Nephrotic Range Research Articles

Nephrotic Range of Proteinuria in Congenital Cyanotic Heart Disease: A Rare Complication

Nephrotic syndrome is a common renal problem in children. But it is an uncommon complication for cyanotic heart diseases. It has been rarely reported in pediatric population with congenital cyanotic heart disease in Bangladesh. We found two cases of congenital cyanotic heart disease those were referred to investigate for nephrotic range of proteinuria.
 Bangladesh J Child Health 2020; VOL 44 (3) :178-180

BDUMP: A Rare Case of Vision Loss in a Lung Transplant Recipient

Introduction Bilateral diffuse uveal melanocytic proliferation (BDUMP) is a potentially devastating rare paraneoplastic syndrome often associated with severe vision loss occurring in patients with underlying carcinoma. In approximately 50% of cases this may be the first sign of an underlying primary malignancy. First described in 1966 there are currently less then 100 cases described in the literature. Treatment of this condition includes treatment of the primary malignancy and plasmapheresis. We describe what we believe to be the first case reported in a lung transplant recipient. Case Report A 72 year old woman status post bilateral lung transplant for COPD was referred to ophthalmology due to increased vision loss for four months, one month prior to presentation she had undergone a left YAG capsulotomy. Maintenance immunosuppression included tacrolimus, mycophenolate mofetil, and prednisone. Fundus exam was remarkable for bilateral retinal pigment epithelium mottling and leopard spot fundus. Exam on the left showed edema. BDUMP was diagnosed, she was admitted to the hospital for plasmapheresis and an extensive evaluation for underlying malignancy. Remarkable laboratory studies showed a new acute kidney injury in the setting of chronic kidney disease with nephrotic range proteinuria. All tumor markers, ANA, ANCA, SPEP, and PLA2R were negative. CT of the head, chest, abdomen, and pelvis were unrevealing, PET scan showed a hypermetabolic breast nodule which was biopsied. The biopsy was consistent with ductal carcinoma in situ, and her mycophenolate was stopped. She completed 14 sessions of plasmapheresis and had improvement in her vision. Summary BDUMP, a rare condition which left untreated can lead to rapid vision loss, is associated with carcinomas of the cervix, uterus, ovary, lung, kidney, pancreas, breast, gastrointestinal tract, and Hodgkin lymphoma. While the exact pathogenesis for BDUMP has not yet been described it has been shown that these patients have a factor in the IgG fraction of serum that selectively causes melanocyte proliferation and thus plasmapheresis is an effective treatment. Patients that are immune suppressed are at an elevated risk for cancer, therefore it's highly important to keep up with health maintenance examinations in this population. Visual changes after transplant are also common, and a complete ophthalmologic examination should be completed with new onset of symptoms.

Serum IgA/C3 ratio and glomerular C3 staining predict progression of IgA nephropathy in children.

BackgroundThis retrospective study aimed to evaluate the significance of serum immunoglobulin A/complement 3 (IgA/C3) ratio and glomerular C3 staining at the onset of disease for predicting progression of IgA nephropathy in children.MethodsA total of 41 children with IgA nephropathy were allocated to two groups according to proteinuria (proteinuria <50 mg/kg/day group and proteinuria ≥50 mg/kg/day group) to compare their clinical data. Receiver operating characteristic (ROC) curves were used to evaluate the optimal cutoff value of serum IgA/C3 ratio in two groups. According to the optimal cutoff value of serum IgA/C3 ratio and glomerular C3 staining, the children were divided into four groups: Group A (serum IgA/C3 ratio <2.025 and glomerular C3 staining <2.0); Group B (serum IgA/C3 ratio ≥2.025 and glomerular C3 staining <2.0); Group C (serum IgA/C3 ratio <2.025 and glomerular C3 staining ≥2.0); and Group D (serum IgA/C3 ratio ≥2.025 and glomerular C3 staining ≥2.0). Then, the risk factors [including proteinuria and glomerular filtration rate (GFR) and pathological findings] were compared in these 4 groups at onset of IgA nephropathy.ResultsSerum IgA/C3 ratio in the proteinuria <50 mg/kg/day group was significantly higher compared to the proteinuria ≥50 mg/kg/day group (P<0.01). According to ROC curves, the optimal cutoff value for the IgA/C3 ratio was 2.025 in two groups. At onset of IgA nephropathy, patients with IgA/C3 ratio <2.025 were predicted with nephrotic range proteinuria. When glomerular C3 staining was at the same level (glomerular C3 staining <2.0), GFR was significantly lower in group B (serum IgA/C3 ratio ≥2.025) compared with group A (serum IgA/C3 ratio <2.025). When serum IgA/C3 ratio was at the same level (serum IgA/C3 ratio <2.025), GFR was significantly lower in group C (glomerular C3 staining ≥2.0) compared with group A (glomerular C3 staining <2.0). Pathological findings and MEST (Oxford classification of IgA nephropathy) scores did not differ among the 4 groups at onset of the disease.ConclusionsSerum IgA/C3 ratio and glomerular C3 staining may be useful markers of the progression of IgA nephropathy in children, but not good markers for pathological findings at the onset of disease.

