Percutaneous Renal Biopsy Research Articles

Renal Oncocytoma: The Diagnostic Challenge to Unmask the Double of Renal Cancer.

Renal oncocytoma represents the most common type of benign neoplasm that is an increasing concern for urologists, oncologists, and nephrologists due to its difficult differential diagnosis and frequent overtreatment. It displays a variable neoplastic parenchymal and stromal architecture, and the defining cellular element is a large polygonal, granular, eosinophilic, mitochondria-rich cell known as an oncocyte. The real challenge in the oncocytoma treatment algorithm is related to the misdiagnosis due to its resemblance, at an initial radiological assessment, to malignant renal cancers with a completely different prognosis and medical treatment. Unfortunately, percutaneous renal biopsy is not frequently performed due to the possible side effects related to the procedure. Therefore, the majority of oncocytoma are diagnosed after the surgical operation via partial or radical nephrectomy. For this reason, new reliable strategies to solve this issue are needed. In our review, we will discuss the clinical implications of renal oncocytoma in daily clinical practice with a particular focus on the medical diagnosis and treatment and on the potential of novel promising molecular biomarkers such as circulating microRNAs to distinguish between a benign and a malignant lesion.

POS-485 Percutaneous ultrasound-guided renal biopsy in children: A retrospective review of a tertiary hospital experience in Lagos, Nigeria

Percutaneous renal biopsy (PRB) is an invasive procedure in nephrology that is extremely important in making diagnosis, prognostication and evaluation of treatment outcome in children with renal diseases. Currently the use of real time ultrasound guided PRB has greatly increased the tissue yield and also reduced the complications associated with the procedure. The prevalence of renal diseases as well as mortality associated with renal diseases is increasing worldwide and especially in developing countries like Nigeria.

Percutaneous Renal Biopsy in Egyptian Children: A Five-year Single-Center Experience.

Ultrasound-guided percutaneous renal biopsy (PRB) stands as an important tool of diagnostic and prognostic value, besides its role in determining the best therapeutic option in some diseases. The advance in medicine over the past years made PRB safer and more feasible. This is an observational retrospective study in a tertiary referral center of pediatric nephrology and was conducted to determine both the indications and the histopathological findings of renal biopsies performed in pediatric patients. The retrospective review of 73 files searching for gender, age, indication for renal biopsy, and histopathological diagnosis of biopsy was done. The statistical analysis was done using Microsoft Excel Worksheet version 2010. The files of 73 cases were reviewed, of which three were excluded due to inadequate sample (success rate of 95.9%). The mean age was 6.9 years (standard deviation ±3.51) with a male-to-female ratio of 1.8:1. The main indication for PRB was nephrotic syndrome (NS) (40%) mainly steroid-resistant NS. Focal segmental glomerulosclerosis was found in most of the cases (46.4%) followed by minimal change disease (32.1%). Among secondary glomerulonephritis, lupus nephritis (LN) was the most common indication (15.7%). Class IV LN came at the top of the list (45.5%). Poststreptococcal GN patients were biopsied when rapidly progressive GN was suspected. Immunoglobulin A nephropathy was found in only 1.4%. Other PRB indications were hematuria (8.6%), unexplained acute (2.9%), or chronic renal failure (4.3%). Renal biopsy remains to be a mainstay diagnostic tool in pediatric nephrology. This study confirms the reliability of PRB as a diagnostic tool which can probably impact the management and hence improve the outcome. The findings in our patients align with findings from other centers and differ in others denoting that disease distribution can vary from one place to another.

