MO305NEPHROTIC SYNDROME AND NEPHROTIC-RANGE PROTEINURIA - CLINICOPATHOLOGICAL DIAGNOSES AND PATIENT CHARACTERISTICS IN A MULTIETHNIC SINGLE CENTRE POPULATION

2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
Selene T Y Teoh ◽  
Su Mein Goh ◽  
Eileen L K Pang ◽  
See Cheng Yeo
Keyword(s):  
Diabetes Mellitus ◽  
Renal Function ◽  
Nephrotic Syndrome ◽  
Serum Albumin ◽  
Renal Biopsy ◽  
Patient Characteristics ◽  
Glomerular Disease ◽  
Significant Difference ◽  
Nephrotic Range Proteinuria ◽  
Abnormal Renal Function

Abstract Background and Aims Nephrotic syndrome (NS) is a common indication for renal biopsy, and a frequent presenting clinical syndrome for certain glomerulonephritis (GN), particularly minimal change disease (MCD), focal segmental glomerulosclerosis (FSGS) or membranous nephropathy (MN). However, less is known on the spectrum of clinicopathological diagnosis and characteristics in patients with nephrotic-range proteinuria without nephrotic syndrome (NRP). We aim to evaluate the differences in clinicopathological diagnosis and characteristics of patients with NS versus NRP. Method All patients who underwent a renal biopsy from the 1st January 2014 to 31st December 2016 were included in the study. Patients with no urine protein quantification, serum albumin or lipid panel done at the time of renal biopsy or no glomeruli seen on renal histology were excluded. Data for renal histopathology, clinical diagnosis and patient characteristics were obtained from review of medical records. Results A total of 362 patients underwent a renal biopsy from the 1st January 2014 to 31st December 2016. Of these, 76 patients had NS and 106 patients had NRP. Mean age was 51.1±17.0, 51.5% were male and 70.9% were Chinese. In the NS group, there were 25 (32.9%) MCD, 10 (13.2%) FSGS of which 8 were primary, 14 (18.4%) lupus nephritis (LN), 11 (14.5%) MN, 8 (10.5%) diabetic glomerulosclerosis, 2 (2.6%) Immunoglobulin A nephropathy (IgAN), 2 (2.6%) amyloidosis and 4 (5.3%) with other diagnoses (2 membranoproliferative GN, 1 acute interstitial nephritis, 1 chronic glomerulosclerosis). In the NRP group, there were 26 (24.5%) diabetic nephropathy, 21 (19.8%) IgAN, 15 (14.2%) LN, 13 (12.3%) FSGS of which 12 were secondary FSGS, 12 (11.3%) chronic glomerulosclerosis, 8 (7.5%) MN, 4 (3.8%) pauci-immune GN and 7 (6.6%) with other diagnosis (2 infection-related glomerulonephritis, 3 had mild chronic changes with proteinuria attributable to overflow proteinuria from myeloma, 1 Alport syndrome, 1 thrombotic microangiopathy). There were significantly more cases of primary GN in the NS group (63.2% versus 28.3%, p<0.001), while the NRP group had more secondary GN and non-GN cases. The most common cause of primary GN is MCD in the NS group and IgAN in the NRP group. LN is the most common secondary GN in both NS and NRP groups. There was no significant difference in age, gender and race between the NS and NRP group. The NS group had a significantly higher proteinuria (10.8±5.5 versus 6.3±3.5, p<0.001) and lower serum albumin (17.0±5.7 versus 28.0±7.6, p<0.001). The NRP group had significantly more patients with abnormal renal function at the time of biopsy (83 (78.30%) versus 39 (51.32%) patients, p<0.001), a higher mean systolic BP (144.1±26.3mmHg versus 134.2±26.2mmHg, p=0.014) and a higher percentage of diabetes mellitus (38 (35.85%) versus 15 (19.74%) patients, p=0.018) compared to the NS group. Conclusion The clinicopathological diagnosis between patients with NS and NRP differ significantly. Patients with NRP are more likely to have secondary glomerular disease or non-glomerular disease, with abnormal renal function and higher prevalence of hypertension and diabetes mellitus.

scholarly journals Effect of Protein A Immunoadsorption in Nephrotic Syndrome of Various Etiologies