Association between albuminuria and thyroid function in patients with chronic kidney disease

PurposeThe relationship between proteinuria and thyroid function remains controversial in patients with chronic kidney disease (CKD). We prospectively investigated the association between kidney and thyroid function in thyroid antibody-negative patients through all CKD stages.MethodsWe enrolled 184 nondialysis patients (mean age: 63.1 ± 16.9 years) without previous thyroid disease or thyroid-specific antibodies. Kidney function was assessed by estimating the glomerular filtration rate (eGFR) classified according KDIGO (CKD G1–5). Kidney damage was assessed by albuminuria (albumin-to-creatinine ratio, ACR) and classified as mild, moderate, or severe (ACR1: <300, ACR2: 300–3000, and ACR3: 3000 mg/g). To evaluate thyroid function, TSH, T4, fT4, T3, fT3, reverse T3 (rT3), and thyroxine-binding globulin (TBG) were measured.ResultsrT3 concentrations correlated negatively with albuminuria (r = −0.286, p < 0.001) and were significantly lower in patients with severe albuminuria than in those with mild or moderate albuminuria (ACR3: 0.28 vs. ACR2: 0.32 vs. ACR1: 0.36 nmol/l, p < 0.001). The severity of albuminuria revealed no impact on TSH, fT4, T3, fT3, and TBG. EGFR correlated with increasing T4, fT4, T3, fT3, and TBG (T4: r = 0.289, p < 0.01; fT4: r = 0.196, p < 0.01; T3: r = 0.408, p < 0.01; fT3: r = 0.390, p < 0.01) but not with rT3.ConclusionsIn thyroid antibody-negative patients presenting advanced CKD (stages 4 and 5), even severe kidney protein loss failed to influence thyroid hormone status. However, albuminuria severity correlated negatively with rT3, which was significantly lower in patients with albuminuria in the nephrotic range.

Nephrotic range proteinuria in an adolescent with a diagnosis of Wilson's disease: Answers.

Nephrotic range proteinuria in an adolescent with a diagnosis of Wilson's disease: Answers.

Nephrotic range proteinuria in an adolescent with a diagnosis of Wilson's disease: Questions.

Nephrotic range proteinuria in an adolescent with a diagnosis of Wilson's disease: Questions.

Retrospective analysis of clinical and pathomorphological features of lupus nephritis in children

PurposeThe aim of the study was to evaluate the clinical course and pathomorphological correlations in Polish children with the diagnosis of lupus nephritis (LN). MethodsWe retrospectively analyzed the medical records of 39 children hospitalized due to LN in 7 pediatric nephrology units in Poland between 2010 and 2019. Demographic data, clinical symptoms at the onset of LN and laboratory parameters were reviewed. We analyzed Systemic Lupus Erythematosus Disease Activity Index (SLEDAI), histological LN findings with the activity (IA) and chronicity index (IC). ResultsWe examined 32 girls and 7 boys, median age at LN onset was 14.75 (IQR 13.0–16.0) years, SLEDAI of 22.0 (IQR 18.0–27.0) points; LN histological class: IV (59.4%), III (18.9%), III/V (10.8%), IV/V (8.1%), VI (2.7%); IA 8.0 (IQR 6.0–11.0) points, IC 1.05 (IQR 0–2.0) points.Children with nephrotic (n ​= ​22) and non-nephrotic (n ​= ​17) proteinuria differed in median Hb level (9.55, IQR 8.3–11.2 vs 10.9, IQR 10.1–11.6 ​g/L; P ​< ​0.05), albumin level (2.5, IQR 2.1–3.19 vs 3.6, IQR 3.4–4.1 ​g/dL; P ​< ​0.001), proteinuria (5.76, IQR 3.0–7.5 vs 1.08, IQR 0.53–1.50 ​g/day; P ​< ​0.0001), eGFR (53.9, IQR 27.0–68.8 vs 96.7, IQR 73.8–106.2 ​mL/min/1.73 ​m2; P ​< ​0.01) and occurrence of hypertension (77% vs 23%; P ​< ​0.01).In multivariate analysis Hb level (β ​= ​8.0; 95%CI, 1.90–14.11) was the significant predictor of eGFR<90 ​mL/min/1.73 ​m2. ConclusionsProliferative forms of LN in children may have a varying clinical presentation.Children with LN with nephrotic range proteinuria have lower Hb level, lower eGFR and higher occurrence of hypertension. Hb level is the significant predictor of eGFR<90 ​mL/min/1.73 ​m2 in children with LN.

