal-change glomerulopathy who received rituximab. Methods: Demographic and clinical data were collected and analyzed from all adult patients with native kidney, biopsy-proven, minimal-change glomerulopathy who were administered rituximab between 2009 and 2014 and cared for at the UNC Kidney Center. Results: Ten patients with corticosteroid-resistant (n = 5) or corticosteroid-dependent (n = 5) idiopathic minimal-change glomerulopathy were treated with rituximab between 2009 and 2014. Rituximab treatment induced remission in all 10 patients with a median time to remission of 2 months. The median time from rituximab to corticosteroid discontinuation was 3.5 months. The median remission time was 29 months and follow-up time was 39.5 months. No serious adverse events attributable to rituximab were observed. Conclusion: Rituximab induced remission in all patients with corticosteroid-dependent or -resistant minimal-change glomerulopathy, and may hold great therapeutic potential with good efficacy and minimal toxicity. Mounting evidence implies that a well-conducted randomized controlled clinical trial using rituximab in adults with minimal-change glomerulopathy in both corticosteroid-resistant and corticosteroid-dependent patients is warranted.

Acute kidney injury KDIGO stage 2 to 3 in HIV-positive patients treated with cART--a case series over 11 years in a cohort of 1,153 patients.

We aimed to explore acute kidney injury (AKI) Kidney Disease Improving Global Guidelines (KDIGO) stage 2 to 3 in a cohort of antiretroviral treated HIV-infected individuals. HIV-infected individuals of the Swiss HIV Cohort Study (Basel site), treated with combination antiretroviral therapy (cART) 2002-2013, were included. AKI was defined and classified according to the KDIGO Clinical Practice Guidelines for AKI. Data were prospectively collected and reports of kidney biopsies obtained from records. Among 1,153 cART-treated patients, 13 experienced AKI KDIGO stage 2 to 3 (1 patient stage 2, 12 patients stage 3; median age 46 years; 9 male; median CD4 count 366 cells/μl), corresponding to an incidence rate of AKI of 0.77 (95% confidence interval 0.45-1.33) per 1000 patient-years. Baseline estimated glomerular filtration rate (eGFR) was 87 ml/min (interquartile range 66-100). Ten patients were treated with tenofovir (TDF). Nine patients (69%) had ≥1 cardiovascular risk factor, only two patients had known pre-existing kidney disease. Three patients needed chronic and two temporary dialysis. AKI was associated with TDF therapy in 6 of 13 (46%) patients (mean TDF exposure time before AKI 41 months). Impaired renal function was partially reversible in all patients. In three patients with biopsy-proven pre-existing kidney disease (AA amyloidosis, calcineurin inhibitor-induced nephropathy and minimal change glomerulopathy), TDF potentially added to AKI. AKI KDIGO stage 2 to 3 demonstrates complex associations at the individual level and can occur without early signs. Although treatment with TDF and presence of cardiovascular risk factors were found frequently, predicting AKI seems very difficult.

Decreased TIM-3 mRNA expression in peripheral blood mononuclear cells from nephropathy patients.

Increasing evidence shows that TIM-1 and TIM-3 in-fluence chronic autoimmune diseases, and their expression levels in immune cells from nephritic patients are still unknown. Real-time transcription-polymerase chain reaction analysis was used to deter-mine expression levels of TIM-1 and TIM-3 mRNA in peripheral blood mononuclear cells (PBMCs) from 36 patients with minimal change glo-merulopathy (MCG), 65 patients with lupus nephritis (LN), 78 patients with IgA nephropathy (IgAN), 55 patients with membranous nephropa-thy (MN), 22 patients with crescentic glomerulonephritis (CGN), 26 patients with anaphylactoid purpura nephritis (APN), and 63 healthy controls. TIM-3 mRNA expression significantly decreased in PBMCs from nephritic patients (LN, P < 0.0001; MCG, P < 0.0001; MN, P = 0.0031; CGN, P = 0.0464; IgAN, P = 0.0002; APN, P = 0.0392) com-pared with healthy controls. In contrast, there was no significant differ-ence in TIM-1 mRNA expression between the patients and the healthy controls. Our results suggest that insufficient expression of TIM-3 mRNA may be involved in the pathogenesis of nephropathy.