POS-486 SECONDARY POLYCYTHEMIA IN NEPHROTIC SYNDROME – AN UNCOMMON ASSOCIATION

Polycythaemia is defined as haemoglobin (Hb) of over16.5 g/dL in men and 16 g/dL in women or haematocrit of over 49% in men and 48% in women (WHO 2017). Primary polycythaemiais caused by erythropoietin (EPO) receptor mutations or Janus Kinase 2 (JAK2) mutations. Secondary polycythaemia is occasionally seen with renal diseases such as cystic kidney disease, tumours, renal artery stenosis and after renal transplantation. Polycythaemia associated with Nephrotic Syndrome (NS) is very rare. This association was first described by Emmanuel DA and Wenzel FJ in 1962. Here, we describe four patients with NS who had polycythaemia at presentation.Heavy protienuria leading to renal interstitial oedema causing hypoxia and increased EPO production and high serum IL8 and increased stability of IL8 mRNA(in minimal change disease) are thought to cause secondary polycythaemia in NS. The resolution of polycythaemia following reduction in proteinuria further strengthens the causation.The exact pathogenesis remains to be elucidated. This is a retrospective observational study conducted in NU Hospitals, Bengaluru, India.There were four patients with NS and polycythaemia, between February 2015 to May 2021. All patients with NSwere subjected to biochemical investigations and ultrasonography (USG) of the abdomen after a detailed history and physical examination as per our hospital protocol. Coagulation profile and blood grouping were done prior to renal biopsy. Percutaneous renal biopsy was done under real-time USG guidance. The tissue obtained was sent for Light microscopic examination, Immunofluorescence microscopy and Electron microscopy. In view of the polycythaemia, all these patients underwent a Chest X Ray, arterial blood gas (ABG) analysis, serum EPO and serum JAK2 mutation studies. All four patients in our study were men. Mean age was 32.25 ± 11.70 years. Mean Hb was 18.75 ± 1.22 g/dL. Mean hematocrit was 54.67 ± 6.5 %. Mean proteinuria at onset was 7.62 ± 7.45 grams/day. Mean serum creatinine at the onset was 1.30 ± 0.58 mg/dL. None had splenomegaly. All had normal total white cell and platelet count. None were smokers. ABG was normal in all. All patients had normal serum EPO levels (mean 3.4±1.64 mU/ml) and negative JAK2 mutation analysis. The etiology of NS was minimal change disease (MCD), primary membranous nephropathy (MGN), primary focal segmental glomerulosclerosis (FSGS) and IgA nephropathy in each one of them. PLA2R antibodies were positive in the serum and renal tissue of the patient with MGN.Immunosuppression was initiated in all patients except the patient with IgA nephropathy who had significant chronicity. Three out of four patients had resolution of polycythemia in parallel with remission of NS.The patient with MCD was detected to have polycythaemia during a relapse. This study highlights polycythaemia as an unusual complication of NS.Most cases of NS and polycythemia documented in the literature correspond to FSGS, mainly associated with polycythaemia vera. There are case reports of secondary polycythaemia associated with primary FSGS, MGN and MCD. We didn't do IL8 and IL11 assay in our patients. To the best of our knowledge ours is probably the largest case series of polycythaemia associated with NS.

POS-210 CT-GUIDED KIDNEY BIOPSY BY INTERVENTIONAL NEPHROLOGITS

Histopathology of renal parenchyma is the key diagnostic for the treatment of kidney diseases by nephrologists. Most nephrologists perform renal biopsy using percutaneous ultrasound-guided technic. Generally, this act is very simple for the majority of patients, but when the kidney size is small or the patient has an extreme obesity, this act could be a real challenge with more risk of complications. In Annonay Hospital (North Ardeche, France) all percutaneous renal biopsies are done by a CT guidance.

A0212 - The role of percutaneous renal tumor biopsy in frail patients with indeterminate small renal masses

A0212 - The role of percutaneous renal tumor biopsy in frail patients with indeterminate small renal masses

POS-293 IMPACT OF DIABETES ON MAJOR BLEEDING COMPLICATIONS OF RENAL BIOPSY

Nephrologists have recently recognized the heterogeneity of kidney diseases in patients with diabetes and begun to actively perform percutaneous renal biopsies (PRBs). However, the impact of diabetes on major bleeding complications of PRBs remains unclear.

Post-Renal Biopsy Deglobulization: Risk Factors and Prognosis: A Study of 157 Biopsies