Heterogeneity in patterns of progression of chronic kidney disease.

Progression of kidney disease is a deceptively simple word for a complex bio-clinical process, evidenced by the number of definitions in the literature. This has led to confusion and differences in interpretation of studies. We describe different patterns of progression, the performance of different definitions of progression and factors associated with chronic kidney disease (CKD) progression in a public renal service in Australia, in a study of patients enrolled in the CKD.QLD Registry with a minimum of 2 years' follow up. Nine patterns of changing estimated glomerular filtration rate (eGFR) over two consecutive 12-month periods were identified. Most common was a stable eGFR over 2 years (30%), and the least was a sustainable improvement of eGFR over both periods (2.1%). There was a lack of congruence between the several definitions of progression of CKD evaluated. More people progressed using the definition of decline of eGFR of >5 mL/min/1.73 m2 /year (year 1 = 30.2%, year 2 = 20.7%) and the least using development of end-stage renal disease (year 1 = 5.4%, year 2 = 9.9%). Age (40-59, ≥80 years), degree of proteinuria at baseline (nephrotic range) and CKD aetiology (renal vascular disease, diabetic nephropathy) were significantly associated with eGFR decline over 2 years. This is one of the first demonstrations of the great variations among and within individuals in the progression of CKD over even a period as short as 2 years. Findings suggest considerable potential for renal function recovery and stability while demonstrating the importance of using identical definitions for comparisons across datasets from different sources.

A case of dasatinib-induced focal segmental glomerulosclerosis in a patient with Philadelphia chromosome positive chronic myeloid leukemia

Focal segmental glomerulosclerosis is a common glomerular histological lesion, which is usually characterised by non-nephrotic range proteinuria or nephrotic syndrome. It may be idiopathic or occurs secondarily to drugs, diabetes, obesity or HIV nephropathy and other infections. Dasatinib, a tyrosine kinase inhibitor that has been used for the treatment of Philadelphia chromosome-positive chronic myeloid leukemia, has a few renal adverse effects. Exceptional cases with non-nephrotic range proteinuria have been reported in relation with dasatinib. In this case, we report a patient with symptoms of nephrotic syndrome and nephrotic range proteinuria, who was diagnosed as focal segmental glomerulosclerosis by kidney biopsy after treated with dasatinib.

Glomerular involvement in children with H syndrome.

H syndrome is a multisystem inflammatory disease caused by mutations in the SLC29A3 gene (OMIM #602782). The protein product, hENT3, is a nucleoside transporter essential for DNA salvage synthesis. Clinical manifestations are hyperpigmentation, hypertrichosis, hepatosplenomegaly, hearing loss, heart anomalies, hypogonadism, short stature, skeletal deformities, and diabetes mellitus. Laboratory findings are consistent with inflammatory processes. Structural kidney anomalies have been described in 6% of patients. Three family members with genetically diagnosed H syndrome (c.1279G>A, p.Gly427Ser). Two of them presented with hypoalbuminemia and nephrotic range proteinuria. Kidney ultrasound was normal. Kidney biopsy performed in one patient presenting with generalized peripheral pitting edema revealed membranous nephropathy. Different treatments including ACE inhibitors, corticosteroids, and immunomodulatory agents failed to improve the clinical outcome. Generalized peripheral pitting edema and glomerulopathy broaden the clinical spectrum of H syndrome. Periodic bloodwork and urinalysis are recommended.

