We reported a biopsy proved case of minimal change nephrotic syndrome in a 72-year-old patient. The minimal change nephrotic syndrome has been steroid sensitive, but the patient had 7 relapses over a span of 5 years. ...We reported a biopsy proved case of minimal change nephrotic syndrome in a 72-year-old patient. The minimal change nephrotic syndrome has been steroid sensitive, but the patient had 7 relapses over a span of 5 years. Each time the dose of steroid is tapered, a relapse of the nephrotic syndrome occurred. Eventually, the patient was complaining of dysphagia and difficulty swallowing. Hospital work-up with barium swallow, endoscopy, and CT of the chest, abdomen and pelvis, revealed a focal stenotic lesion with mild to moderate esophageal dysmotility 7/15/2022. A diagnosis of an ulcerating lesion with biopsy confirmed a neuro-endocrine carcinoma of the gastro-esophageal junction was entertained. The CT of the chest/abdomen/pelvis, 7/19/2022, has shown, an esophageal mass of 5.1 × 5.6 × 7 cm of the gastro-esophageal junction with ulceration. No evidence of spread beyond the esophagus and stomach. The histology revealed a poorly differentiated neuroendocrine tumor of the gastro-esophageal junction. The patient underwent several rounds of chemotherapy, radiation, and surgery culminating in tumor control. His nephrotic syndrome was resolved after the tumor has been controlled by surgery and chemotherapy.展开更多
Nephrotic syndrome is one of the most common childhood kidney diseases. It is mostly found in the age group of 2 to 8 years. Around 10%-15% of nephrotic syndrome cases are non-responders of steroid treatment(SRNS).Ang...Nephrotic syndrome is one of the most common childhood kidney diseases. It is mostly found in the age group of 2 to 8 years. Around 10%-15% of nephrotic syndrome cases are non-responders of steroid treatment(SRNS).Angiotensin converting enzyme(ACE)(I/D) gene association studies are important for detecting kidney disease and herein we assessed the association of ACE(I/D) polymorphism with nephrotic syndrome in South Indian children. We recruited 260 nephrotic syndrome(162 boys and 98 girls) and 218(140 boys and 78 girls) control subjects. ACE I/D polymorphism was analyzed by PCR using genotype allele specific primers. In ACE(I/D), we did not find significant association for the ungrouped data of nephrotic syndrome children and the control subjects. Kidney biopsies were done in 86 nephrotic syndrome cases(minimal change disease, n = 51;focal segmental glomerulosclerosis, n = 27;diffuse mesangial proliferation, n = 8). We segregated them into the minimal change disease/focal segmental glomerulosclerosis groups and observed that the ACE’D’ allele was identified with borderline significance in cases of focal segmental glomerulosclerosis and the ’Ⅰ’ allele was assessed as having very weak association in cases of minimal change disease. ’Ⅱ’ genotype was weakly associated with minimal change disease. Gender specific analysis revealed weak association of’ID’ genotype with female nephrotic syndrome in females. Dominant expression of DD genotype was observed in males with nephrotic syndrome. Our finding indicated that ACE(I/D) has moderate association with focal segmental glomerulosclerosis. However, due to the limited number of biopsy proven focal segmental glomerulosclerosis subjects enrolled, further studies are required to confirm these results.展开更多
Background:Minimal change nephropathy(MCD)is a common pathological type of nephrotic syndrome and is often associated with acute kidney injury(AKI).This study aimed to investigate the clinical characteristics and rela...Background:Minimal change nephropathy(MCD)is a common pathological type of nephrotic syndrome and is often associated with acute kidney injury(AKI).This study aimed to investigate the clinical characteristics and related factors of AKI in patients with MCD and nephrotic syndrome.Methods:Patients from Chinese People’s Liberation Army General Hospital who were diagnosed with pathological renal MCD with clinical manifestations of nephrotic syndrome were included from January 1,2013 to December 31,2017.Patients diagnosed with membranous nephropathy(MN)by renal biopsy from January 1,2013 to December 31,2017 are included as a control population.We retrospectively analyzed the clinical and pathological characteristics of patients as well as the percentages and clinical characteristics of AKI in different age groups.We assessed the correlation of pathological characteristics with serum creatinine using multivariate linear regression analysis.Results:A total of 367 patients with MCD were included in the analysis,with a sex ratio of 1.46:1(male:female)and an age range of 6 to 77 years.Among all the patients,109 developed AKI(29.7%),and of these patients,85 were male(78.0%).In the 