1999 ◽  
Vol 10 (9) ◽  
pp. 2014-2017
Author(s):  
VINCENT L. M. ESNAULT ◽  
DOMINIQUE BESNIER ◽  
ANGELO TESTA ◽  
PIERRE COVILLE ◽  
PIERRE SIMON ◽  
...  
Keyword(s):  
Diabetes Mellitus ◽  
Nephrotic Syndrome ◽  
Serum Albumin ◽  
Antihypertensive Drugs ◽  
Immunosuppressive Treatment ◽  
Protein A ◽  
Short Term ◽  
Focal And Segmental Glomerulosclerosis ◽  
Albumin Infusion ◽  
Baseline Levels

Abstract. Protein A immunoadsorption (IA) has proved effective in reducing proteinuria in patients with nephrotic syndrome after recurrence of focal and segmental glomerulosclerosis (FSGS) in kidney transplants. The effect of IA in nephrotic syndrome of other etiologies remains unknown. Nine patients with nephrotic syndrome secondary to membranous nephropathy (four cases), diabetes mellitus (one case), IgA nephropathy (two cases), and amyloidosis (two cases) had three to five IA of 2.5 plasma volumes over 4 to 8 d. Patients received no concomitant immunosuppressive treatment, and antihypertensive drugs were left unchanged. Proteinuria decreased from 12.64 ± 5.49 to 3.35 ± 2.2 g/24 h (mean ± SD) in all patients after three to five IA. Hematocrit decreased from 37.32 to 32.64% (12.5% hemodilution) and serum albumin from 25.43 to 18.6 g/L (26.4% decrease). Proteinuria returned to baseline levels within 1 mo, as described in recurrent FSGS following transplantation. When serum albumin balance was controlled by albumin infusion after IA in two patients, comparable decreases in proteinuria were observed. Therefore, IA is effective in producing short-term reduction of proteinuria in nephrotic syndromes related not only to FSGS but also to membranous and IgA nephropathies, diabetes mellitus, and amyloidosis, which suggests that IA removes a nonspecific circulating hemodynamic-altering or permeability-increasing factor.

scholarly journals Horseshoe kidney with PLA2R-positive membranous nephropathy

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Shuai-Shuai Shi ◽  
Xian-Zu Yang ◽  
Xiao-Ye Zhang ◽  
Hui-Dan Guo ◽  
Wen-Feng Wang ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Renal Biopsy ◽  
Membranous Nephropathy ◽  
Horseshoe Kidney ◽  
Glomerular Disease ◽  
Urinary Stones ◽  
Abdominal Ultrasonography ◽  
Glomerular Diseases ◽  
Structural Abnormalities ◽  
Diagnosis Rate

Abstract Background Horseshoe kidney (HSK) is a common congenital defect of the urinary system. The most common complications are urinary tract infection, urinary stones, and hydronephrosis. HSK can be combined with glomerular diseases, but the diagnosis rate of renal biopsy is low due to structural abnormalities. There are only a few reports on HSK with glomerular disease. Here, we have reported a case of PLA2R-positive membranous nephropathy occurring in a patient with HSK. Case presentation After admission to the hospital due to oedema of both the lower extremities, the patient was diagnosed with nephrotic syndrome due to abnormal 24-h urine protein (7540 mg) and blood albumin (25 g/L) levels. Abdominal ultrasonography revealed HSK. The patient’s brother had a history of end-stage renal disease due to nephrotic syndrome. Therefore, the patient was diagnosed with PLA2R-positive stage II membranous nephropathy through renal biopsy under abdominal ultrasonography guidance. He was administered adequate prednisone and cyclophosphamide, and after 6 months of treatment, urinary protein excretion levels significantly decreased. Conclusion The risk and difficulty of renal biopsy in patients with HSK are increased due to structural abnormalities; however, renal biopsy can be accomplished through precise positioning with abdominal ultrasonography. In the literature, 20 cases of HSK with glomerular disease have been reported thus far. Because of the small number of cases, estimating the incidence rate of glomerular diseases in HSK is impossible, and the correlation between HSK and renal pathology cannot be stated. Further studies should be conducted and cases should be accumulated to elucidate this phenomenon.

