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Sample records for childhood iga nephropathy

  1. IgA Nephropathy

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    ... reliable ways to diagnose IgA nephropathy; therefore, the diagnosis of IgA nephropathy requires a kidney biopsy. A kidney biopsy is ... reliable ways to diagnose IgA nephropathy; therefore, the diagnosis of IgA nephropathy requires a kidney biopsy. Researchers have not yet ...

  2. Association between polymorphisms in Interleukin-17 receptor A gene and childhood IgA nephropathy

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    Seung-Ah Baek

    2010-02-01

    Full Text Available Purpose : Interleukin-17 (IL-17 is produced by activated CD4+T cells and exhibits pleiotropic biological activity on various cell types. IL-17 was reported to be involved in the immunoregulatory response in IgA nephropathy (IgAN. Our aim was to investigate the association between single-nucleotide polymorphisms (SNPs in IL-17 receptor A (IL-17RA gene and childhood IgAN. Methods : We analyzed the SNPs in the IL-17RA in 156 children with biopsy-proven IgAN and 245 healthy controls. We divided the IgAN patients into 2 groups and compared them with respect to proteinuria (?#180; and >4 mg/m2/h, ?#180;0 and >40 mg/m2/h, respectively and the presence of pathological levels of biomarkers of diseases such as interstitial fibrosis, tubular atrophy, or global sclerosis. Results : No difference was observed between the SNP genotypes rs2895332, rs1468488, and rs4819553 between IgAN patients and control subjects. In addition, no significant difference was observed between allele frequency of SNPs rs2895 332, rs1468488, and rs4819553 between patients in the early and advanced stage of the disease. However, significant difference was observed between the genotype of SNP rs2895332 between patients with proteinuria (>4 mg/m2/h and those without proteinuria (codominant model OR 0.36, 95% CI 0.19–0.66, P<0.001; dominant model OR 0.35, 95% CI 0.17–0.69 P=0.002; recessive model OR 0.12, 95% CI 0.01–1.06 P=0.025. Conclusion : Our results indicate that the SNP in IL-17RA (rs2895332 may be related to the development of proteinuria in IgAN patients.

  3. Crescentic IgA nephropathy.

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    Abuelo, J G; Esparza, A R; Matarese, R A; Endreny, R G; Carvalho, J S; Allegra, S R

    1984-11-01

    We report five cases of crescentic IgA nephropathy. All are males, 16-60 years of age. One case each came to medical attention with uremia, nephrotic syndrome, and gross hematuria; two cases presented with microhematuria and proteinuria on routine urinalysis. All had hypertension, azotemia (serum creatinine 1.6-9.4 mg/dl), proteinuria (greater than 6 g/24 hr in four cases), hypoalbuminemia (less than 3 g/dl), and hematuria (gross in two cases). All progressed to end-stage renal failure renal failure ending in dialysis (three cases) or death from unrelated causes (two cases). Prednisone, 60 mg/day for 1 month in two patients (with two 1-g doses of iv methylprednisolone in 1 case) did not improve the serum creatinine level, but one patient subsequently experienced a less rapid fall in renal function. A crescentic glomerulonephritis was present in all biopsies (crescents in 31-80% of glomeruli; mean, 50%). The size and stage of the crescents were variable. Numerous glomeruli had focal or diffuse sclerosis. In all cases, there was a 3 or 4+ deposition of IgA. Low-intensity staining for IgG and IgM was noted in four and three patients, respectively. On electron microscopy, dense granular mesangial deposits were noted in all cases and in four patients capillary subepithelial deposits were also observed. This form of IgA nephropathy is not common, but some studies indicate that it may occur in about 5% of patients with IgA nephropathy.

