S. Sreelatha, B. D'souza, V. D'souza, K. Rajendiran
Increased reactive oxygen species (ROS) in nephrotic syndrome (NS) are involved in the oxidation of membrane proteins, lipoproteins and several receptor molecules ultimately leading to their functional deficit. It is involved in the pathogenesis of dyslipidemia in NS and also increases the oxidation of LDL (oxLDL), which is an important risk factor in thrombus generation and atherosclerosis. Myeloperoxidase (MPO) is an early predictor of myocardial infarction and adverse cardiac events in patients with chest pain. MPO can also foresee the recurrent acute coronary syndrome (ACS) and myocardial infarction in patients. ‘MPO oxidized LDL’ also induces ROS production, lipid accumulation and reduces the antioxidant response in macrophages, however in an augmented way by using different pathways and might be more atherogenic. Paraoxonase 1 (PON1) prevents the oxidative modification of serum lipoproteins, which is one of the crucial steps in the initiation of atherogenesis. PON1 also contributes to the anti-atherogenic effect of HDL-c. Adult NS patients have increased lipid hydroxide levels and significantly decreased PON1 activity and total sulfhydryl levels when compared to healthy controls. While the increased risk of cardiovascular disease in NS patients is well documented, the exact etiology still remains controversial. This prevents the development of a specific treatment modality for the same. MPO as well as PON1 were found as important markers for the management of cardiovascular risk in NS patients. Estimation of these enzymes can therefore be performed in routine clinical practice as prognostic markers, owing to its ease of estimation and cost effectiveness.
{"title":"Role of oxidant-antioxidant enzymes in managing the cardiovascular risks in nephrotic syndrome patients","authors":"S. Sreelatha, B. D'souza, V. D'souza, K. Rajendiran","doi":"10.34172/jnp.2022.17276","DOIUrl":"https://doi.org/10.34172/jnp.2022.17276","url":null,"abstract":"Increased reactive oxygen species (ROS) in nephrotic syndrome (NS) are involved in the oxidation of membrane proteins, lipoproteins and several receptor molecules ultimately leading to their functional deficit. It is involved in the pathogenesis of dyslipidemia in NS and also increases the oxidation of LDL (oxLDL), which is an important risk factor in thrombus generation and atherosclerosis. Myeloperoxidase (MPO) is an early predictor of myocardial infarction and adverse cardiac events in patients with chest pain. MPO can also foresee the recurrent acute coronary syndrome (ACS) and myocardial infarction in patients. ‘MPO oxidized LDL’ also induces ROS production, lipid accumulation and reduces the antioxidant response in macrophages, however in an augmented way by using different pathways and might be more atherogenic. Paraoxonase 1 (PON1) prevents the oxidative modification of serum lipoproteins, which is one of the crucial steps in the initiation of atherogenesis. PON1 also contributes to the anti-atherogenic effect of HDL-c. Adult NS patients have increased lipid hydroxide levels and significantly decreased PON1 activity and total sulfhydryl levels when compared to healthy controls. While the increased risk of cardiovascular disease in NS patients is well documented, the exact etiology still remains controversial. This prevents the development of a specific treatment modality for the same. MPO as well as PON1 were found as important markers for the management of cardiovascular risk in NS patients. Estimation of these enzymes can therefore be performed in routine clinical practice as prognostic markers, owing to its ease of estimation and cost effectiveness.","PeriodicalId":16515,"journal":{"name":"Journal of Nephropathology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-12-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"45383435","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Mahmoud Beheshti Monfared, H. Ghaderi, Zahra Ansari Aval, M. Hekmat, S. Mirjafari, Reza Beheshti Monfared
The clinical manifestations of extragonadal germ cell tumor (EGGCT) depend on its location and are often caused by compression on surrounding structures. Pericardial effusion is absolutely rare as an initial clinical manifestation for this tumor. We report a 31-year-old man presenting to the emergency department with dyspnea. Two-dimensional echocardiography revealed a massive pericardial effusion with impending tamponade. The patient was immediately transferred to the operating room where a cardiac surgeon drained bloody effusion. A mediastinal mass measuring 173×105×105 mm was accidentally noticed during COVID-19 work-up. Fine core needle biopsy of the mass led to the diagnosis of a germ cell tumor, which was treated appropriately. This study shows the importance of proper work-up in pericardial effusion cases with chest CT-scan as an important part of it.