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

2021 ◽  
Author(s):  
Raghvendra Narayan ◽  
Shivani Singh
Keyword(s):  
Nephrotic Syndrome ◽  
Serum Albumin ◽  
Protein Level ◽  
Urinary Protein ◽  
Mantoux Test ◽  
Laboratory Methods ◽  
X Ray ◽  
Urinary Protein Level ◽  
Chest X Ray ◽  
Nephrotic Range Proteinuria

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.

scholarly journals The impact of coexisting diabetes mellitus on clinical outcomes in patients with idiopathic membranous nephropathy: a retrospective observational study

2020 ◽  
Author(s):  
Zhiyong Xie ◽  
Zhilian Li ◽  
Wei Dong ◽  
Yuanhan Chen ◽  
Ruizhao Li ◽  
...  
Keyword(s):  
Diabetes Mellitus ◽  
Renal Function ◽  
Uric Acid ◽  
Clinical Outcomes ◽  
Independent Risk Factor ◽  
Blood Lipids ◽  
Renal Function Deterioration ◽  
Significant Difference ◽  
Remission Rates ◽  
The Impact

Abstract Background Idiopathic membranous nephropathy (IMN) is frequently coexisted with diabetes mellitus (DM). Few researches investigate clinical outcomes in IMN patients coexisting diabetes mellitus (DM), including remission rates, renal survival and complications. Concurrent DM also pose therapeutic challenges to IMN patients due to the influence of glucocorticoids and immunosuppressant on metabolic disorders. We performed this study to investigate the impact of DM on clinical outcomes in IMN and the influence of therapeutic regime on metabolic parameters in diabetic IMN patients. Methods Two hundred and six adult hospitalized patients diagnosed with biopsy-proven IMN were retrospectively studied, including 42 patients coexisted with DM. Clinical outcomes including remission rates, renal outcome and complications were compared between groups. Impact of cyclophosphamide and ciclosporin on metabolism and complications were analyzed in IMN patients coexisting DM. Results IMN patients coexisted with DM were presented with advanced age, lower level of eGFR and hemoglobin. Patients coexisted with DM experienced worse renal function deterioration and higher incidence of infection. COX regression analysis showed that DM was an independent risk factor for renal function deterioration in IMN patients. There was no significant difference in remission rates and incidence of venous thromboembolism between two groups. Further exploration on the impact of therapeutic regimens on complications and metabolism showed that cyclophosphamide and ciclosporin had no significant difference in incidence of complications including infection and venous thromboembolism, and posed comparable influences on blood glucose, uric acid and blood lipids in IMN patients coexisted with DM. Conclusion Coexisting DM was an independent risk factor for renal function deterioration in IMN patients but did not influence the remission of proteinuria. Glucocorticoids in combination with cyclophosphamide or ciclosporine had similar impact on complications and metabolic index including blood glucose, uric acid and blood lipids in IMN patients coexisted with DM.

scholarly journals The THE ROLE OF ASTAXANTHIN COMPARED WITH METFORMIN IN PREVENTING GLYCATED HUMAN SERUM ALBUMIN FROM POSSIBLE UNFOLDING: A MOLECULAR DYNAMIC STUDY

2019 ◽  
pp. 276-282
Author(s):  
SYAHPUTRA WIBOWO ◽  
SRI WIDYARTI ◽  
AKHMAD SABARUDIN ◽  
DJOKO WAHONO SOEATMADJI ◽  
SUTIMAN BAMBANG SUMITRO
Keyword(s):  
Diabetes Mellitus ◽  
Human Serum Albumin ◽  
Serum Albumin ◽  
Human Serum ◽  
Root Mean Square ◽  
Conformational Changes ◽  
Glycated Albumin ◽  
Mean Square ◽  
Discovery Studio ◽  
Significant Difference

Objectives: Albumin in diabetes mellitus undergoes conformational changes that affect the ability as an endogenous scavenger. Treatment with astaxanthin (ASX) expected to improve the function of albumin in case of diabetes mellitus. The objectives of this study are to compare the capability of ASX and metformin to prevent conformational changes on glycated albumin. Methods: Data mining is performed to obtain human serum albumin (HSA) (4K2C), glucose (79025), ASX (5281224), and metformin (4091). Data preparation used PyRx and Discovery Studio 2016 Client. PyRx is utilized for docking and analysis of receptor-ligand interactions with LigPlus and Discovery Studio 2016 Client. YASARA is used for molecular dynamics simulations with a running time of 15.000 ps. Results: A description of the glycated-HSA (gHSA) conformational changes that are bound to metformin has been successfully carried out. Changes that occur were unfolding and release of bonds in gHSA. Unfolding on gHSA includes the release of bonds between sites A and B. The root mean square deviation (RMSD) backbone value of metformin-gHSA shows a significant difference with gHSA at 8650 ps where gHSA showed 6.47 nm while the metformin-gHSA was 8.06 nm and continues to increase up to 15.72 nm at the end of the simulation. RMSD and root mean square fluctuation residues of gHSA which were interacted with ASX showed conditions close to normal HSA. In 11725 ps ASX-gHSA remained stable at 5.78 nm, whereas gHSA increased to 8.13 nm. gHSA at the end of the simulation showed a number of 9.052 nm while the normal HSA was 7.561 nm. Conclusion: This result indicated that ASX prevents gHSA from possible unfolding.