  4. Childhood Henoch-Sch(o)nlein Purpura Nephritis and IgA Nephropathy:One Disease Entity?--A Clinico-pathologically Comparative Study

    Institute of Scientific and Technical Information of China (English)

    2005-01-01

    In order to characterize their relationship through clinicopathological comparison between IgA nephropathy and Henoch-Schonlein purpura nephritis (HSPN), 31 children with IgA nephropathy aged between 3 to 15 years and 120 children with HSPN aged between 4 to 15 years were compared with each other in clinical manifestation, blood biochemistry, serum immunology and followup study. Renal pathological findings under light microscope, immunofluorescence and electronic microscope were analyzed and also compared between 31 children with IgA nephropathy and 32 biopsied children with HSPN. The results showed that the onset age was over 12 years in 25.8 %children with IgA nephropathy, but only 10 % in HSPN (P<0.05). The clinical patterns of IgA nephropathy and HSPN were similar, but extra-renal manifestations were more often in HSPN, all of them had skin purpura, 59 % had gastrointestinal symptoms and 47 % suffered from arthralgia,compared with only abdominal pain in 3.2 % children with IgA nephropathy. The renal pathological investigation showed global sclerosis in 35.5 % of IgA nephropathy and 3.1% of HSPN, mesangial sclerosis in 41.9 % of IgA nephropathy and 6.3 % of HSPN, but endothelial proliferation in 65.6% of HSPN and 29 % of IgA nephropathy (all P<0.01). Thin basement membrane nephropathy was only found in 6.5 % children with IgA nephropathy, no in HSPN. The electronic dense deposits in HSPN were sparse, loose and wildly spread in glomerular mesangium, subendothelial area and even intra basement membrane, but it was dense, lumpy and mostly limited in mesangium and paramesangium in IgA nephropathy. Predominant IgA deposits were found in 81.2 %of HSPN, and overwhelming IgG deposits in 12.5 % of HSPN with relatively weak IgA deposits,moreover 6.3 % of HSPN showed linear IgG deposits in glomerular capillary. Totally 71.9 % of HSPN had IgG deposits in glomeruli and only 19.4 % of IgA nephropathy showed glomerular IgG deposits (P<0.01). No IgG deposit was observed

  5. Immune regulation in IgA nephropathy

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    Eijgenraam, Jan-Willem

    2008-01-01

    IgA nephropathy (IgAN) is the most common form of glomerulonephritis worldwide. The hallmark of the disease is depositions of polymeric IgA1 in the mesangium of the glomeuli. These depositions will lead to inflammation in the kidneys and eventually to deterioration of renal function. The pathogenesi

  6. Role of complement in IgA nephropathy.

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    Daha, Mohamed R; van Kooten, Cees

    2016-02-01

    Immunoglobulin A nephropathy (IgAN) is characterized by the deposition of IgA in the mesangium of glomeruli. This mesangial IgA has been found to consist mainly of polymeric IgA1 which drives the activation of the mesangial cells and results in excessive production of several inflammatory mediators. The activation of mesangial cells is amplified by the ability of IgA to activate the complement system, originally thought to occur mainly via the alternative pathway of complement. However more recent studies indicate that lectin pathway involvement has a strong association with progression of renal disease. In this review we summarize the contribution of complement to the IgA- mediated inflammatory process.

  7. Diagnosis and treatment of primary glomerular diseases Membranous nephropathy, focal segmental glomerulosclerosis and IgA nephropathy.

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    Deegens, J.K.J.; Wetzels, J.F.M.

    2005-01-01

    Membranous nephropathy, focal segmental glomerulosclerosis (FSGS) and IgA nephropathy are the most frequent and important primary glomerulopathies. Idiopathic membranous nephropathy and primary FSGS usually present with a nephrotic syndrome with or without renal insufficiency, whereas IgA nephropath

  8. IgA nephropathy: Causes, prognosis and treatment

    Institute of Scientific and Technical Information of China (English)

    Francesco Paolo Schena; Diletta Domenica Torres; Giuseppina Cerrullo

    2005-01-01

    @@ IgA nephropathy (IgAN) or Berger's disease is the most common form of primary glomerulonephritis in many renal biopsy registries and it is very frequent in the Eastern regions of the world, such as China (32,1%), Hong Kong (35%), Japan (47,4% )[1,2].