586 patients with MN,27(4.6%)patients developed AKI.The percentage of AKI in MCD patients was significantly higher than that in MN patients(χ^(2)=41.063,P<0.001).The percentage of AKI increased with age in the MCD patients.The percentage of AKI in patients aged 50 years or older was 52.9%(46/87),which was significantly higher than that[22.5%(63/280)]in patients under 50 years(χ^(2)=6.347,P=0.013).We observed statistically significant differences in age(43[27,59]years vs.28[20,44]years,Z=5.487,P<0.001),male(78.0%vs.51.4%,χ^(2)=22.470,P<0.001),serum albumin(19.9±6.1 g/L vs.21.5±5.7 g/L,t=2.376,P=0.018),serum creatinine(129.5[105.7,171.1]μmol/L vs.69.7[57.7,81.9]μmol/L,Z=14.190,P<0.001),serum urea(10.1[6.2,15.8]mmol/L vs.4.7[3.6,6.4]mmol/L,Z=10.545,P<0.001),IgE(266.0[86.7,963.0]IU/ml vs.142.0[35.3,516.5]IU/ml,Z=2.742,P=0.007),history of diabetes(6.4%vs.1.2%,P=0.009),and history of hypertension(23.9%vs.5.1%,χ^(2)=28.238,P<0.001)between the AKI group and the non-AKI group.According to multivariate linear regression analysis,among the renal pathological features analyzed,renal tubular epithelial cell damage(β=178.010,95%CI:147.888-208.132,P<0.001)and renal interstitial edema(β=28.833,95%CI:11.966-45.700,P=0.001)correlated with serum creatinine values.Conclusions:The percentage of AKI in MCD patients is significantly higher than that in MN patients.Patients over 50 years old are more likely to develop AKI.Renal tubular epithelial cell injury and renal interstitial edema may be the main pathological lesions that are associated with elevated serum creatinine in patients with MCD.展开更多
Introduction: Idiopathic nephrotic syndrome represents 25% to 30% of glomerulonephritis in adults. These glomerulonephritides are responsible of about the half of chronic kidney failure examined as well in United Stat...Introduction: Idiopathic nephrotic syndrome represents 25% to 30% of glomerulonephritis in adults. These glomerulonephritides are responsible of about the half of chronic kidney failure examined as well in United States as in Europe or Africa. The aim of this study was to determine the anatomoclinic, therapeutic and progression patterns of idiopathic nephritic syndrome in Dakar. Patients and Methods: It is a retrospective ten-year study in the nephrology department of Aristide Le Dantec Hospital. Patients with idiopathic nephrotic syndrome were included. We analyzed anatomoclinic, therapeutic and progression data of idiopathic nephrotic syndrome. Results: On 202 patients with nephrotic syndrome, 156 (77%) were primitive. The mean age was 29.7 ± 12 years with a sex ratio of 2.4. Edema was found in 98 patients (62.8%) and hypertension in 63 patients (40%). The mean proteinuria was 6.8 ± 4.8 g/24h. Histologic lesions found at renal biopsy were focal segmental glomerulosclerosis in 71 patients (45.5%), minimal change disease in 68 patients (43.5%) and membranous nephropathy in 8 patients (5%). 134 patients (85.8%) received steroids alone, 12 patients (7.6%) received cyclophosphamide and 4 patients (2.5%) azathioprine in association with steroids. 44 patients (28.2%) reached remission. The factors of poor prognosis were: age, above 40 years, proteinuria above 10 g/24h, existence of renal failure at admission, absence of use of steroids therapy. Conclusion: This study shows that idiopathic nephrotic syndrome is frequent in our country with a prevalence of 77%. The most common lesion found at the renal biopsy is the focal segmental glomerulosclerosis. Remission is found only in 28% which is very low. 33% of patients progress towards chronic kidney disease due to the lack of early diagnosis and the use of traditional medicine.展开更多
Calcineurin inhibitors (CNI) are potent immunosuppressive agents in prophylaxis against graft rejection and autoimmune diseases including primary glomerulopathies. Previous research showed reversible;acute afferent ar...Calcineurin inhibitors (CNI) are potent immunosuppressive agents in prophylaxis against graft rejection and autoimmune diseases including primary glomerulopathies. Previous research showed reversible;acute afferent arteriolar vasculopathy and irreversible chronic interstitial fibrosis associated with CNI nephrotoxicity. In this case report we describe a patient, with minimal change disease, that had developed chronic and progressive renal disease while receiving therapeutic dose of Tacrolimus. His serum creatinine had reached 537 umol/L and his nephrotic state worsened. Kidney biopsy showed chronic interstitial nephritis. Tacrolimus was discontinued and he was treated with 1 mg/kg prednisone in addition to Mycophenolate mofetil (MMF) 1 g twice daily. By the 2<sup>nd</sup> month;serum creatinine returned to normal and by the 3<sup>rd</sup> month serum albumin too. After 1 month of therapy;the dose of Prednisone was tapered down gradually till 5 mg daily by the end of 3<sup>rd</sup> month. Moreover, the dose of MMF was reduced to 500 mg X2 by the end of 3<sup>rd</sup> month. After 2 years of follow up;he remained stable and without relapse of NS or renal failure. In conclusion, reversible renal disease, due to chronic interstitial nephritis can be induced by CNI which is amenable to treatment with Prednisone and MMF.展开更多