Treatment of Frequently Relapsing Nephrotic Syndrome

Source: Gellerman J, Weber L, Pape L, et alfor the Gesellschaft für Pädiatrische Nephrologie. Mycophenolate mofetil versus cyclosporin A in children with frequently relapsing nephrotic syndrome [published online ahead of print June 27, 2013]. J Am Soc Nephrol; doi: 10.1681/ASN.2012121200Investigators from Germany conducted a multicenter, randomized, open-label, crossover trial comparing the efficacy and safety of mycophenolate mofetil (MMF) to cyclosporin A (CsA) in pediatric patients with frequently relapsing steroid-sensitive nephrotic syndrome (FR-SSNS). FR-SSNS was defined as >4 relapses in 12 months. Patients with FR-SSNS who were 3 to 18 years old and had minimal change glomerulopathy on biopsy, an estimated glomerular filtration rate (eGFR) of ≥90 ml/min per 1.73 m2, and were in remission after a recent relapse were eligible for inclusion. Participants were randomized to receive either MMF for 12 months followed by CsA for 12 months (Group A) or CsA for 12 months followed by MMF for 12 months (Group B).Demographic and clinical characteristics were collected at baseline. During the study period of 2 years, participants had monthly physical examinations, assessment of side effects, screening for proteinuria, and therapeutic drug monitoring. Other blood tests (eg, lipid profile) were performed every 3 months and blood pressure (BP) monitoring was performed at 12 and 24 months. The primary study outcome was the number of relapses per patient per year during the treatment period. Secondary endpoints included pharmacokinetic profiles, cumulative dose of corticosteroids, side effects, and changes in eGFR, BP, and lipid profile.A total of 60 patients (80% boys) were enrolled and randomized to the 2 treatment groups. There were no significant differences between groups in participant age, eGFR, duration of disease, or relapses before study entry. Most participants (75%) had received previous courses of immunosuppressive therapy in addition to corticosteroids.Significantly more patients relapsed while on MMF than on CsA during the first year of the trial (1.10 relapses per patient per year vs 0.24, P = .03) but not during the second year. The time without relapse was significantly longer with CsA compared to MMF during the first year (P < .05), but not during the second year. The eGFR decreased from 12 months to 24 months in Group A (corresponding with CsA treatment), and increased from 12 months to 24 months in Group B (corresponding with MMF treatment). There were no statistically significant follow-up differences between groups with regards to blood pressure, lipid levels, or adverse events.The authors conclude that MMF was inferior to CsA in preventing relapses in children with FR-SSNS but may be a less nephrotoxic treatment.Dr Sethna has disclosed no financial relationship relevant to this commentary. This commentary does not contain a discussion of an unapproved/investigative use of a commercial product/device.Idiopathic nephrotic syndrome, namely minimal change nephrotic syndrome, diffuse mesangial proliferation, and focal segmental glomerulosclerosis, account for 90% of all cases of nephrotic syndrome with an incidence in the United States of 2 to 7 per 100,000.1 The first line therapy is corticosteroids. Approximately 80% of cases are steroid-responsive at presentation, indicating a favorable prognosis for kidney function.1 Children who become steroid-dependent (SDNS) or frequently relapse are more difficult to treat.Although the pathogenesis of SDNS and FR-SSNS are not fully understood, an underlying immunological defect is suspected and serves as the rationale for use of second-line immunosuppressant treatment. Such second-line agents are also utilized to avoid serious side effects of prolonged steroid exposure. The preferred class and sequence of immunomodulatory drugs for the treatment of SDNS and FR-SNSS have varied with time and region. Alkylating agents such as cyclophosphamide, chlorambucil, levamisole, and the calcineurin inhibitor CsA have been used for over 20 years.2 Severe side effects, however, have led to consideration of several new classes of drugs that target various stages of T- and B-cell action. Tacrolimus, a calcineurin inhibitor that inhibits interleukin-2-driven T-cell activation, has shown promising results in various single-centered studies. 3 Tacrolimus has a milder side effect profile than CsA; however, nephrotoxicity remains a limitation.MMF, a T- and B-cell proliferation inhibitor, has been recently introduced for the treatment of SSNS. Although the current study demonstrated inferiority of MMF compared to CsA in preventing relapses, it is efficacious in inducing remission of nephrotic syndrome with the advantage of no risk of nephrotoxicity. More recently, the monoclonal antibody rituximab, an anti-B-cell treatment, has been used as a “rescue medication” for especially difficult patients. Past studies have shown promising results although long-term side effects and remission sustainability are uncertain.4 Treatment of SDNS and FR-SSNS in children can often prove to be challenging. There is continued need for large-scale randomized controlled trials that provide strong evidence to guide therapy.