{"title":"A rare presentation of extragonadal germ cell tumor; massive pericardial effusion with impending tamponade","authors":"Mahmoud Beheshti Monfared, H. Ghaderi, Zahra Ansari Aval, M. Hekmat, S. Mirjafari, Reza Beheshti Monfared","doi":"10.34172/jnp.2022.17219","DOIUrl":"https://doi.org/10.34172/jnp.2022.17219","url":null,"abstract":"The clinical manifestations of extragonadal germ cell tumor (EGGCT) depend on its location and are often caused by compression on surrounding structures. Pericardial effusion is absolutely rare as an initial clinical manifestation for this tumor. We report a 31-year-old man presenting to the emergency department with dyspnea. Two-dimensional echocardiography revealed a massive pericardial effusion with impending tamponade. The patient was immediately transferred to the operating room where a cardiac surgeon drained bloody effusion. A mediastinal mass measuring 173×105×105 mm was accidentally noticed during COVID-19 work-up. Fine core needle biopsy of the mass led to the diagnosis of a germ cell tumor, which was treated appropriately. This study shows the importance of proper work-up in pericardial effusion cases with chest CT-scan as an important part of it.","PeriodicalId":16515,"journal":{"name":"Journal of Nephropathology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-12-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"44390162","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
M. Behairy, A. ElShaarawy, Somia Bawady, F. Elsayed, W. Bichari
Introduction: Podocyturia can be considered as a noninvasive marker for evaluation and follow up of glomerular disease progression. Objectives: In this study, we aimed to assess the clinical utility of urinary podocin as an index of lupus nephritis activity. Patients and Methods: This cross-sectional study included 45 patients with systemic lupus erythematosus (SLE). Patients were subdivided into three groups: group (I) 10 SLE, patients without clinical or laboratory evidence of lupus nephritis (LN), which were assessed by Systemic Lupus Activity Measure (SLAM) score of the disease activity. Group (II), which included 15 patients with evident active LN before starting the immunosuppressive induction treatment and group (III) which is consisted of 20 patients with LN in partial or complete remission. Urinary podocin assay was conducted by enzyme-linked immunosorbent assay enzyme-linked immunosorbent assay (ELISA) technique. Results: There was a statistically significant difference between the studied groups regarding urinary podocin levels. The mean of urinary podocin (ng/mL) was (2.29 ± 0.71, 37.20 ± 14.38, 10.5 ±2.30; P≤0.001) in the three groups consecutively, with significant decrease of urinary podocin in LN patients after remission versus high level in patients with active LN. Highly significant positive correlations were found between urinary podocin and global SLAM activity (r = 0.852; P≤ 0.001), SLAM-Renal score (r= 0.854; P≤0.001), urine albumin to creatinine ratio, (mg/g) (r=0.895; P≤0.001). Highly significant negative correlations of urinary podocin and C3 (r=0.803; P≤ 0.001), C4 (r= -0.760; P≤0.001) and eGFR (r = -0.759; P≤0.001) were detected. Conclusion: Urinary podocin as non-invasive biomarker is significantly correlated to SLE disease activity and LN activity measured by global SLAM clinical score with both high sensitivity and specificity. Urinary podocin can be also considered as a prognostic marker in the management of LN patients.