文摘We reported a biopsy proved case of minimal change nephrotic syndrome in a 72-year-old patient. The minimal change nephrotic syndrome has been steroid sensitive, but the patient had 7 relapses over a span of 5 years. Each time the dose of steroid is tapered, a relapse of the nephrotic syndrome occurred. Eventually, the patient was complaining of dysphagia and difficulty swallowing. Hospital work-up with barium swallow, endoscopy, and CT of the chest, abdomen and pelvis, revealed a focal stenotic lesion with mild to moderate esophageal dysmotility 7/15/2022. A diagnosis of an ulcerating lesion with biopsy confirmed a neuro-endocrine carcinoma of the gastro-esophageal junction was entertained. The CT of the chest/abdomen/pelvis, 7/19/2022, has shown, an esophageal mass of 5.1 × 5.6 × 7 cm of the gastro-esophageal junction with ulceration. No evidence of spread beyond the esophagus and stomach. The histology revealed a poorly differentiated neuroendocrine tumor of the gastro-esophageal junction. The patient underwent several rounds of chemotherapy, radiation, and surgery culminating in tumor control. His nephrotic syndrome was resolved after the tumor has been controlled by surgery and chemotherapy.
基金supported by Networking Resource Centre in Biological Sciences (NRCBS funded by UGC, Govt. of India)
文摘Nephrotic syndrome is one of the most common childhood kidney diseases. It is mostly found in the age group of 2 to 8 years. Around 10%-15% of nephrotic syndrome cases are non-responders of steroid treatment(SRNS).Angiotensin converting enzyme(ACE)(I/D) gene association studies are important for detecting kidney disease and herein we assessed the association of ACE(I/D) polymorphism with nephrotic syndrome in South Indian children. We recruited 260 nephrotic syndrome(162 boys and 98 girls) and 218(140 boys and 78 girls) control subjects. ACE I/D polymorphism was analyzed by PCR using genotype allele specific primers. In ACE(I/D), we did not find significant association for the ungrouped data of nephrotic syndrome children and the control subjects. Kidney biopsies were done in 86 nephrotic syndrome cases(minimal change disease, n = 51;focal segmental glomerulosclerosis, n = 27;diffuse mesangial proliferation, n = 8). We segregated them into the minimal change disease/focal segmental glomerulosclerosis groups and observed that the ACE’D’ allele was identified with borderline significance in cases of focal segmental glomerulosclerosis and the ’Ⅰ’ allele was assessed as having very weak association in cases of minimal change disease. ’Ⅱ’ genotype was weakly associated with minimal change disease. Gender specific analysis revealed weak association of’ID’ genotype with female nephrotic syndrome in females. Dominant expression of DD genotype was observed in males with nephrotic syndrome. Our finding indicated that ACE(I/D) has moderate association with focal segmental glomerulosclerosis. However, due to the limited number of biopsy proven focal segmental glomerulosclerosis subjects enrolled, further studies are required to confirm these results.
基金supported by the grants from the National Key Research and Development Program of China(No.2016YFC0906202)the Natural Science Foundation of Hainan Province(No.20158332)Sanya Technology Innovation Fund(No.2014YW33)。
文摘Background:Minimal change nephropathy(MCD)is a common pathological type of nephrotic syndrome and is often associated with acute kidney injury(AKI).This study aimed to investigate the clinical characteristics and related factors of AKI in patients with MCD and nephrotic syndrome.Methods:Patients from Chinese People’s Liberation Army General Hospital who were diagnosed with pathological renal MCD with clinical manifestations of nephrotic syndrome were included from January 1,2013 to December 31,2017.Patients diagnosed with membranous nephropathy(MN)by renal biopsy from January 1,2013 to December 31,2017 are included as a control population.We retrospectively analyzed the clinical and pathological characteristics of patients as well as the percentages and clinical characteristics of AKI in different age groups.We assessed the correlation of pathological characteristics with serum creatinine using multivariate linear regression analysis.Results:A total of 367 patients with MCD were included in the analysis,with a sex ratio of 1.46:1(male:female)and an age range of 6 to 77 years.Among all the patients,109 developed