Introduction: Percutaneous renal biopsy (PRB) is the gold standard for the diagnosis of most renal diseases. It is a safe and effective modality for the collection of renal tissue. However, many safety measures are not based on sufficient evidence and therefore vary considerably from a center to another. The aim of this work is to determine the rate of bleeding complications, to identify the risk factors for these complications, and to clarify the post renal biopsy prognosis. Materials and Methods: We performed a single-center retrospective observational study in the nephrology department at the University Hospital of Fez, including all patients who underwent percutaneous renal biopsy on native kidney between January 2018 and December 2019. Results: Overall, 157 biopsies were performed. Deglobulization was present in 20.4% (40) of patients, the mean age of patients was 41.57 ± 16.11 years [16.78]. The sex ratio M/F: 1.22. Diabetes mellitus was present in four cases (11.1%), arterial hypertension was present in four cases (11.1%). On clinical examination, systolic hypertension was found in 45.7%, diastolic hypertension in 45%, antihypertensive therapy was initiated in all patients with hypertension before. Hyperuremia was present in 29 patients (80.6%), renal failure was present in 77.8%. Anemia was present in 55.6%, thrombocytopenia in six cases (16.7%). Radiologically, the size of the kidneys was reduced in 5 patients (17.2%), differentiation was limited in 5 patients (17.2%). Major complications occurred in 3.8% (6/157). These six patients had a lumbar pain and required blood transfusions. A radiological embolization procedure was indicated in only one patient. Minor complications were seen in 21.6% (34/157). The diagnoses that were retained in patients with deglobulization were: Lupus in 34.71%, pauci-immune vasculitis in 13.79%, membranous glomerulonephritis in 10.34%, focal and segmental hyalinosis in 10.34%, membrano-proliferative glomerulonephritis in 10.34%. In univariate and multivariate analysis, the major risk factors for deglobulization found in our patients were: hyperuremia 80.6% (p: 0.017), acute renal failure 77.8% (p: 0.04), acute hemodialysis 24.7% (p: 0.02), hyperphosphatemia 63.6% (0.04). Conclusion: Renal biopsies are an overall safe procedure with rare major complications. Post-renal biopsy deglobulization is common. Routine post-biopsy ultrasound may not be necessary. Renal biopsies can be performed safely if risk factors are controlled, such as renal failure, hyperuremia, hyperphosphatemia, hemodialysis patients and a diagnosis of lupus nephropathy.

Desmopressin Acetate Before Percutaneous Ultrasound-Guided Kidney Biopsy in Patients with Renal Failure - Is it Really Beneficial?

The most common complication of percutaneous renal biopsy is bleeding, which can be seen in up to one-third of cases. The aim of this study was to evaluate the effect of prebiopsy administration of intranasal desmopressin acetate in reducing the incidence of biopsy-related bleeding complications in patients with significant renal dysfunction who underwent renal biopsy. This was a retrospective, observational study of percutaneous native renal biopsies performed at our center from July 2014 to June 2018. Bleeding complication rates of patients with renal failure (estimated glomerular filtration rate [eGFR] <30 mL/minute/1.73 m2) who received desmopressin and those who did not receive desmopressin were compared. Desmopressin administration before renal biopsy in patients with eGFR <30 mL/minute/1.73 m2 was associated with a significant reduction of bleeding complications (major and minor together; P = 0.025) and no significant reduction in major complications (P = 0.616) or intervention rates (P = 0.251) when compared with a group that did not receive desmopressin. While prebiopsy intranasal desmopressin use was associated with a significant reduction of overall bleeding complications including major and minor complications, there was no reduction in the rate of other major complications and interventions.

Paradigm Shift in the Management of Pseudoaneurysm Following Nephrolithotomy-A Case Series

Renal artery pseudoaneurysm after nephrolithotomy or pyelolithotomy is rare, but is more often following Percutaneous Nephrolithotomy (PCNL) or renal biopsy. It is a potentially life-threatening condition, with an incidence of 0.1 to 0.3% after nephrolithotomy or pyelolithotomy. Manifestation may vary, from asymptomatic to symptoms like local bloody discharge, haematuria, pain, high blood pressure, and rupture causing a life-threatening condition. A minimally invasive technique, selective or super-selective angioembolisation is the treatment of choice. This case series reports, five cases presented with either bloody wound discharge or intermittent haematuria in the emergency department, post nephrolithotomy. All patients were resuscitated in the emergency department with intravenous fluid and packed red blood cell transfusion. After resuscitation, three patients were diagnosed with renal artery pseudoaneurysm on Computed Tomography of Kidneys, Ureters and Bladder (CT KUB) and angiography, and two patients with digital subtraction angiography during the intervention. All were treated with super-selective renal artery angioembolisation, with endovascular coiling of different sizes. All patients made an uneventful recovery, and on followup no complication was evident. Now-a-days the popularity of angioembolisation for renal artery pseudoaneurysm has increased, which shows a paradigm shift from a traditional surgical intervention.

Retraction: Indications for percutaneous ultrasound-guided renal biopsy and complications associated with it: An observational study

Retraction: Indications for percutaneous ultrasound-guided renal biopsy and complications associated with it: An observational study

Native renal biopsy: outcomes with a 4-h observation period in low-risk outpatients.