{"title":"Urinary podocin; is it a valuable disease activity biomarker in patients with lupus nephritis?","authors":"M. Behairy, A. ElShaarawy, Somia Bawady, F. Elsayed, W. Bichari","doi":"10.34172/jnp.2022.17268","DOIUrl":"https://doi.org/10.34172/jnp.2022.17268","url":null,"abstract":"Introduction: Podocyturia can be considered as a noninvasive marker for evaluation and follow up of glomerular disease progression. Objectives: In this study, we aimed to assess the clinical utility of urinary podocin as an index of lupus nephritis activity. Patients and Methods: This cross-sectional study included 45 patients with systemic lupus erythematosus (SLE). Patients were subdivided into three groups: group (I) 10 SLE, patients without clinical or laboratory evidence of lupus nephritis (LN), which were assessed by Systemic Lupus Activity Measure (SLAM) score of the disease activity. Group (II), which included 15 patients with evident active LN before starting the immunosuppressive induction treatment and group (III) which is consisted of 20 patients with LN in partial or complete remission. Urinary podocin assay was conducted by enzyme-linked immunosorbent assay enzyme-linked immunosorbent assay (ELISA) technique. Results: There was a statistically significant difference between the studied groups regarding urinary podocin levels. The mean of urinary podocin (ng/mL) was (2.29 ± 0.71, 37.20 ± 14.38, 10.5 ±2.30; P≤0.001) in the three groups consecutively, with significant decrease of urinary podocin in LN patients after remission versus high level in patients with active LN. Highly significant positive correlations were found between urinary podocin and global SLAM activity (r = 0.852; P≤ 0.001), SLAM-Renal score (r= 0.854; P≤0.001), urine albumin to creatinine ratio, (mg/g) (r=0.895; P≤0.001). Highly significant negative correlations of urinary podocin and C3 (r=0.803; P≤ 0.001), C4 (r= -0.760; P≤0.001) and eGFR (r = -0.759; P≤0.001) were detected. Conclusion: Urinary podocin as non-invasive biomarker is significantly correlated to SLE disease activity and LN activity measured by global SLAM clinical score with both high sensitivity and specificity. Urinary podocin can be also considered as a prognostic marker in the management of LN patients.","PeriodicalId":16515,"journal":{"name":"Journal of Nephropathology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-12-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"44276656","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
V. Ganesan, K. Sethuraman, S. Sureshkumar, Venkataraman Prabhu
Introduction: Patients with chronic kidney disease (CKD) have increasingly been diagnosed with cognitive impairment. Glycogen synthase kinase 3β (GSK3β) is directly causing both phosphorylated tau (pTau) and amyloid β (Aβ) accumulation in Alzheimer’s disease (AD). GSK3β expression is more abundant in human platelets than in other blood cells. Recombinant human erythropoietin (rHuEPO) is a common medicine for treating anemia in patients with CKD, as well as a neuroprotective agent. Objectives: The goal of this research is to find out how platelet GSK3β regulates plasma Aβ, total Tau and tau phosphorylated at threonine 181 (p-tau181) levels in CKD patients with cognitive dysfunction and also the efficacy of rHuEPO treatment. Patients and Methods: The study included 60 participants, which consist of 30 CKD without cognitive dysfunction and 30 CKD with cognitive dysfunction based on the neuropsychological examination. The expression of GSK3β in platelets was evaluated using a western blot and plasma Aβ, total Tau, pTau 181 levels were quantified by ELISA. The data were compared statistically (P< 0.05) to AD, normocytic normochromic anemic and healthy patients. Results: In CKD with cognitive dysfunction subjects, platelet GSK3β expression and plasma Aβ, total Tau and pTau181 levels were significantly (P< 0.05) altered like AD when compared to normocytic normochromic anemic, healthy and CKD without cognitive dysfunction subjects. In post rHuEPO (100 IU/kg, weekly twice, six months) treatment, the altered protein abnormalities were retrieved significantly (P<0.05) compared to pre-treatment. Conclusion: This study established that platelet GSK3β expression and plasma Aβ, total Tau, pTau181 are the candidate biomarkers for cognitive dysfunction in CKD patients. The clinical utility of rHuEPO as a GSK3β inhibitor and therapeutic agent for cognitive dysfunction in CKD has been determined.
{"title":"Platelet glycogen synthase kinase 3β regulates plasma β amyloid and phosphorylated tau levels in chronic kidney disease patients with cognitive dysfunction; therapeutic role of erythropoietin","authors":"V. Ganesan, K. Sethuraman, S. Sureshkumar, Venkataraman Prabhu","doi":"10.34172/jnp.2022.17238","DOIUrl":"https://doi.org/10.34172/jnp.2022.17238","url":null,"abstract":"Introduction: Patients with chronic kidney disease (CKD) have increasingly been diagnosed with cognitive impairment. Glycogen synthase kinase 3β (GSK3β) is directly causing both phosphorylated tau (pTau) and amyloid β (Aβ) accumulation in Alzheimer’s disease (AD). GSK3β expression is more abundant in human platelets than in other blood cells. Recombinant human erythropoietin (rHuEPO) is a common medicine for treating anemia in patients with CKD, as well as a neuroprotective agent. Objectives: The goal of this research is to find out how platelet GSK3β regulates plasma Aβ, total Tau and tau phosphorylated at threonine 181 (p-tau181) levels in CKD patients with cognitive dysfunction and also the efficacy of rHuEPO treatment. Patients and Methods: The study included 60 participants, which