scholarly journals Histopathological Features of Primary Nephrotic Syndrome in Children

2017 ◽  
Vol 38 (1-2) ◽  
pp. 20
Author(s):  
M P Damanik ◽  
N Yoshikawa
Keyword(s):  
Nephrotic Syndrome ◽  
Renal Biopsy ◽  
Membranoproliferative Glomerulonephritis ◽  
Membranous Glomerulonephritis ◽  
Steroid Treatment ◽  
Mesangial Proliferative Glomerulonephritis ◽  
Primary Nephrotic Syndrome ◽  
Biopsy Specimens ◽  
Significant Difference ◽  
Steroid Sensitive

 Renal biopsy was performed on 28 out of 50 children with primary nephrotic syndrome encountered during the period January 1994 - December 1995. Light microscope (LM) and immunofluorescence microscope QM) examinations were performed on all biopsy specimens. LM examination indicated minimal changes (MC) in 13 cases (46.4%), focal segmental glomerulosclerosis (FSGS) in 10 (35.7%), membranous glomerulonephritis (MG) in 2 (7.1%), mesangial proliferative glomerulonephritis (MPG) in 7 (7.1 %), and membranoproliferative glomerulonephritis (MPGN) in 1 (3.6%). On IM examination, immunoglobulin deposit was not detected in any MC patients, whereas in FSGS, lgG, lgM, C3 and fibrinogen deposits were found. In the MG group, IgG deposition was detected in one case. In the MPG cases, depositions of lgA, IgG, lgM, C3 and fibrinogen were detected and in the case of MPGN, deposits of lgM and C3 were found. Regarding to response to steroid treatment in the MC group, there was a significant difference between the steroid sensitive and steroid insensitive (p<0.05). For the FSGS abnormality in the steroid treatment of U1e insensitive patients, there was found significant difference with the steroid sensitive abnormality (p<0.05). In conclusion, nephritic symptoms (hematuria, proteinuria, azothemia) are possibly the non minimal group and hence, it would be necessary to carry out renal biopsy to prove this.

scholarly journals Nephrotic syndrome due to focal segmental glomerulosclerosis occurring in early pregnancy

2011 ◽  
Vol 4 (2) ◽  
pp. 80-82 ◽  
Author(s):  
A Smyth ◽  
C A Wall
Keyword(s):  
Nephrotic Syndrome ◽  
Renal Biopsy ◽  
Focal Segmental Glomerulosclerosis ◽  
Early Stage ◽  
Treatment Options ◽  
Renal Outcome ◽  
Successful Pregnancy ◽  
Healthy Baby ◽  
Segmental Glomerulosclerosis ◽  
Nephrotic Range Proteinuria

Physiological proteinuria is common during pregnancy, but pathological proteinuria may also occur. Nephrotic range proteinuria most commonly occurs due to preeclampsia, but primary renal disease may also occur. We present a case of a woman who presented with nephrotic range proteinuria and nephrotic syndrome at an early stage of her second pregnancy. Due to proteinuria, haematuria, progressive symptoms and high need for medical treatment, renal biopsy was performed and she was noted to have focal segmental glomerulosclerosis (FSGS). She commenced immunosuppression but remained nephrotic during pregnancy. She delivered a healthy baby boy at 35 weeks gestation. Postpartum she remained nephrotic, and developed further complications requiring renal replacement therapy. Despite a successful pregnancy, this case of FSGS highlights that despite aggressive treatment FSGS in pregnancy is often associated with adverse renal outcome. We discuss investigations, including renal biopsy and empiric treatment options in this report.

scholarly journals Nephrotic Syndrome and Idiopathic Membranous Nephropathy Associated with Autosomal-Dominant Polycystic Kidney Disease