Current practice guidelines recommend that 'low-risk' outpatients undergoing percutaneous native renal biopsy (PRB) are observed for 6-8 h to identify post-biopsy complications. We performed a retrospective review of 225 PRB procedures in low-risk outpatients who were observed for a 4-h period to determine the safety with regard to complication rate and timing. PRB was performed using a standardised protocol and under ultrasound guidance with a 16- or 18-gauge needle. Bleeding complications occurred in 7% (16/225) of patients, of which 88% (14/16) were detected within a 4-h period. The two undetected complications presented more than 72 h after the procedure. This suggests that a 4-h observation period may be safe and adequate in identifying the majority of patients who will experience significant complications in the first 24 h, with a potential saving of time and resources.

The Spectrum of Kidney Disease in Type Two Diabetic Patients: A Single-Center Study

Background: Diabetic nephropathy (DN) is the dominant reason for end-stage kidney disease linked with a rise in cardiovascular mortality rate. However, besides DN, type 2 diabetic patients may also suffer from various non-diabetic renal diseases (NDRD). Aim: The objective of the current research was to assess the occurrence and type of NDRD diagnosed by kidney biopsy in type 2 diabetic subjects, evaluate the association of various clinical and laboratory characteristics with histopathology findings, and identify essential predictors of NDRD. Methods: Retrospective analysis has been performed through medical record revision of 101 patients with type 2 diabetes undergoing percutaneous renal biopsy at Qilu Hospital of Shandong University (Jinan, China) between January 2015 and December 2020. Results: Renal biopsy results showed that NDRD was found in 59 patients (58.42%), while DN existed in 32 patients (31.68%) and 10 patients (9.90%) showed DN complicated with NDRD. Membranous nephropathy was prevailing NDRD (42%), followed by focal segmental glomerulosclerosis (11.6%) and IgA nephropathy (10.1%). In univariate analysis, patients with NDRD had older age (p < 0.018), a short duration of diabetes (p < 0.000), lower proteinuria (p < 0.030), and had higher hemoglobin levels (p < 0.006) compared to non-NDRD patients. In multivariate logistic regression analysis, the short course of diabetes (OR 0.986; 95% CI = 0.978 - 0.993; p = 0.000) and older age (OR 1.080; 95% CI = 1.028 - 1.134; p = 0.002) were significant risk factors for NDRD occurrence. In ROC analysis for NDRD, the duration of diabetes ≤ 78 months (cut-off value (0.725, 0.313)) illustrated the highest AUC. Conclusions: Clinical parameters such as short duration of diabetes, older age, higher hemoglobin level, and lower proteinuria might be associated with NDRD in type 2 diabetic patients. An early diagnosis of NDRD poses a favorable renal prognosis because it requires a different approach than DN, further larger multicenter randomized prospective investigations focused on identifying possible risk markers of NDRD are still in priority.

Veno-Arterial Index (VAI)—A Promising Tool For Identifying Renal Transplant Rejection

The objective of this study is to evaluate the veno-arterial index (VAI) as a predictor of renal rejection in kidney transplant patients. A diagnostic test study was conducted between January 2014 and May 2018. Renal transplant patients who underwent percutaneous renal biopsy were included. The VAI was obtained by measuring the flow velocity in the renal segmental vein and dividing this value by the peak systolic velocity of the segmental artery in this same location. The records of 77 patients were analyzed. Among these patients, 32 (42%) were positive for transplant rejection and 29 presented with acute rejection. In patients with renal rejection, the median VAI was 0.67 (interquartile range [IQR] = 0.56-0.87), and in kidneys with a negative biopsy for rejection, the median VAI was 0.41 (IQR = 0.27-0.57), with a statistically significant difference ( P = .007) and a value much higher than that obtained for the general population of 0.30 (IQR = 0.18-0.44). Subacute and acute rejected kidneys had an even higher VAI of 0.725 (IQR = 0.57-0.87; P = .0001). Although the resistive index has a good correlation with glomerular filtration, it is not possible by that index to differentiate among the multiple causes that can lead to graft dysfunction. The results of this study show that the VAI might be a useful parameter, which when elevated could predict renal transplant rejection.