consist of 30 CKD without cognitive dysfunction and 30 CKD with cognitive dysfunction based on the neuropsychological examination. The expression of GSK3β in platelets was evaluated using a western blot and plasma Aβ, total Tau, pTau 181 levels were quantified by ELISA. The data were compared statistically (P< 0.05) to AD, normocytic normochromic anemic and healthy patients. Results: In CKD with cognitive dysfunction subjects, platelet GSK3β expression and plasma Aβ, total Tau and pTau181 levels were significantly (P< 0.05) altered like AD when compared to normocytic normochromic anemic, healthy and CKD without cognitive dysfunction subjects. In post rHuEPO (100 IU/kg, weekly twice, six months) treatment, the altered protein abnormalities were retrieved significantly (P<0.05) compared to pre-treatment. Conclusion: This study established that platelet GSK3β expression and plasma Aβ, total Tau, pTau181 are the candidate biomarkers for cognitive dysfunction in CKD patients. The clinical utility of rHuEPO as a GSK3β inhibitor and therapeutic agent for cognitive dysfunction in CKD has been determined.","PeriodicalId":16515,"journal":{"name":"Journal of Nephropathology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-12-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49340890","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Lupus nephritis (LN) is one of the most serious manifestations of systemic lupus erythematosus (SLE). Despite available guidelines recommendations on appropriate therapeutic agents, up to one-third of LN patients still do not meet expected response to initial corticosteroid or immunosuppressive treatment. We report a 17-year-old Indonesian female who was diagnosed LN with persistent proteinuria manifestations. Renal biopsy was suggestive of class II LN. Corticosteroid was given for a month without therapeutic response, and the patient was given combination of moderate dose methylprednisolone and mycophenolic acid resulted in complete remission after nine months therapy. Despite the existing guidelines, choices of LN treatment might be individual depends on disease severity (clinical, laboratory and histopathological findings) and demographic factors. The combination of mycophenolic acid and corticosteroid might be better option than high dose corticosteroid to treat class II LN for minimizing the adverse event of corticosteroid.
{"title":"Treatment of class II lupus nephritis with combination therapy of mycophenolic acid and corticosteroid; a case report","authors":"Faizal Armando Nugroho, B. D. Shanti, W. Widodo","doi":"10.34172/jnp.2022.17267","DOIUrl":"https://doi.org/10.34172/jnp.2022.17267","url":null,"abstract":"Lupus nephritis (LN) is one of the most serious manifestations of systemic lupus erythematosus (SLE). Despite available guidelines recommendations on appropriate therapeutic agents, up to one-third of LN patients still do not meet expected response to initial corticosteroid or immunosuppressive treatment. We report a 17-year-old Indonesian female who was diagnosed LN with persistent proteinuria manifestations. Renal biopsy was suggestive of class II LN. Corticosteroid was given for a month without therapeutic response, and the patient was given combination of moderate dose methylprednisolone and mycophenolic acid resulted in complete remission after nine months therapy. Despite the existing guidelines, choices of LN treatment might be individual depends on disease severity (clinical, laboratory and histopathological findings) and demographic factors. The combination of mycophenolic acid and corticosteroid might be better option than high dose corticosteroid to treat class II LN for minimizing the adverse event of corticosteroid.","PeriodicalId":16515,"journal":{"name":"Journal of Nephropathology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-11-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48762427","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Introduction: Chronic kidney disease (CKD) poses a financial burden on most patients from low/ middle income countries. Glycaemic control with affordable hypoglycemic agents may influence on the prognosis of diabetic nephropathy. Objectives: We aimed to compare the rates of CKD progression and proteinuria in the type 2 diabetic population in response to the use of various hypoglycemic agents. Patients and Methods: A retrospective cross-sectional study of a total of 250 patients of Afro-Caribbean descent at the University hospital of the West Indies between 2018 and 2019 was conducted. The use of hypoglycaemic agents and changes in albuminuria were calculated as odds ratios with a 95% confidence interval (CI). A P value<0.05 was considered statistically significant. Results: Of 250 patients with diabetic nephropathy, the number of rapid CKD progression was highest in patients on insulin (26.3%). In comparison, number of rapid progressions in patients receiving metformin, dipeptidyl peptidase 4 (DPP-4 inhibitors), sulfonylurea and pioglitazone were 19.1%, 22.2%, 21.9% and 20%, respectively. After eliminating confounding factors, comparison within the group analysis on DPP-4 inhibitors (n= 171) demonstrated 62.6% significant improvement in quantitative proteinuria with reduction of mean spot urine albumin creatinine ratio (ACR) from 362.1 ±338.9 mg/g to 303 ±300.1 mg/g (ORs, 0.77; 95% CI 0.41 to 0.97; P = 0.03). Conclusion: Type 2 diabetic patients requiring insulin were found to have progression of CKD than patients on oral hypoglycaemic agents. Among the affordable oral hypoglycaemic agents, DPP-4 inhibitors had an association with reduction in albuminuria.