  9. Impact of the -675 4G/5G polymorphism of the plasminogen activator inhibitor-1 gene on childhood IgA nephropathy.

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    Han, Su-Ryun; Kim, Cheon-Jong; Lee, Byung-Cheol

    2012-04-01

    Plasminogen activator inhibitor-1 (PAI-1) is an important regulator of the fibrinolytic pathway and extracellular matrix (ECM) turnover. The -675 4G/5G polymorphism in the PAI-1 promoter is associated with altered PAI-1 transcription, suggesting that this polymorphism may be a candidate risk factor for diseases characterized by ECM accumulation, such as immunoglobulin A nephropathy (IgAN) and mesangial proliferative glomerulonephritis (MesPGN). We genotyped childhood patients with biopsy-confirmed IgAN (n=111) and MesPGN (n=47), and healthy control subjects (n=230) for the -675 4G/5G PAI-1 polymorphism by polymerase chain reaction-restriction fragment length polymorphism methods. The distribution of the 4G/4G (27.9%), 4G/5G (45.1%) and 5G/5G (27.0%) genotypes in IgAN patients was significantly different from the healthy controls (32.2, 54.3 and 13.5%, respectively) (p=0.0092). There was no significant difference in the genotype distributions of the 4G/5G polymorphism between MesPGN patients and the healthy controls. Regarding the impact of the polymorphism on IgAN, the 4G/4G genotype was markedly increased in patients with proteinuria (≥1,000 mg/day) and/or hypertension when compared to patients without proteinuria and hypertension (OR=5.23, 95% CI 1.34-20.38, P=0.0183). These findings indicate that the PAI-1 gene polymorphism may affect the susceptibility of childhood IgAN.

  10. Minimal change disease versus IgA nephropathy.

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    Jabur, Wael Latif

    2009-01-01

    IgA nephropathy is the most common type of the glomerulonephritis all over the world. However, its clinical presentation is variable, as is the underlying histopathological lesion. We report herein a case of an adult with steroid responsive minimal change disease and IgA mesangial deposits. During the first two weeks of therapy with prednisolone, the patient reported dramatic improvement in his clinical condition and remitted his disease. Unfortunately, at the end of the second month of prednisolone therapy, an acute flare of viral hepatitis was diagnosed. Interes-tingly, the acute viral flare was without a concomitant relapse of proteinuria.

  11. Minimal change disease versus IgA nephropathy

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    Jabur Wael

    2009-01-01

    Full Text Available IgA nephropathy is the most common type of the glomerulonephritis all over the world. However, its clinical presentation is variable, as is the underlying histopathological lesion. We report herein a case of an adult with steroid responsive minimal change disease and IgA mesangial deposits. During the first two weeks of therapy with prednisolone, the patient reported dramatic improvement in his clinical condition and remitted his disease. Unfortunately, at the end of the second month of prednisolone therapy, an acute flare of viral hepatitis was diagnosed. Interes-tingly, the acute viral flare was without a concomitant relapse of proteinuria.

  12. Bilateral scleritis and sclerokeratitis associated with IgA nephropathy

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    Garza-Leon, Manuel; Flores, Diana; Alarcón-Galván, Gabriela; Sánchez-Martínez, Concepción

    2012-01-01

    Purpose The purpose of this study is to report a case of bilateral nodular scleritis in a patient with final diagnosis of IgA nephropathy. Methods This is an observational case report. Results A male patient, 42 years old, presented with a bilateral nodular scleritis and OD sclerokeratitis. He had a previous history of acute otitis media and developed posterior renal failure and arterial hypertension. Clinical and systemic findings suggest Wegener's granulomatosis. A kidney biopsy was perform...

