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Objective To analyze the clinicopathological features and prognosis of minimal change nephropa-thy with IgA deposition(MCD-IgA)in children.Methods The clinical and pathological data of 10 cases in Chil-dren's Hospital of Nanjing Medical University from January 2010 to December 2015 with MCD-IgA were retrospective-ly analyzed,and 24 cases of minimal change nephrotic syndrome(MCD-NS)and 21 cases of IgA nephropathy clini-cally manifested with nephrotic syndrome(NS-IgAN)were selected as controls.Results (1)Clinical manifesta-tions:there were no significant differences in age,gender,incidence of hematuria,level of 24 hours urine protein,serum albumin and cholesterol levels and elevated serum IgA ratio in MCD-IgA compared with MCD-NS group.Compared with MCD-IgA and MCD-NS,NS -IgAN group showed older age of onset[(8.6 ± 2.1)years vs.(4.8 ± 2.4) years,(4.0 ± 1.6)years],higher level of serum albumin[(22.8 ± 4.3)g/L vs.(19.0 ± 1.9)g/L,(16.8 ± 3.0) g/L],and lower level of serum total cholesterol[(7.9 ± 1.9)mmol/L vs.(9.9 ± 2.7)mmol/L,(9.8 ± 2.1)mmol/L], and all the differences were significant(all P<0.05).NS-IgAN group was all associated with gross hematuria.(2) Pathology:the light microscope lesions in MCD-IgA and MCD-NS group were mild,but it was usually associated with severe histologic lesions in NS-IgAN,such as endocapillary proliferation,segmental sclerosis,crescent formation,tuft necrosis and chronic tubulointerstitial lesions;in MCD -NS group,immunofluorescence was negative. In MCD -IgA group,IgA deposition intensity was weak(less than + +),and 3 cases(30.0%)were accompanied with C3deposi-tion.In NS-IgAN group,IgA deposition intensity was stronger(more than + + +),and most of the cases were accom-panied with C3and other immunoglobulins deposition.Under electron microscope,both MCD-IgA and MCD-NS showed wide foot process effacement,and a small amount of mesangial electron dense deposit was detected in 9 cases of MCD-IgA.In NS-IgAN group,large amount of electron dense deposit was found in the mesangial region,and only 8 cases (38.0%)showed more than 50% of foot process effacement.(3)Prognosis:in MCD-IgA group,9 patients were ster-oid-dependent or frequently relapsed,1 case showed steroid-resistance,6 patients required additional agents.Except 1 case lost,with an average of(61.5 ± 28.8)months were followed up,8 patients achieved complete remission;In MCD-NS group,20 cases were steroid-dependent or frequently relapsed,4 cases were steroid-resistant,23 cases re-quired additional immunosuppressive agents.Followed up for an average of(36.4 ± 12.5)months,22 cases(91.7%) achieved complete remission;In NS-IgAN group,all cases were steroid-resistant and combined with cyclophospha-mide treatment;followed up for an average of(38.6 ± 15.2)months,19 cases(90.5%)achieved complete remission. Conclusions The clinical manifestations and prognosis of MCD-IgA were similar to MCD-NS,but the clinical and pathological findings of MCD-IgA were different from those of NS-IgAN.It is deduced that the nature of MCD-IgA is still a MCD,and that the IgA deposition may be nonspecific.
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OBJECTIVE: To explore the clinicopathological characteristics and therapeutic approach for mercury poisoningassociated nephritic syndrome. METHODS: Clinical and pathological data of 9 patients diagnosed with mercury poisoningassociated nephritic syndrome were analyzed. RESULTS: Among the 9 cases,2 cases were mercury smelters,1 case was instrument production worker,1 case was traditional Chinese medicine preparation worker,4 cases were psoriasis treated by folk prescription of traditional Chinese medicine,and 1 case used cosmetics for whitening and freckle removal. All cases showed clinical manifestation of nephritic syndrome that included large amount of proteinuria,hypoalbuminemia,edema and hyperlipidemia. The concentrations of urine mercury in the patients were from 0. 09 to 0. 75 mg / L before treatment.Pathology examination on renal biopsy showed that there were 4 cases of minimal change nephropathy and 5 cases of membranous nephropathy at stage Ⅰ. The patients with nephritic syndrome were cured after mercury driving and treatment with corticosteroid. CONCLUSION: The kidney histopathology changes in patients with mercury poisoning-associated nephritic syndrome are primarily characterized by minimal change and membranous nephropathy. The mercury driving combined treatment with corticosteroid can completely relieve the clinical symptoms of the patients with mercury poisoningassociated nephritic syndrome.