{"title":"Hypoglycemic agents and prognostic outcomes of chronic kidney disease patients with type 2 diabetes","authors":"Kyaw Kyaw Hoe, Ting Han, T. Hoe","doi":"10.34172/jnp.2022.17294","DOIUrl":"https://doi.org/10.34172/jnp.2022.17294","url":null,"abstract":"Introduction: Chronic kidney disease (CKD) poses a financial burden on most patients from low/ middle income countries. Glycaemic control with affordable hypoglycemic agents may influence on the prognosis of diabetic nephropathy. Objectives: We aimed to compare the rates of CKD progression and proteinuria in the type 2 diabetic population in response to the use of various hypoglycemic agents. Patients and Methods: A retrospective cross-sectional study of a total of 250 patients of Afro-Caribbean descent at the University hospital of the West Indies between 2018 and 2019 was conducted. The use of hypoglycaemic agents and changes in albuminuria were calculated as odds ratios with a 95% confidence interval (CI). A P value<0.05 was considered statistically significant. Results: Of 250 patients with diabetic nephropathy, the number of rapid CKD progression was highest in patients on insulin (26.3%). In comparison, number of rapid progressions in patients receiving metformin, dipeptidyl peptidase 4 (DPP-4 inhibitors), sulfonylurea and pioglitazone were 19.1%, 22.2%, 21.9% and 20%, respectively. After eliminating confounding factors, comparison within the group analysis on DPP-4 inhibitors (n= 171) demonstrated 62.6% significant improvement in quantitative proteinuria with reduction of mean spot urine albumin creatinine ratio (ACR) from 362.1 ±338.9 mg/g to 303 ±300.1 mg/g (ORs, 0.77; 95% CI 0.41 to 0.97; P = 0.03). Conclusion: Type 2 diabetic patients requiring insulin were found to have progression of CKD than patients on oral hypoglycaemic agents. Among the affordable oral hypoglycaemic agents, DPP-4 inhibitors had an association with reduction in albuminuria.","PeriodicalId":16515,"journal":{"name":"Journal of Nephropathology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-11-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49411346","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Atthaphong Phongphithakchai, Suntornwit Praditau-Krit, P. Dandecha, Ussanee Boonsrirat, Poowadon Wetwittayakhlung
Anti-glomerular basement membrane (anti-GBM) nephritis is uncommon glomerular disease caused by autoantibodies targeting the capillary beds of the kidney. The clinical presentation of the disease is a variable nephritic syndrome, rapidly progressing to glomerulonephritis. Treatment outcomes are dependent on predictors at first diagnosis. We presented a case of 58-year-old man who did not have underlying disease presented with marked abdominal distension and acute kidney injury. He had no evidence of chronic renal disease before admission however, laboratory test showed microscopic haematuria (RBC 30-50 per high-powered field), proteinuria (2.9 g/d), and renal failure (serum creatinine 610 µmol/L) compatible with rapidly progressive glomerulonephritis; hence, a renal biopsy was conducted. The pathology showed 100% crescentic glomerulonephritis with IgG deposits in a linear pattern at the GBM. The initial serum anti-GBM titre was 105.59 RU/mL. This patient had poor renal prognosis factors for treatment response. After a discussion regarding treatment option with the patient, we decided to give intensive immunosuppressive therapy and plasmapheresis due to his good baseline functional status. The patient achieved partial remission and is not dialysis dependent. In conclusion, despite a poor renal prognosis with 100% crescents and serum creatinine ≥ 600 µmol/L, the treated patient had a good survival status and did not become dialysis-dependent. However, immunosuppressive treatment should be performed along with careful monitoring for infection to avoid infection-related morbidity and mortality.