2011 ◽  
Vol 11 ◽  
pp. 1041-1047 ◽  
Author(s):  
Ramón Peces ◽  
Jorge Martínez-Ara ◽  
Carlos Peces ◽  
Mariluz Picazo ◽  
Emilio Cuesta-López ◽  
...  
Keyword(s):  
Renal Function ◽  
Nephrotic Syndrome ◽  
Kidney Disease ◽  
Complete Remission ◽  
Low Dose ◽  
Autosomal Dominant ◽  
Polycystic Kidney ◽  
Autosomal Dominant Polycystic Kidney ◽  
Nephrotic Range Proteinuria ◽  
Dose Prednisone

We report the case of a 38-year-old male with autosomal-dominant polycystic kidney disease (ADPKD) and concomitant nephrotic syndrome secondary to membranous nephropathy (MN). A 3-month course of prednisone 60 mg daily and losartan 100 mg daily resulted in resistance. Treatment with chlorambucil 0.2 mg/kg daily, low-dose prednisone, plus an angiotensin-converting enzyme inhibitor (ACEI) and an angiotensin II receptor blocker (ARB) for 6 weeks resulted in partial remission of his nephrotic syndrome for a duration of 10 months. After relapse of the nephrotic syndrome, a 13-month course of mycophenolate mofetil (MFM) 2 g daily and low-dose prednisone produced complete remission for 44 months. After a new relapse, a second 24-month course of MFM and low-dose prednisone produced partial to complete remission of proteinuria with preservation of renal function. Thirty-six months after MFM withdrawal, complete remission of nephrotic-range proteinuria was maintained and renal function was preserved. This case supports the idea that renal biopsy is needed for ADPKD patients with nephrotic-range proteinuria in order to exclude coexisting glomerular disease and for appropriate treatment/prevention of renal function deterioration. To the best of our knowledge, this is the first reported case of nephrotic syndrome due to MN in a patient with ADPKD treated with MFM, with remission of proteinuria and preservation of renal function after more than 10 years. Findings in this patient also suggest that MFM might reduce cystic cell proliferation and fibrosis, preventing progressive renal scarring with preservation of renal function.

scholarly journals RENAL BIOPSY IN PAEDIATRIC POPULATION

1969 ◽  
Vol 3 (2) ◽  
pp. 314-317
Author(s):  
AHMAD ZEB KHAN ◽  
RIAZ GUL ◽  
AZIZ AHMAD
Keyword(s):  
Nephrotic Syndrome ◽  
Renal Biopsy ◽  
Focal Segmental Glomerulosclerosis ◽  
Minimal Change Disease ◽  
Paediatric Population ◽  
Minimal Change ◽  
Segmental Glomerulosclerosis ◽  
Renal Biopsies ◽  
A Prospective Study ◽  
Nephrotic Range Proteinuria

OBJECTIVE: To find out the pattern of glomerulopathies in paediatric population, undergoing renalbiopsy at Khyber Teaching Hospital, Peshawar.METHODS: This was a prospective study carried out at the department of Nephrology at Khyber TeachingHospital, Peshawar from June 2010 till June 2012. Ultrasound guided percutaneous renal biopsies werecarried out in patients with the finding of; 1 ) Nephrotic range proteinuria in children. 2) Non-Nephroticrange proteinuria with evidence of hypertension / haematuria / deranged renal function or active sedimentson urine microscopy. 3) Steroid resistant nephrotic syndrome in children (patients not responding to steroidin eight weeks time) and 4) Children with nephrotic syndrome who were not tolerant of steroid therapy orwere considered for immunosuppressive drugs.RESULT: A total of 155 renal biopsies were done. Out of these 90 were male patients and 65 were females.The most common histopathological lesion among children population was minimal change disease(42.66%) followed by focal segmental glomerulosclerosis (25.33%) and membranous GN (16.0%). Weobserved that nephrotic range proteinuria was most prevalent in minimal change disease and membranousGN followed by focal segmental glomerulosclerosis. While non-nephrotic range proteinuria was mostlyseen in patients with membranoprolifirative GN.CONCLUSION: In paediatric population, minimal change disease is the most common encounteredglomerulopathy, followed by focal segmental glomerulosclerosisand membranous GN.KEY WORDS: Nephrotic syndrome, Renal biopsy, Proteinuria, Glomerulopathy

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