Patientin mit Diabetes mellitus Typ 1 und akut aufgetretenen Ödemen

A 47-year-old woman with type 1 diabetes mellitus was presented for evaluation of progressive oedema, fatigue and weight gain. Her medical history was significant for arterial hypertension and autoimmune thyroiditis requiring substitution therapy with levothyroxine. Physical examination revealed bilateral malleolar and crural oedema, swelling of the eyelids and two-sided pleural effusions. DIAGNOSTIC, TREATMENT AND COURSE: The blood level of albumin was very low, urine analysis showed proteinuria of > 8 g/day. The kidney biopsy revealed only slight changes. This led in combination with the blood and urine results to the diagnosis of minimal change glomerulopathy. After initiation of high dose prednisolone the patient achieved near total remission within four weeks. Prednisolone therapy was tapered over several months. In patients with diabetes mellitus and suddenly occurring nephrotic syndrome other diseases than diabetic nephropathy have to be considered. In most cases a kidney biopsy is mandatory.

Histological patterns of idiopathic steroid resistant nephrotic syndrome in Egyptian children: A single centre study

Idiopathic steroid resistant nephrotic syndrome (ISRNS) represents about 10-20% of children with nephrotic syndrome with variable outcome. To determine the histological patterns of ISRNS in Egyptian children and the histological details of the commonest types which might be the reason for the steroid resistance. The study included 53 cases with ISRNS. Their renal biopsies were retrieved from the archive of Electron microscopy unit and pathology department, Ain Shams University Specialized Hospital (ASUSH) in the duration from 2005-2011. The biopsies were examined histologically, with immunohistochemistry, and by electron microscopy. They were 36 males (67.9%) and 17 females (32.1%), the age at diagnosis ranged from 1.5- 16 years with a mean of 6.71 years. Lower limb oedema was the commonest presentation (100%), haematuria was revealed in (17%) of cases. Histological examination showed three major patterns; Focal segmental glomerulosclerosis (FSGS) in 30.2%, minimal change glomerulopathy (MCG) in 24.5% and IgA nephropathy in 13.2 %. Mesangial hypercellularity was very common among MCG patients (85.3% ±6.7). Tubulointerstitial inflammation and fibrosis were common among cases with IgA nephropathy (40.4% ±11, 53.7% ±8 respectively). ISRNS in Egyptian children could be attributed mainly to three major diseases (FSGS, MCG and IgA nephropathy). Mesangial hypercellularity and severe tubulointerstitial disease might be the major causes for steroid resistance in MCG and IgA nephropathy respectively. Renal biopsy with electron microscopy examination should be done for all children with nephrotic syndrome at first time of presentation for proper assignment of treatment protocol.

Accelerated recovery from nephrotic syndrome with acute renal failure by double filtration plasmapheresis in a patient with lupus podocytopathy

We describe a case of an adult female who presented with nephrotic syndrome. She was diagnosed with systemic lupus erythematosus with serum antinuclear antibodies, leucopenia with lymphopenia, butterfly erythema, and nephrotic syndrome. Renal biopsy revealed normal glomeruli with diffuse effacement of the foot processes, consistent with lupus podocytopathy. Although human albumin replacement was performed initially, acute renal failure developed rapidly. Therefore, she was treated with double filtration plasmapheresis (DFPP) in addition to oral steroid. After steroid therapy combined with DFPP, the renal function and proteinuria improved rapidly. Although the impact of DFPP on the treatment of lupus nephritis remains to be delineated, our observations suggest that DFPP in lupus podocytopathy played a pivotal role in facilitating the early recovery from renal injuries. Because of the rapid improvement of renal function without any change in body weight by DFPP, acute renal failure in the setting of lupus podocytopathy might contribute to an alternative pathophysiological factor for the diminished glomerular filtration rate, similar to that observed in the setting of idiopathic minimal change glomerulopathy.

Benign fasciculations and Corticosteroid use: possible association? An update

Fasciculations are characterized by visible subtle and fast contractions of muscle, even wormlike in movement, by the contraction of a fascicle of muscle fibers. The authors present the case study of a 28-year-old patient with the appearance of migratory and diffuse fasciculations with an onset after partial tapering off of oral corticosteroides (60 mg total dose) indicated for treatment of Minimal change Glomerulopathy. Clinical Neurological physical exam allied with an ENMG, besides other complementary laboratory exams were used for screening the above-mentioned patient. Afterwards, current research relating to the topic at hand was made in order to update the data available in the Bireme, Scielo and PubMed Data Banks using the following key words: Fasciculation's, motor neuron disease, and benign fasciculations in the Portuguese, English as well as Spanish language. Although fasciculation's are most commonly associated with Motor neuron disease as well as with certain metabolic disorders, they may also be present in individuals with absolutely no underlying pathological disorders. In our case, fasciculation potentials that have been present for six months, with no other signs of a neurogenic disorder as well as absence of laboratory findings, the patient received a diagnosis of Benign Fasciculation Syndrome (BFS).We believe that the use of corticosteroides in high doses with subsequent tapering contributed to the fasciculation's, especially due to the changes that this causes on the ionic channels. Fasciculation's are symptoms seen in a large range of conditions, and also being the main symptom of the so-called Benign Fasciculation Syndrome. We have presented an example of this clinical syndrome in a patient whose complaint was fasciculation's, with complete clinical remission of symptoms following complete tapering off of corticosteroid six months previously.