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Objective To screen early urine protein markers for minimal change nephropathy.Methods Adriamycin nephropathy was employed as minimal change nephropathy model.Urinary protein and ConA captured glycoproteins were respectively profiled.Results By profiling urine proteome,25 differential proteins were identified.These differential proteins were from leaked plasma proteins,secreted proteins from immuno-and inflammatory cells,specifically asecreted proteins from urinary tract,and so on.They took part in different pathogenic process,eg.hemodynamic changes,podocytes injury,immunological disorder and so on.By profiling ConA-enriched urinary glycoproteome,21 differential proteins were identified,among which 12(57%) were different from the above 25 differential proteins.This indicates that the knowledge of urine glycoproteome is complementary to urine proteome in understanding kidney condition.Conclusion These differential proteins can be potential indicators of minimal change nephropathy,and can help better understand the pathogenesis by further studying their functions.
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Objective To investigate the expression of nestin, a type Ⅵ intermediate filament protein in the glomeruli with foot process effacement and the potential relationship between nestin expression in the kidney and the degree of proteinuria. Method Immunohistochemistry was used to determine the localization of nestin in the kidney samples obtained from needle biopsies of normal human and patients with minimal change disease (MCD). Puromycin aminonucleoside (PAN) nephrosis rat models were established by a single intraperitoneal injection of PAN. Both real time quatitative reverse PCR and Western blot methods were applied to evaluate the levels of nestin expression at day 1, 4, 10 and 20 after PAN injection. Results Immunohistochemistry showed that the expression of nestin in glomeruli of MCD patients was significantly reduced compared with normal samples (0.93±0.08 vs 1.65±0.12, P<0.05) . The mRNA and protein expressions of nestin in the rat kidney were transitorily increased by 1.23 folds and 1.48 folds of control group (NC) after 1 day of PAN injection (P<0.05), then decreased quickly in the following days. The mRNA levels of nestin in the kidney were 35.8% and 12.1% of NC after 4 days and 10 days of PAN injection, respectively, (P<0.01) as determined by real time PCR. After 20 days of PAN injury, nestin mRNA expression partly recovered to 65.8% of NC (P< 0.05 ). The protein levels of nestin detected by Western blot presented the similar trend, which were 77.0%, 58.0% and 83.4% of NC after 4 days, 10 days and 20 days of PAN injection, respectively (P<0.05). The degree of proteinuria in puromycin aminonucleoside nephrosis rats was negatively correlated with both mRNA and protein levels of nestin in the kidney(r=-0.667,P<0.05 and r=-0.621 ,P<0.05, respectively). Conclusions The expression of intermediate filament protein nestin is down-regnlated in the kidney characterized with foot process effacement and negatively correlated with the degree of proteinuria in puromycin aminonucleoside nephrosis rats. Nestin may play a potential role in modulating the structure and function of podocyte.
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Nephrotic syndrome is a relatively rare complication of malignancy. A few cases of nephrotic syndrome accompanying Hodgkin's disease, non-Hodgkin lymphoma, leukemia and other malignancies have been reported since the first case of the nephrotic syndrome associated with extrarenal malignancy was reported in 1922. Hodgkin's disease and solid tumors are known to be the most common malignancies accompanying nephrotic syndrome. The pathologic findings of kidney in patients with Hodgkin's disease commonly show minimal change nephropathy. Membranous glomerulonephropathy is the most common pathologic feature in patients with solid tumors. Although membranous glomerulonephropathy related to small cell lung cancer has rarely been reported in Korea, minimal change nephropathy accompanying small cell lung cancer has never been reported. We present here a case of a 70 year-old male with minimal change nephropathy that was related to small cell lung cancer. We detected small cell lung cancer during the diagnosis work-up of nephrotic syndrome. We suggest that nephrotic syndrome can be a manifestation of underlying malignancy.