  13. The association of single nucleotide P-selectin gene polymorphism with IgA nephropathy

    Institute of Scientific and Technical Information of China (English)

    王朝晖

    2006-01-01

    Objective IgA nephropathy is one of the most com- mon form of primary glomerulonephritis throughout the world and a main renal disease which causes renal failure. P-selectin plays an important role in the pathogenesis and development of IgA nephropathy. The purpose of this study is to find a possible relationship between P-selectin gene polymorphism and IgA nephropathy. Methods In this study,a comprehensive P-selectin gene sur-

  14. Efficacy and safety of mycophenolate mofetil treatment in IgA nephropathy: a systematic review

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    Chen, Youyuan; Li, Yumin; Yang, ShengLin; Li, Yan; Liang, Min

    2014-01-01

    Background IgA nephropathy is the most common primary glomerular disease worldwide and also the most frequent cause of kidney failure. Mycophenolate mofetil (MMF) is a selective immunosuppressant widely used in many autoimmune diseases. However, the benefits and risks of MMF for the treatment of IgA nephropathy remain uncertain. Methods A systematic review and meta-analysis of randomized controlled trials (RCTs) was performed to assess the efficacy and safety of MMF in IgA nephropathy patient...

  15. Nefropatia por IgA nas espondiloartrites IgA nephropathy in spondyloarthritis

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    Daniela Castelo Azevedo

    2011-02-01

    Full Text Available Pacientes com espondiloartrites poderiam ser mais acometidos pela nefropatia por IgA do que a população geral, havendo, possivelmente, um mecanismo etiopatogênico comum. O seguinte artigo relaciona quatro casos que exemplificam essa possível associaçãoSpondyloarthritis patients can be more frequently affected by IgA nephropathy than the general population, and a common etiopathogenic mechanism can be involved. We report four cases that may exemplify that association

  16. Gluten and IgA nephropathy: you are what you eat?

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    Cheung, Chee Kay; Barratt, Jonathan

    2015-08-01

    Although extensively studied, the relationship between dietary antigens-in particular, gluten-and IgA nephropathy remains unclear. Using a double transgenic mouse model of IgA nephropathy that expresses both human IgA1 and human CD89, Papista et al. report that a gluten-free diet protects against the development of IgA deposition and glomerular injury, and that these events occur with the introduction of dietary gluten.

  17. Diagnosis and treatment of patients with IgA nephropathy in Japan

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    Yasuhiko Tomino

    2016-12-01

    Full Text Available Chronic kidney disease (CKD is a worldwide public health problem that affects millions of people from all racial and ethnic groups. Although CKD is not one specific disease, it is a comprehensive syndrome that includes IgA nephropathy. As reported by the Japanese Society of Nephrology, 13.0 million people have CKD. In Japan, major causes of end-stage kidney disease are type 2 diabetic nephropathy, chronic glomerulonephritis, especially IgA nephropathy, hypertensive nephrosclerosis, and polycystic kidney disease. IgA nephropathy is characterized by polymeric IgA1 with aberrant galactosylation (galactose-deficient IgA1 increased in the blood and deposited in the glomerular mesangial areas, as well as partially in the capillary walls. The tonsils are important as one of the responsible regions in this disease. The clarification of the mechanism of galactose-deficient IgA1 production will pave the way for the development of novel therapies. The results of future research are eagerly awaited. At present, the most important therapeutic goals in patients with IgA nephropathy are the control of hypertension, the decrease of urinary protein excretion, and the inhibition of progression to end-stage kidney disease. Several investigators have reported that renin–angiotensin–aldosterone system inhibitors reduce levels of urinary protein excretion and preserve renal function in patients with IgA nephropathy. In Japan, tonsillectomy and steroid pulse therapy are more effective for patients with IgA nephropathy.

  18. An update on pathology of IgA nephropathy.

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    Soares, Maria Fernanda

    2016-12-01

    IgA Nephropathy (IgAN) is the commonest of the glomerular diseases in the world. Its progression rate of 30-40% of the cases em 20-30 years makes IgAN an important healthcare issue in Nephrology. Diagnosis of IgAN depends on biopsy findings, particularly at immunofluorescence microscopy. The frequence of IgAN diagnosis is variable in different populations and depends on screening and biopsy indication policies. IgAN pathogenesis is considered multifactorial; its primordial defect is the production of galactosis-deficient IgA molecules. This review paper discusses the most uptodate aspects of the pathogenesis, pathological classification and clinical implications of IgAN.