Steroidowrażliwość oraz steroidooporność w dziecięcym idiopatycznym zespole nerczycowym – czy zaszła zmiana na przestrzeni dwóch dekad?

BackgroundIt is widely known that childhood idiopathic nephrotic syndrome (INS) seems to be benign, mostly minimal change glomerulopathy, sensitive to steroids with the tendency to relapse. There are reports that the histopathology and response to treatment in INS may be changing. AimThe aim of the study was to search for changes in the characteristics of the steroid sensitivity and steroid resistance in childhood INS within twenty years. Material and methodsRetrospective chart analysis was done to identify all children with new-onset nephrotic syndrome admitted to the Division of Pediatric Nephrology in Zabrze within 1986–2005. Comparison of clinical characteristics and histopathology in subgroups of steroid sensitive 59 vs 66 and steroid resistant 17 vs 36 patients in two following decades was performed. ResultsIn a group of steroid sensitive patients no significant difference in clinical characteristics at onset apart from increasing frequency of hematuria 16.9% vs 40.9%, p = 0.0034 was found. The frequency of renal biopsy as well as the frequency of relapses didn’t differ significantly in this group. In a group of steroid resistant patients the only clinical feature that differed significantly was a decrease in number of patients with hypertension 70.6% vs 38.9%, p = 0.031. There was no significant difference in histopathology of steroid resistant nephrotic syndrome with only slight increase in focal segmental glomerulosclerosis (FSGS) 17.6% vs 25%, p = 0.73 and mesangio-proliferative glomerulonephritis (MesPGN) 35.3% vs 47.2%, p = 0.41. ConclusionsIn analyzed population besides the increasing rate of hematuria in steroid sensitive patients and the decreasing rate of hypertension in steroid resistant patients, there have been no other changes in clinical and histopathological characteristics of steroid sensitivity and steroid resistance in childhood INS within the period of twenty years.

Minimal change glomerulopathy in a cat

A 6-year-old domestic shorthair male castrated cat was evaluated for sudden onset of vomiting and anorexia. A diagnosis of hypereosinophilic syndrome (HES) was made, and the cat was treated with imatinib mesylate. The cat had an initial clinical improvement with the normalization of the peripheral eosinophil count. After approximately 8 weeks of treatment, lethargy and anorexia recurred despite the normal eosinophil count and a significant proteinuric nephropathy was identified. Treatment with imatinib was discontinued. Ultrasound guided renal biopsies exhibited histologic, ultrastructural, and immunostaining changes indicative of a minimal change glomerulopathy (MCG) which has not previously been reported in the literature in a cat. The proteinuria and HES initially improved while the cat was treated with more traditional medications; however, both the problems persisted for 30 months that the cat was followed subsequently. Previous studies demonstrating the safety and efficacy of imatinib in cats do not report any glomerular injury or significant adverse drug reactions, and the exact cause of this cat's proteinuric nephropathy is uncertain. Nonetheless, the possibility of an adverse drug reaction causing proteinuria should be considered when initiating treatment with imatinib in a cat.

A Case of Minimal Change Disease Treated Successfully with Mycophenolate Mofetil in a Patient with Systemic Lupus Erythematosus

The World Health Organization classifies lupus nephritis as class I to V or VI. However, a few cases of minimal change glomerulopathy have been reported in association with systemic lupus erythematosus (SLE). Mycophenolate mofetil has been shown to be effective for treatment of minimal change disease and lupus nephritis. A 24-year-old woman diagnosed with SLE five years prior to presentation complained of a mild generalized edema. The urinalysis showed microscopic hematuria and proteinuria. The assessed amount of total proteinuria was 1,618 mg/24 hours. A renal biopsy demonstrated diffuse fusion of the foot processes of podocytes on electron microscopy. Mycophenolate mofetil was started in addition to the maintenance medications of prednisolone 10 mg/day and hydroxychloroquine 400 mg/day. After six months of treatment, the microscopic hematuria and proteinuria resolved, and the total urine protein decreased to 100 mg/24 hours.