Clinical and laboratory features that differentiate familial from sporadic focal segmental glomerulosclerosis in adolescents and adults

Background: Focal segmental glomerulosclerosis (FSGS) is an important cause of end-stage kidney disease in children and adults. Although most cases are sporadic (s), familial (f) presentation is also described. The purpose of the present study was to establish clinical and laboratory profiles of fFSGS vs. primary sFSGS, contributing to the distinguishing diagnosis in clinical practice and best management, in particular when mutation analysis is not available. Methods: Demographic, clinical and laboratorial parameters were studied in 124 patients 12 years and older with FSGS, subdivided in sFSGS (n=89) and fFSGS (n=35). Results: General and clinical features were similar, as well as serum creatinine at disease presentation. Proteinuria levels were more frequently ≥ 3g/day in sFSGS (63.8%) than in fFSGS (44%, p=0.080), and serum albumin levels were &lt; 3.0 g/dL in 45.8% and 20%, respectively (p=0.046). The groups were statistically different regarding steroid resistance, corresponding to 60% in sFSGS and 100% in fFSGS (p=0.001). Conclusions: The studied groups were clinically similar, except that proteinuria tended to be higher (nephrotic range) and serum albumin was lower in sFSGS vs. fFSGS. In addition, all treated fFSGS patients were steroid resistant. At presentation it is important to characterize if the patient has fFSGS, that will contribute to further disease management, and disease history will be the first clue for such differential diagnosis.

Rituximab Treatment of Membranous Nephropathy after Failure of other Therapies- A Case report

Membranous Nephropathy (MN) also known as membranous glomerulonephritis is a slowly progressive kidney disease characterized of subepithelial immune complex deposits and subsequent thickening of glomerular basement membrane. MN is a common cause of proteinuria that may progress to ERDS. The decision to treat MN with immunosuppressive medications is complicated by the well-known natural history of disease and it depends on patient presentation and disease progression. Herein, we present a case of a 48-year-old male patient with weight gain, lower extremity edema, arterial hypertension, and nephrotic range proteinuria (13.5 gr).

Spot Urine Protein-to-Creatinine Ratio Compared with Dipstick Proteinuria as a Primary Screening Tool for Renal Disease in a Community Setting.

The Kidney Disease: Improving Global Outcomes (KDIGO) advocates the use of dipstick proteinuria as a primary screening tool. However, the performance of dipstick urinary for proteinuria has been adjudged to be weak, unreliable with poor predictive value. This study aimed to determine and compare significant proteinuria (SP) using spot urinary protein-to-creatinine ratio (UPr/UCr) as a primary screening tools with dipstick proteinuria among the high-risk African children. This study was a cross-sectional study, involving 33 schools in Ogbomoso land, Southwestern Nigeria. A total of 1316 apparently healthy children were recruited through a multistage sampling technique from both rural and urban communities using a semi-structured questionnaire. Dipstick urinalysis was performed on early morning urine samples. Urinary protein was determined using a turbidimetric method, while Jaffe's reaction was used to measure urinary creatinine concentration. Statistical analysis was performed using IBM SPSS Statistics version 23.0 for Windows. The prevalence of SP using spot UPr/UCr (≥0.2) and dipstick proteinuria (≥1+) was 18.0% and 0.8%, respectively (P <0.001). Of the 224 subjects with SP using UPr/UCr, the females (140; 20.1%) had higher proportion of SP compared to males (84; 15.4% - P = 0.032). Nephrotic range proteinuria was detected in 9/10 (90%) using UPr/UCr, while urinary dipstick method identified only 3/10 (30%). A biserial correlation coefficient (r = 0.092; P =0.001) and inter-rater agreement (Cohen's Kappa = 0.01) were poor and McNemar's test was P<0.0001. In the community, UPr/UCr ratio appeared to perform better than dipstick as a primary screening tool for renal disease and may be adopted in the early detection of SP as a marker of kidney disease against the current KDIGO guideline of dipstick proteinuria.

Clinical Presentation, Histology, and Outcomes of Immunoglobulin A Nephropathy: A Single-Center Experience from Pakistan.