AKI(29.7%),and of these patients,85 were male(78.0%).In the 586 patients with MN,27(4.6%)patients developed AKI.The percentage of AKI in MCD patients was significantly higher than that in MN patients(χ^(2)=41.063,P<0.001).The percentage of AKI increased with age in the MCD patients.The percentage of AKI in patients aged 50 years or older was 52.9%(46/87),which was significantly higher than that[22.5%(63/280)]in patients under 50 years(χ^(2)=6.347,P=0.013).We observed statistically significant differences in age(43[27,59]years vs.28[20,44]years,Z=5.487,P<0.001),male(78.0%vs.51.4%,χ^(2)=22.470,P<0.001),serum albumin(19.9±6.1 g/L vs.21.5±5.7 g/L,t=2.376,P=0.018),serum creatinine(129.5[105.7,171.1]μmol/L vs.69.7[57.7,81.9]μmol/L,Z=14.190,P<0.001),serum urea(10.1[6.2,15.8]mmol/L vs.4.7[3.6,6.4]mmol/L,Z=10.545,P<0.001),IgE(266.0[86.7,963.0]IU/ml vs.142.0[35.3,516.5]IU/ml,Z=2.742,P=0.007),history of diabetes(6.4%vs.1.2%,P=0.009),and history of hypertension(23.9%vs.5.1%,χ^(2)=28.238,P<0.001)between the AKI group and the non-AKI group.According to multivariate linear regression analysis,among the renal pathological features analyzed,renal tubular epithelial cell damage(β=178.010,95%CI:147.888-208.132,P<0.001)and renal interstitial edema(β=28.833,95%CI:11.966-45.700,P=0.001)correlated with serum creatinine values.Conclusions:The percentage of AKI in MCD patients is significantly higher than that in MN patients.Patients over 50 years old are more likely to develop AKI.Renal tubular epithelial cell injury and renal interstitial edema may be the main pathological lesions that are associated with elevated serum creatinine in patients with MCD.
文摘Introduction: Idiopathic nephrotic syndrome represents 25% to 30% of glomerulonephritis in adults. These glomerulonephritides are responsible of about the half of chronic kidney failure examined as well in United States as in Europe or Africa. The aim of this study was to determine the anatomoclinic, therapeutic and progression patterns of idiopathic nephritic syndrome in Dakar. Patients and Methods: It is a retrospective ten-year study in the nephrology department of Aristide Le Dantec Hospital. Patients with idiopathic nephrotic syndrome were included. We analyzed anatomoclinic, therapeutic and progression data of idiopathic nephrotic syndrome. Results: On 202 patients with nephrotic syndrome, 156 (77%) were primitive. The mean age was 29.7 ± 12 years with a sex ratio of 2.4. Edema was found in 98 patients (62.8%) and hypertension in 63 patients (40%). The mean proteinuria was 6.8 ± 4.8 g/24h. Histologic lesions found at renal biopsy were focal segmental glomerulosclerosis in 71 patients (45.5%), minimal change disease in 68 patients (43.5%) and membranous nephropathy in 8 patients (5%). 134 patients (85.8%) received steroids alone, 12 patients (7.6%) received cyclophosphamide and 4 patients (2.5%) azathioprine in association with steroids. 44 patients (28.2%) reached remission. The factors of poor prognosis were: age, above 40 years, proteinuria above 10 g/24h, existence of renal failure at admission, absence of use of steroids therapy. Conclusion: This study shows that idiopathic nephrotic syndrome is frequent in our country with a prevalence of 77%. The most common lesion found at the renal biopsy is the focal segmental glomerulosclerosis. Remission is found only in 28% which is very low. 33% of patients progress towards chronic kidney disease due to the lack of early diagnosis and the use of traditional medicine.
文摘Calcineurin inhibitors (CNI) are potent immunosuppressive agents in prophylaxis against graft rejection and autoimmune diseases including primary glomerulopathies. Previous research showed reversible;acute afferent arteriolar vasculopathy and irreversible chronic interstitial fibrosis associated with CNI nephrotoxicity. In this case report we describe a patient, with minimal change disease, that had developed chronic and progressive renal disease while receiving therapeutic dose of Tacrolimus. His serum creatinine had reached 537 umol/L and his nephrotic state worsened. Kidney biopsy showed chronic interstitial nephritis. Tacrolimus was discontinued and he was treated with 1 mg/kg prednisone in addition to Mycophenolate mofetil (MMF) 1 g twice daily. By the 2<sup>nd</sup> month;serum creatinine returned to normal and by the 3<sup>rd</sup> month serum albumin too. After 1 month of therapy;the dose of Prednisone was tapered down gradually till 5 mg daily by the end of 3<sup>rd</sup> month. Moreover, the dose of MMF was reduced to 500 mg X2 by the end of 3<sup>rd</sup> month. After 2 years of follow up;he remained stable and without relapse of NS or renal failure. In conclusion, reversible renal disease, due to chronic interstitial nephritis can be induced by CNI which is amenable to treatment with Prednisone and MMF.