  19. An update on pathology of IgA nephropathy

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    Maria Fernanda Soares

    Full Text Available Abstract IgA Nephropathy (IgAN is the commonest of the glomerular diseases in the world. Its progression rate of 30-40% of the cases em 20-30 years makes IgAN an important healthcare issue in Nephrology. Diagnosis of IgAN depends on biopsy findings, particularly at immunofluorescence microscopy. The frequence of IgAN diagnosis is variable in different populations and depends on screening and biopsy indication policies. IgAN pathogenesis is considered multifactorial; its primordial defect is the production of galactosis-deficient IgA molecules. This review paper discusses the most uptodate aspects of the pathogenesis, pathological classification and clinical implications of IgAN.

  20. Lupus vulgaris with tubercular lymphadenitis and IgA nephropathy.

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    Khaira, Ambar; Rathi, Om P; Mahajan, Sandeep; Sharma, Alok; Dinda, Amit K; Tiwari, Suresh C

    2008-02-01

    A 14-year-old girl presented with a 10-year history of a large crusted plaque over the right thigh for 10 years and small reddish plaque over the left upper back for 3 months. On routine evaluation, she was found to have hematuria. Skin biopsy from the lesion was suggestive of skin tuberculosis (lupus vulgaris), and kidney biopsy showed features of IgA nephropathy (IgAN). Fine-needle aspiration from the inguinal lymph node was consistent with granulomatous disease. The patient has been on anti-tubercular treatment, and the hematuria has subsided.

  1. Association of liver cirrhosis related IgA nephropathy with portal hypertension.

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    Kalambokis, Georgios; Christou, Leonidas; Stefanou, Dimitrios; Arkoumani, Evdokia; Tsianos, Epameinondas V

    2007-11-21

    A high incidence of IgA nephropathy has been reported in patients with liver cirrhosis, though, clinically evident nephrotic syndrome is very uncommon. Impaired hepatic clearance of circulating IgA immune complexes and subsequent deposition in renal glomeruli has been considered principally in the pathogenesis of liver cirrhosis associated IgA nephropathy. Here we report on a patient with cryptogenic liver cirrhosis and splenic vein thrombosis, who presented with nephrotic syndrome. Renal biopsy showed findings consistent with IgA nephropathy. Lower endoscopy showed features of portal hypertensive colopathy. Following initiation of propranolol and anticoagulant treatment to reduce portal pressure, a gradual decrease of proteinuria and hematuria to normal range was noted. The potential pathogenetic role of portal hypertension in the development of IgA nephropathy in cirrhotic patients is discussed.

  2. Association of liver cirrhosis related IgA nephropathy with portal hypertension

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    A high incidence of IgA nephropathy has been reported in patients with liver cirrhosis, though, clinically evident nephrotic syndrome is very uncommon. Impaired hepatic clearance of circulating IgA immune complexes and subsequent deposition in renal glomeruli has been considered principally in the pathogenesis of liver cirrhosis associated IgA nephropathy. Here we report on a patient with cryptogenic liver cirrhosis and splenic vein thrombosis, who presented with nephrotic syndrome. Renal biopsy showed findings consistent with IgA nephropathy. Lower endoscopy showed features of portal hypertensive colopathy. Following initiation of propranolol and anticoagulant treatment to reduce portal pressure, a gradual decrease of proteinuria and hematuria to normal range was noted. The potential pathogenetic role of portal hypertension in the development of IgA nephropathy in cirrhotic patients is discussed.