Clinical presentation, kidney biopsy findings, and clinical outcomes of immunoglobulin A nephropathy (IgAN) are highly variable. The objective of this study is to study the clinical presentation, histologic patterns, and outcomes of IgAN in the Pakistani population, as no significant data are available in international literature from this part of the world. A retrospective chart review was conducted of all patients with biopsy-proven IgAN between January 2007 and December 2017. Of a total of 977 renal biopsies, 50 patients had biopsy-proven IgAN (5.1%). The median age at the time of biopsy was 34 years (27-42); 92% of patients were between 18 and 40 years. Thirty-eight (76%) were male. Ninety-two percent of patients had significant proteinuria of >1 g/day, with 32% having nephrotic range proteinuria. The mean estimated glomerular filtration rate (eGFR) at presentation was 46.58 mL/min/1.73 m2. Seventy-eight percent of patients were hypertensive at the time of presentation and most of them had uncontrolled hypertension (HTN). The most common lesion on light microscopy was focal necrotizing glomerulonephritis (26%), followed by mesangial expansion with segmental/global glomerulosclerosis (22%). Crescents were seen in 38% of cases. Of 50 patients, a follow-up of at least six months was available for 32 patients. Most of the patients who had an eGFR of <30 mL/min at presentation progressed to kidney failure at six-month follow-up period. IgAN usually presents in young male adults in the age range of 18-40 years, with most patients having severe clinical presentation characterized by nephrotic-range proteinuria, HTN, renal insufficiency, and severe histological stages.

Interpretation of 24-Hour Urinary Protein Level for Diagnosis of Nephrotic Syndrome in Marked Hypoalbuminemia

Nephrotic syndrome is a common renal problem in childhood and is characterised by generalised oedema, massive proteinuria, hypoalbuminemia and hyperlipidemia. There are various laboratory methods to quantify proteinuria. Among them 24-hour urinary protein estimation is considered a gold standard for diagnosis of nephrotic syndrome. Nephrotic range proteinuria is considered when 24-hour urinary protein is more than 40 mg/m2/hr. There is scarce literature available regarding the changes in quantitative proteinuria when there is marked hypoalbuminemia (serum albumin less than 2.5 gm/dL). This series is about three patients of nephrotic syndrome (6 yers old male, 4 years old male and 5 years old male), having marked hypoalbuminemia and their 24-hour urinary protein level resulted into non-nephrotic range. All the patients underwent relevant physical, clinical examinations and laboratory blood and urine investigations(Haemoglobin, Mantoux test, chest x-ray, urine routine, urine culture and sensitivity, lipid profile, serum albumin and 24 hour urinary protein). All the cases were managed with Prednisolone and diuretics like Furosemide and were followed up till the subside of proteinuria and oedema conditions.

The Evaluation of the Children with Renal Transplant: Single Centre Experience

Objective: This study aimed to evaluate patients with renal transplantation in terms of clinical and laboratory. parameters. Material and Methods: This study was performed retrospectively with records of 48 patients who underwent renal transplantation before 18 years of age, between June 2008 and July 2019. Results: Congenital malformations of the urinary tract were the most common underlying causes of chronic kidney disease stage 5. Surgical complications occurred in 33.4% of the patients and BK viremia was the most common opportunistic viral infection during the follow-up. At the last clinic visit, 57.4% of our patients had CKD stage 1, hypertension and nephrotic range proteinuria were seen in eight and two patients, respectively. Conclusion: Although renal transplantation is the most ideal renal replacement therapy, patients may experience various complications during the follow-up. Therefore, they should be monitored regularly

Secondary renal amyloidosis complicating psoriatic arthropathy

Introduction: Secondary AA amyloidosis is a classical complication of chronic inflammatory conditions like rheumatoid arthritis, ankylosing spondylitis and inflammatory bowel disease or chronic infectious diseases like tuberculosis, bronchiectasis and chronic osteomyelitis. Amyloidosis is an extremely rare complication of psoriatic arthritisand is associated with a poor prognosis.1-3 Materials and Methods: We report a case of a 42 year old male, a known case of psoriasis since 1 year and on methotrexate 15mg /day since 6 month in view of psoriatic arthropathy. Laboratory findings revealed urinary spot protein in the nephrotic range along with hyperlipidemia, hypoproteinemia and albuminuria suggestive of nephrotic syndrome. USG guided renal biopsy revealed renal amyloidosis with congo red positivity showing apple green birefringence. Patient expired within 48 hours after renal biopsy due to pneumonia. Complete autopsy was performed and histopathology showed amyloidosis restricted to the kidneys. Result: USG guided renal biopsy revealed renal amyloidosis with congo red positivity showing apple green birefringence. Patient expired within 48 hours after renal biopsy due to pneumonia. Complete autopsy was performed and histopathology showed amyloidosis restricted to the kidneys. Conclusion: Amyloidosis should be considered in patients with long-standing severe psoriasis and psoriatic arthropathy who develop renal symptoms and there can be rapid onset of amyloidosis after the first articular signs. Prognosis is usually poor. Keywords: Psoriatic nephropathy, Renal amyloid, Secondary amyloidosis.