41-jährige Patientin mit ST-Hebungsinfarkt und multiplen arteriellen Embolien

A 41-year-old woman presented with acute angina in the emergency unit. Additionally, she reported pain in both legs and a weight loss of 5 kilograms within the last 10 days. ECG revealed an acute anterior myocardial infarction. However, immediate coronary angiography showed open arteries with minimal arteriosclerosis. A characteristic rise of cardiac enzymes together with an akinesis of the anterior wall and an adjacent mural thrombus was highly suggestive of a transient coronary thrombosis. Further investigations showed occlusion of multiple arteries in both legs and a splenic infarct. Although there was a typical risk profile including smoking, hyperlipidemia and regular estrogen medication, a further work-up was started. Urin analysis was decisive for the presence of proteinuria and a severe nephrotic syndrome. The definite diagnosis was made by direct biopsy of the kidney that revealed the characteristic findings of a minimal change glomerulopathy. Rapid remission could be induced by high-dose oral steroids. During routine work-up of coronary syndromes, especially in those with normal coronaries, rare but treatable causes of myocardial infarction and coagulopathy have to be thought of and should carefully be excluded.

Characterisation of renal immune cell infiltrates in children with nephrotic syndrome

There is increasing evidence that not only T cells but also B cells may play an important role in the pathogenesis of idiopathic nephrotic syndrome (NS). We have evaluated the infiltrating immune cells found in renal biopsies from 38 children with NS using immunohistochemistry techniques involving antibodies against T cells (CD3, CD4, CD8, FoxP3), B cells (CD20), macrophages (CD68) and follicular dendritic cells (CD21). Kidney biopsies with thin basement membrane disease were used as controls. We found higher numbers of interstitial CD3-positive T cells and macrophages in patients with focal segmental glomerulosclerosis (FSGS) than in those with minimal change glomerulopathy (MCGN) and in the controls, and significantly lower FoxP3-positive cells in patients with FSGS, MCGN and steroid-dependent NS than in the controls. Significantly higher numbers of glomerular B cells were found in FSGN patients than in MCGN patients and controls. Of note, in three patients who were later successfully treated with anti-CD20 antibody rituximab, the number of renal B cells was negligible in the preceding biopsy. In conclusion, the higher numbers of interstitial CD3-positive T cells in renal biopsies of pediatric patients with FSGS argue for a higher inflammatory activity. The significantly higher number of glomerular B cells in FSGS patients may indicate a particular pathogenetic role or epiphenomenon in this disease. However, patients with no interstitial or glomerular B cells could also benefit from rituximab treatment.

A Clinicopathologic Study of Glomerular Disease: Experience of the King Fahd Hospital of the University, Eastern Province, Saudi Arabia

Published studies from different centers in Saudi Arabia have reported contradicting results regarding glomerular lesions. In this retrospective study, we report our experience in King Fahd Hospital of the University at Al-Khobar in the Eastern province, including a description of the morphologic and clinical characteristics of primary and secondary glomerular disease. The study included 233 renal biopsies obtained from patients presenting with glomerular manifestations over a period of 23 years (1986–2008), investigated by light microscopy, immunofluorescence (149 cases) and electron microscopy (34 cases). One hundred and eighty-seven cases (80.3%) were primary glomerulonephritides. Minimal change glomerulopathy was the most common type of primary glomerulonephritis found (29.4% of primary glomerulonephritides), followed by mesangioproliferative glomerulonephritis (19.8%), and focal/segmental glomerulosclerosis (15.5%). Membranoproliferative glomerulonephritis was found in 9.6% of cases, membranous glomerulopathy in 8.6%, IgA nephropathy in 6.4%, end-stage glomerulopathy in 5.9%, crescentic glomerulonephritis in 3.2%, and IgM nephropathy in 1.6%. Of the secondary glomerulonephritides (46 cases constituting 19.7% of the biopsies), lupus nephritis was the most frequently diagnosed disease (71.7% of secondary glomerulonephritides). Diabetic glomerulosclerosis was found in 10.9% of cases, amyloidosis in 6.5%, and Alport syndrome in 4.3%. Wegener's granulomatosis, Henoch-Schönlein purpura nephritis and hypertensive nephrosclerosis each represented 2.2% of cases (one case each). Other than a significantly higher incidence of minimal change glomerulopathy and lupus nephritis ( p < 0.001) and a significantly lower prevalence of membranoproliferative glomerulonephritis ( p = 0.029), our results are generally comparable to those reported by the Saudi registry for glomerulopathy and in some neighboring countries. Ageand sex-adjusted analyses revealed that minimal change glomerulopathy and lupus nephritis were also the most prevalent primary and secondary glomerulopathies in the pediatric age group (below 15 years) as well as in adults, females and males.