Association Between Diabetes and Major Bleeding Complications of Renal Biopsy

IntroductionNephrologists have recently recognized the heterogeneity of kidney diseases among patients with diabetes and begun to actively perform percutaneous renal biopsies (PRBs). Nevertheless, the association between diabetes and major bleeding complications of PRB remains unclear.MethodsIn this retrospective cohort study using the Diagnosis Procedure Combination database in Japan, we identified patients who underwent an elective PRB from July 2010 to March 2018. The primary outcome was the occurrence of major bleeding complications, defined as red blood cell transfusion within 7 days after PRB or invasive hemostasis after PRB. Multivariable regression analysis was performed to analyze the association between diabetes and major bleeding complications with adjustment for patient and hospital characteristics.ResultsWe identified 76,302 patients, including 8245 with diabetes. The proportion of PRBs performed for patients with diabetes continuously increased over time. Major bleeding complications occurred in 678 patients (0.9%), including 622 (0.8%) with red blood cell (RBC) transfusion and 109 (0.1%) with invasive hemostasis. Diabetes was significantly associated with major bleeding complications (relative risk [RR] = 2.41; 95% CI 2.00–2.90). Among patients with diabetes, multiagent or insulin treatment had significant association with major bleeding complications (RR = 1.57; 95% CI 1.18–2.10), compared with single-agent diabetes treatment.ConclusionDiabetes is significantly associated with major bleeding complications of PRBs. Moreover, severity of diabetes has association with increases in major bleeding complications. Thus, nephrologists should carefully judge whether the anticipated benefits outweigh the relatively high risk of major bleeding complications when considering PRB for patients with diabetes.

Performing an Ultrasound-Guided Percutaneous Needle Kidney Biopsy: An Up-To-Date Procedural Review.

Ultrasound-guided percutaneous renal biopsy (PRB) has revolutionized the clinical practice of nephrology in the last decades. PRB remains an essential tool for the diagnosis, prognosis, and therapeutic management of several renal diseases and for the assessment of renal involvement in systemic diseases. In this study, we examine the different applications and provide a review of the current evidence on the periprocedural management of patients. PRB is recommended in patients with significant proteinuria, hematuria, acute kidney injury, unexpected worsening of renal function, and allograft dysfunction after excluding pre- and post-renal causes. A preliminary ultrasound examination is needed to assess the presence of anatomic anomalies of the kidney and to identify vessels that might be damaged by the needle during the procedure. Kidney biopsy is usually performed in the prone position on the lower pole of the left kidney, whereas in patients with obesity, the supine antero-lateral position is preferred. After preparing a sterile field and the injection of local anesthetics, an automatic spring-loaded biopsy gun is used under ultrasound guidance to obtain samples of renal parenchyma for histopathology. After the procedure, an ultrasound scan must be performed for the prompt identification of potential early bleeding complications. As 33% of complications occur after 8 h and 91% occur within 24 h, the ideal post-procedural observation time is 24 h. PRB is a safe procedure and should be considered a routine part of the clinical practice of nephrology.

Clinical outcomes in pediatric patients with normal renal histopathology.

BackgroundThere have been some cases where abnormal histopathologic findings could not be found in the kidney could even with proper specimen collection through percutaneous renal biopsy (PRB) in accordance with its indication. We analyzed the incidence and clinical outcomes of children who showed normal histopathological findings in their PRBs.MethodsThe medical records of 552 pediatric subjects who underwent PRB between 2005 and 2016 were reviewed. Twenty-six subjects were excluded because allograft biopsy was performed in nine subjects, and the age at biopsy was greater than 18 years in 17 subjects. Finally, 526 subjects were enrolled in this study.ResultsOf the 526 pediatric patients, 32 (6.1%) showed no histopathological abnormalities in their PRBs. The male-to-female ratio of the patients was 1.9:1, and the mean ages at the first visit and at biopsy were 10.6 ± 4.1 and 11.4 ± 3.8 years, respectively. In accordance with the biopsy indications, recurrent gross hematuria showed the highest incidence rate, but combined hematuria and proteinuria had the lowest incidence rate regarding normal renal histopathology among all the subjects. At a mean follow-up of 35.5 ± 23.6 months, urinary abnormalities had improved in more than 50% of the subjects with normal renal histopathology, and none of the patients showed progression to end-stage renal disease or required rebiopsy due to symptom worsening during the follow-up period.ConclusionThe clinical outcomes of children with normal PRB histopathologic findings are generally good. Further studies to evaluate their long-term outcomes are needed.