Renal disease associated with myeloproliferative neoplasms and myelodysplastic syndrome/myeloproliferative neoplasms.

Renal changes in patients with myeloproliferative neoplasms (MPNs) or myelodysplastic syndrome (MDS)/MPNs have been addressed by few, respectively no, reports. The aim of this study was to focus on a systematic evaluation of renal biopsies in patients with MPNs or MDS/MPNs. The cohort comprised 29 patients (23 men) aged 67±11years (mean±standard deviation), diagnosed with chronic myeloid leukaemia (n=5), polycythaemia vera (n=9), primary myelofibrosis (n=5), essential thrombocythaemia (n=2), or chronic myelomonocytic leukaemia (n=4), as well as MPNs or MDS/MPNs not otherwise specified (n=4). Patients manifested with proteinuria (93%), partially in the nephrotic range (46%), haematuria (72%), and impaired kidney function (93%). The most prominent histological findings included double-contoured glomerular basement membranes (71%), acute endothelial damage (68%), intracapillary platelet aggregation (62%), mesangiolysis (21%), thrombotic microangiopathy (24%), segmental glomerulosclerosis (66%), mesangial hypercellularity and sclerosis, extramedullary haematopoiesis (17%), and also IgA nephropathy (21%) and glomerulonephritis (GN) with features of infection-related GN (21%). MPN and MDS/MPN patients showed significantly more chronic changes than age-matched and sex-matched controls, including global and segmental glomerulosclerosis, mesangial sclerosis, and hypercellularity, whereas the extent of arteriosclerosis was comparable. MPN and MDS/MPN patients show glomerular scarring that exceeds age-related phenomena. Ongoing endothelial damage, growth factors released by platelets and deposition of immune complexes are probably the causative mechanisms. Early recognition of renal failure heralded by proteinuria and haematuria, and consequent control of risk factors for kidney failure, should be recommended for MPN and MDS/MPN patients.

Nephrotic range proteinuria associated with focal segmental glomerulosclerosis reversed with pioglitazone therapy in a patient with Dunnigan type lipodystrophy

Familial partiallipodystrophy (FPLD)is a rare disorder associated withsevere insulin resistance, hypertriglyceridemia, lowserumHDLcholesterol and proteinuricrenaldisease. Although proteinuric renal disease is not common among in patients with partial lipodystrophy, we report a patient with Dunnigan type FPLD complicated by nephrotic syndrome which resolved following treatment with thePPARγagonist pioglitazone, CPAP, diet, and exercise.

Monoclonal gammopathy of renal significance; experience of a tertiary care hospital in India

Introduction: Monoclonal gammopathy of renal significance (MGRS) disorders are indolent B-cell or plasma cell lymphoproliferative neoplasms which do not meet the hematological criteria for malignancy, however they cause renal dysfunction as a result of production of nephrotoxic monoclonal immunoglobulin (MIg). Objectives: To study the clinical presentation, laboratory features, light microscopy and immunofluorescence (IF) characteristics of all cases of MGRS diagnosed at our hospital over a period of five years. Patients and Methods: A record of all renal biopsies performed at our hospital between 2014-2019 was accessed from the database. Out of 1356 kidney biopsies, 68 had evidence of MIg deposition on immunofluorescence. Only six cases met the criteria of MGRS. Histopathological and immunofluorescence characteristics were studied to classify the lesions as per International Kidney and Monoclonal Gammopathy (IKMG) Research Group classification. Results: All six cases presented with deranged renal function. Four had sub-nephrotic and one had nephrotic range proteinuria. MIg was identified in only one case on serum protein electrophoresis and free light chain assay. Using a conjunction of histomorphology of renal lesions, special stains and immunofluorescence all six cases of MGRS were categorized as per IKMG classification into monoclonal immunoglobulin deposition disease (two cases), AL amyloidosis, light chain cast nephropathy, proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID) and light chain proximal tubulopathy (LCPT). Conclusion: MGRS presents as renal failure and proteinuria. MIg may not be detected on protein electrophoresis due to low-secretion in serum. A kidney biopsy is essential to study the morphology of renal lesions and identify MIg deposition.