{"title":"Successful treatment with intense immunosuppressive therapy in an initially 100% crescentic lesion of anti-GBM nephritis","authors":"Atthaphong Phongphithakchai, Suntornwit Praditau-Krit, P. Dandecha, Ussanee Boonsrirat, Poowadon Wetwittayakhlung","doi":"10.34172/jnp.2022.17275","DOIUrl":"https://doi.org/10.34172/jnp.2022.17275","url":null,"abstract":"Anti-glomerular basement membrane (anti-GBM) nephritis is uncommon glomerular disease caused by autoantibodies targeting the capillary beds of the kidney. The clinical presentation of the disease is a variable nephritic syndrome, rapidly progressing to glomerulonephritis. Treatment outcomes are dependent on predictors at first diagnosis. We presented a case of 58-year-old man who did not have underlying disease presented with marked abdominal distension and acute kidney injury. He had no evidence of chronic renal disease before admission however, laboratory test showed microscopic haematuria (RBC 30-50 per high-powered field), proteinuria (2.9 g/d), and renal failure (serum creatinine 610 µmol/L) compatible with rapidly progressive glomerulonephritis; hence, a renal biopsy was conducted. The pathology showed 100% crescentic glomerulonephritis with IgG deposits in a linear pattern at the GBM. The initial serum anti-GBM titre was 105.59 RU/mL. This patient had poor renal prognosis factors for treatment response. After a discussion regarding treatment option with the patient, we decided to give intensive immunosuppressive therapy and plasmapheresis due to his good baseline functional status. The patient achieved partial remission and is not dialysis dependent. In conclusion, despite a poor renal prognosis with 100% crescents and serum creatinine ≥ 600 µmol/L, the treated patient had a good survival status and did not become dialysis-dependent. However, immunosuppressive treatment should be performed along with careful monitoring for infection to avoid infection-related morbidity and mortality.","PeriodicalId":16515,"journal":{"name":"Journal of Nephropathology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-10-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43800256","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
M. Askari, A. Ghadiri-anari, Asma Jaafarinia, S. Kharazmi, R. Hemayati
Introduction: Diabetes mellitus (DM) is a metabolic disorder appearing as a main public health problem nowadays. Objectives: This study aimed to evaluate the effect of calcitriol on microalbuminuria in patients with type 2 DM (T2DM). Patients and Methods: This double-blind randomized clinical trial was performed on 38 patients with T2DM who had micro- albuminuria. These patients were randomly classified into two groups of treatment and control. The treatment group received calcitriol 0.25 μg daily since the control group received a placebo. Duration of treatment was three months. In baseline, serum creatinine (Cr), fasting blood sugar (FBS), glycated hemoglobin (HbA1c), cholesterol (Chol), triglyceride (TG), low-density lipoprotein (LDL-c), high-density lipoprotein (HDL-c), and micro-albuminuria were measured. Patients were followed up for three months. P<0.05 was set as a significant level. Results: In baseline, two groups did not differ significantly in terms of serum Cr, FBS, HbA1c, Chol, TG, HDL-c, LDL-c, and micro-albuminuria (P> 0.05). After the intervention, there was no significant difference between the two groups regarding the median of serum Cr, FBS, HbA1c, Chol, TG, LDL-c, HDL-c, and microalbuminuria. The median of microalbuminuria in the treatment and control groups was decreased at 46 mg/g and 11 mg/g, respectively. The difference in median of micro-albuminuria was not statically significant between the two groups; however, a significant difference was detected in the treatment group before and after the intervention (P=0.03). Conclusion: Administration of calcitriol could reduce micro-albuminuria after three months. Therefore, the addition of calcitriol to angiotensin-converting enzyme (ACE) inhibitors and angiotensin receptor blockers (ARBs) in patients with T2DM and micro- albuminuria may have a beneficial effect on reducing their proteinuria. Trial Registration: The trial protocol was approved by the Iranian Registry of Clinical Trials (identifier: IRCT2016091429812N; https://en.irct.ir/trial/23865, ethical code; IR.SSU.Rec.65415).