Role of Renal Biopsy in Managing Paediatric Renal Disease in a Tertiary Care Setting

Introduction: The contribution of renal biopsy is of major importance in many paediatrics renal diseases. This prospective study analysed the clinic-pathological spectrum of the biopsied cases, complications related to renal biopsy and its effect on management. Methods: USG guided percutaneous renal biopsy was performed in indicated children. Laboratory findings, complications of the procedure, histological diagnosis and its effect on management were obtained from all patients who underwent renal biopsy from April 2019 to March 2020. The data were finally analysed using descriptive statistics. Results: Total 50 cases were enrolled during the study period. There were 24 males and 26 females with age range from two to 16 years. The common indications for biopsy were acute nephritic syndrome (25 cases), including both primary glomerulonephritis (11 cases) and secondary glomerulonephritis (14 cases), of which lupus nephritis contributed (11/25) cases, followed by atypical/ steroid resistant or dependent nephrotic syndrome (18 cases). Among primary glomerulonephritis, diffuse proliferative glomerulonephritis was most common pathological finding (11/25). One had crescentric glomerulonephritis. Lupus nephritis predominantly had Class IV/V pathology (6/11). Of 18 nephrotic syndrome cases, nine had minimal-change-disease and five had focal-segmental-glomerulosclerosis. The complications associated with procedure were self-resolving gross hematuria in eight cases, perirenal hematoma requiring plasma transfusion in one case. Adequate renal tissue sample was obtained in 47 of the renal biopsies. Three cases underwent repeat biopsy. Immunosuppressive therapy was altered as per the biopsy report among 18 cases. Conclusions: Our study showed that renal biopsy is a safe, reliable and effective technique in children. It resolutes many diagnostic dilemma and helps in effective management.

Predictors of perirenal haematoma post-percutaneous ultrasound-guided renal biopsy.

ObjectiveTo perform a prospective study to determine the risk factors associated with perirenal haematoma development after percutaneous renal biopsy (PRB).MethodsThis multivariate prospective study collected demographic and clinical data from all consecutive adult patients that underwent real-time ultrasound-guided PRB of native kidneys. All biopsies were performed by two well-trained ultrasound physicians using 16G biopsy needles. Routine renal ultrasounds were performed within 12–24 h after biopsies in order to observe post-biopsy perirenal haematoma formation. Patients were stratified based on the occurrence of post-biopsy haematoma development.ResultsThis prospective study enrolled 218 patients and stratified them into a haematoma group (n = 126) and a non-haematoma group (n = 92). Binary logistic regression analysis identified female patients (odds ratio [OR] 1.990; 95% confidence interval [CI] 1.125, 3.521), patients with a body mass index (BMI) ≥28 kg/m2 (OR 2.660; 95% CI 1.097, 6.449) and patients with immediate post-biopsy active bleeding (IPAB) (OR 2.572; 95% CI 1.422, 4.655) as being more likely to have perirenal haematoma after real-time ultrasound guided PRB of native kidneys.ConclusionFemale sex, a BMI ≥28 kg/m2 and IPAB were risk factors for perirenal haematoma after real-time ultrasound-guided PRB of native kidneys.

Rare presentation of primary hepatic marginal zone B-cell lymphoma and membranous glomerulopathy in a 3-year-old boy

Abstract Introduction/Objective Primary hepatic marginal zone B-cell lymphoma (MZL) is a rare entity that has been described with chronic inflammatory disorders such as chronic viral hepatitides B and C and primary biliary cholangitis. We report MZL in a 3-year-old African-American boy that was discovered incidentally at autopsy. Methods/Case Report The patient first came to medical attention at 9-months of age with bilateral periorbital edema that progressed to generalized edema of the[PL1] face, extremities, and abdomen. Laboratory testing showed nephrotic syndrome with massive proteinuria (50-60 g/day). A percutaneous renal biopsy specimen showed membranous glomerulopathy with PLA2R detected by immunofluorescence, and granular IgG deposition along tubular basement membranes. Serum was negative for anti-PLA2R antibodies. The patient suffered recurrent upper respiratory tract infections requiring several intensive care hospitalizations. At 3 years of age, he was transported unresponsive and pulseless to the emergency department where he was pronounced dead. A brother with the infantile nephrotic syndrome had died from sepsis at 2 years of age. Suspicion of abuse led to an autopsy by the medical examiner, who discovered nodular masses in the right and left liver lobes, which histologically showed a serpiginous infiltrate of small B-lymphocytes positive for CD20, CD43 admixed with plasmacytoid cells with kappa light chain restriction. There were no extrahepatic tumors. Transmission electron microscopy of glomeruli showed membranous glomerulopathy. Kidneys showed dense reactive interstitial lymphocytic infiltrate with germinal centers Results (if a Case Study enter NA) NA Conclusion To our knowledge, this is the first reported case of MZL presenting in a pediatric patient with membranous glomerulopathy. Both diagnoses are associated with autoimmune diseases and persistent, chronic inflammation. Recurrent infections in this patient suggest an immunoregulatory disorder, setting the stage for chronic inflammation, as seen in the kidney, with progression to MZL in the liver.