{"title":"The effects of calcitriol on microalbuminuria in patients with type 2 diabetes mellitus; a double-blind randomized clinical trial","authors":"M. Askari, A. Ghadiri-anari, Asma Jaafarinia, S. Kharazmi, R. Hemayati","doi":"10.34172/jnp.2022.17163","DOIUrl":"https://doi.org/10.34172/jnp.2022.17163","url":null,"abstract":"Introduction: Diabetes mellitus (DM) is a metabolic disorder appearing as a main public health problem nowadays. Objectives: This study aimed to evaluate the effect of calcitriol on microalbuminuria in patients with type 2 DM (T2DM). Patients and Methods: This double-blind randomized clinical trial was performed on 38 patients with T2DM who had micro- albuminuria. These patients were randomly classified into two groups of treatment and control. The treatment group received calcitriol 0.25 μg daily since the control group received a placebo. Duration of treatment was three months. In baseline, serum creatinine (Cr), fasting blood sugar (FBS), glycated hemoglobin (HbA1c), cholesterol (Chol), triglyceride (TG), low-density lipoprotein (LDL-c), high-density lipoprotein (HDL-c), and micro-albuminuria were measured. Patients were followed up for three months. P<0.05 was set as a significant level. Results: In baseline, two groups did not differ significantly in terms of serum Cr, FBS, HbA1c, Chol, TG, HDL-c, LDL-c, and micro-albuminuria (P> 0.05). After the intervention, there was no significant difference between the two groups regarding the median of serum Cr, FBS, HbA1c, Chol, TG, LDL-c, HDL-c, and microalbuminuria. The median of microalbuminuria in the treatment and control groups was decreased at 46 mg/g and 11 mg/g, respectively. The difference in median of micro-albuminuria was not statically significant between the two groups; however, a significant difference was detected in the treatment group before and after the intervention (P=0.03). Conclusion: Administration of calcitriol could reduce micro-albuminuria after three months. Therefore, the addition of calcitriol to angiotensin-converting enzyme (ACE) inhibitors and angiotensin receptor blockers (ARBs) in patients with T2DM and micro- albuminuria may have a beneficial effect on reducing their proteinuria. Trial Registration: The trial protocol was approved by the Iranian Registry of Clinical Trials (identifier: IRCT2016091429812N; https://en.irct.ir/trial/23865, ethical code; IR.SSU.Rec.65415).","PeriodicalId":16515,"journal":{"name":"Journal of Nephropathology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-09-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49492171","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
F. Yaghoubi, D. Babakhani, Farnoosh Tavakoli, F. Tavakoli
A 14-year-old boy with a past medical history of bone marrow transplantation (BMT) was referred to the emergency department with the loss of consciousness and seizure. On admission, the blood test indicated strict hyponatremia with hypokalemia, hypomagnesemia, hypophosphatemia, hypoglycemia, and low-serum low-density lipoprotein cholesterol (LDL-C). After six days, the patient suffered from dysarthria, dysphagia, behavioral disturbances, disorientation, and obtundation. Based on the physical examination, hyperreflexia and upward bilateral plantar reflexes were outstanding. Lumbar puncture, spiral brain CT scan, and MRI were normal. Hence, MRI repeated 2 weeks later, and the T2-weighted image indicated the bilateral symmetric hyperintense lesions in the basal ganglia. The osmotic demyelination syndrome (ODS) is a scarce and serious neurologic complication of the quick correction of chronic strict hyponatremia.
{"title":"Osmotic demyelination syndrome after bone marrow transplantation","authors":"F. Yaghoubi, D. Babakhani, Farnoosh Tavakoli, F. Tavakoli","doi":"10.34172/jnp.2022.10","DOIUrl":"https://doi.org/10.34172/jnp.2022.10","url":null,"abstract":"A 14-year-old boy with a past medical history of bone marrow transplantation (BMT) was referred to the emergency department with the loss of consciousness and seizure. On admission, the blood test indicated strict hyponatremia with hypokalemia, hypomagnesemia, hypophosphatemia, hypoglycemia, and low-serum low-density lipoprotein cholesterol (LDL-C). After six days, the patient suffered from dysarthria, dysphagia, behavioral disturbances, disorientation, and obtundation. Based on the physical examination, hyperreflexia and upward bilateral plantar reflexes were outstanding. Lumbar puncture, spiral brain CT scan, and MRI were normal. Hence, MRI repeated 2 weeks later, and the T2-weighted image indicated the bilateral symmetric hyperintense lesions in the basal ganglia. The osmotic demyelination syndrome (ODS) is a scarce and serious neurologic complication of the quick correction of chronic strict hyponatremia.","PeriodicalId":16515,"journal":{"name":"Journal of Nephropathology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-07-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46683190","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Laura Panaro, M. Trezzi, M. Ardini, M. Marchini, M. Delsante, D. Rolla
Introduction: Chronic active antibody-mediated rejection (cAMR) is a significant and rapid destructive form of allograft rejection and it is related to donor specific antibodies (DSA). Interleukin 6 (IL-6) plays an important role in mediating the allograft rejection by promoting CD4+ T cells differentiation to Th17 phenotype while inhibiting Treg. Tocilizumab is a humanized monoclonal antibody directed to IL-6 receptor (IL6-R). The aim of the study is to demonstrate the efficacy of tocilizumab as rescue therapy for cAMR. Case Presentation: A 50-year-old man with Alport syndrome and with positive DSA against B7 e B55 underwent a second kidney transplant (HLA 2 mismatch). He received thymoglobulin and three plasma exchanges as induction therapy. Proteinuria (1-1.3 g/24 h) and decline in kidney function (serum creatinine; 1.5 mg/dL) appeared at 9 months. Kidney biopsy showed endocapillary proliferation, mononuclear cells infiltration, glomerular basal membrane duplication and tubulitis suggestive of cAMR. The patient has been treated with tocilizumab (6 mg/kg/mon) for six months. Reduction of proteinuria (0.6 g/24 h) and mild improvement of kidney function (serum creatinine; 1.3 mg/dL) were observed after tocilizumab treatment. A second biopsy revealed a significant decrease of glomerulitis and peritubular capillaritis. A significant reduction in DSA was detected. Conclusion: Inhibition of the IL-6 receptor by tocilizumab may represent a novel and cheering approach to treat cAMR.
引言：慢性活性抗体介导的排斥反应（cAMR）是同种异体移植物排斥反应的一种重要且快速的破坏性形式，它与供体特异性抗体（DSA）有关。白细胞介素6（IL-6）通过促进CD4+T细胞向Th17表型分化，同时抑制Treg，在介导同种异体移植物排斥反应中发挥重要作用。托奇利珠单抗是一种针对IL-6受体（IL6-R）的人源化单克隆抗体。本研究的目的是证明托西利珠单抗作为cAMR的抢救疗法的疗效。病例介绍：一名患有Alport综合征且B7 e B55 DSA阳性的50岁男性接受了第二次肾移植（HLA 2错配）。他接受了胸腺球蛋白和三次血浆置换作为诱导治疗。9个月时出现蛋白尿（1-1.3 g/24小时）和肾功能下降（血清肌酐；1.5 mg/dL）。肾活检显示毛细血管内增殖、单核细胞浸润、肾小球基底膜重复和提示cAMR的小管炎。患者已接受托西利珠单抗（6 mg/kg/月）治疗6个月。tocilizumab治疗后观察到蛋白尿减少（0.6 g/24小时）和肾功能轻度改善（血清肌酐；1.3 mg/dL）。第二次活检显示肾小球炎和管周毛细血管炎明显减少。DSA显著降低。结论：tocilizumab对IL-6受体的抑制作用可能是治疗cAMR的一种新方法。
{"title":"Tocilizumab in a patient affected by chronic active antibody mediated rejection; histological improvement, reduction of proteinuria and renal function stabilization","authors":"Laura Panaro, M. Trezzi, M. Ardini, M. Marchini, M. Delsante, D. Rolla","doi":"10.34172/jnp.2022.17220","DOIUrl":"https://doi.org/10.34172/jnp.2022.17220","url":null,"abstract":"Introduction: Chronic active antibody-mediated rejection (cAMR) is a significant and rapid destructive form of allograft rejection and it is related to donor specific antibodies (DSA). Interleukin 6 (IL-6) plays an important role in mediating the allograft rejection by promoting CD4+ T cells differentiation to Th17 phenotype while inhibiting Treg. Tocilizumab is a humanized monoclonal antibody directed to IL-6 receptor (IL6-R). The aim of the study is to demonstrate the efficacy of tocilizumab as rescue therapy for cAMR. Case Presentation: A 50-year-old man with Alport syndrome and with positive DSA against B7 e B55 underwent a second kidney transplant (HLA 2 mismatch). He received thymoglobulin and three plasma exchanges as induction therapy. Proteinuria (1-1.3 g/24 h) and decline in kidney function (serum creatinine; 1.5 mg/dL) appeared at 9 months. Kidney biopsy showed endocapillary proliferation, mononuclear cells infiltration, glomerular basal membrane duplication and tubulitis suggestive of cAMR. The patient has been treated with tocilizumab (6 mg/kg/mon) for six months. Reduction of proteinuria (0.6 g/24 h) and mild improvement of kidney function (serum creatinine; 1.3 mg/dL) were observed after tocilizumab treatment. A second biopsy revealed a significant decrease of glomerulitis and peritubular capillaritis. A significant reduction in DSA was detected. Conclusion: Inhibition of the IL-6 receptor by tocilizumab may represent a novel and cheering approach to treat cAMR.","PeriodicalId":16515,"journal":{"name":"Journal of Nephropathology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-07-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49060302","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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