  3. Proteomic analysis of urinary exosomes from patients of early IgA nephropathy and thin basement membrane nephropathy.

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    Moon, Pyong-Gon; Lee, Jeong-Eun; You, Sungyong; Kim, Taek-Kyun; Cho, Ji-Hoon; Kim, In-San; Kwon, Tae-Hwan; Kim, Chan-Duck; Park, Sun-Hee; Hwang, Daehee; Kim, Yong-Lim; Baek, Moon-Chang

    2011-06-01

    To identify biomarker candidates associated with early IgA nephropathy (IgAN) and thin basement membrane nephropathy (TBMN), the most common causes presenting isolated hematuria in childhood, a proteomic approach of urinary exosomes from early IgAN and TBMN patients was introduced. The proteomic results from the patients were compared with a normal group to understand the pathophysiological processes associated with these diseases at the protein level. The urinary exosomes, which reflect pathophysiological processes, collected from three groups of young adults (early IgAN, TBMN, and normal) were trypsin-digested using a gel-assisted protocol, and quantified by label-free LC-MS/MS, using an MS(E) mode. A total of 1877 urinary exosome proteins, including cytoplasmic, membrane, and vesicle trafficking proteins, were identified. Among the differentially expressed proteins, four proteins (aminopeptidase N, vasorin precursor, α-1-antitrypsin, and ceruloplasmin) were selected as biomarker candidates to differentiate early IgAN from TBMN. We confirmed the protein levels of the four biomarker candidates by semi-quantitative immunoblot analysis in urinary exosomes independently prepared from other patients, including older adult groups. Further clinical studies are needed to investigate the diagnostic and prognostic value of these urinary markers for early IgAN and TBMN. Taken together, this study showed the possibility of identifying biomarker candidates for human urinary diseases using urinary exosomes and might help to understand the pathophysiology of early IgAN and TBMN at the protein level.

  4. Aberrantly Glycosylated IgA1 as a Factor in the Pathogenesis of IgA Nephropathy

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    Mototsugu Tanaka

    2011-01-01

    Full Text Available Predominant or codominant immunoglobulin (Ig A deposition in the glomerular mesangium characterizes IgA nephropathy (IgAN. Accumulated glomerular IgA is limited to the IgA1 subclass and usually galactose-deficient. This underglycosylated IgA may play an important role in the pathogenesis of IgAN. Recently, antibodies against galactose-deficient IgA1 were found to be well associated with the development of IgAN. Several therapeutic strategies based on corticosteroids or other immunosuppressive agents have been shown to at least partially suppress the progression of IgAN. On the other hand, several case reports of kidney transplantation or acquired IgA deficiency uncovered a remarkable ability of human kidney to remove mesangial IgA deposition, resulting in the long-term stabilization of kidney function. Continuous exposure to circulating immune complexes containing aberrantly glycosylated IgA1 and sequential immune response seems to be essential in the disease progression of IgAN. Removal of mesangial IgA deposition may be a challenging, but fundamental approach in the treatment of IgAN.

  5. A case of rapidly progressive IgA nephropathy in a patient with exacerbation of Crohn’s disease

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    Choi Ji-Young; Yu Chung; Jung Hee-Yeon; Jung Min; Kim Yong-Jin; Cho Jang-Hee; Kim Chan-Duck; Kim Yong-Lim; Park Sun-Hee

    2012-01-01

    Abstract Background IgA nephropathy has been reported as a renal involvement in Crohn’s disease. Crescentic IgA nephropathy, which accounts for fewer than 5% of cases of IgA nephropathy, has a poorer prognosis than other forms of crescentic glomerulonephritis. We recently experienced a case of rapidly progressive IgA nephropathy concurrent with exacerbation of Crohn’s disease. Case presentation An 18-year-old male diagnosed with Crohn’s disease underwent a hemicolectomy 2 years prior previous...

  6. Current Understanding of the Role of Complement in IgA Nephropathy

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    Maillard, Nicolas; Wyatt, Robert J.; Julian, Bruce A.; Kiryluk, Krzysztof; Gharavi, Ali; Fremeaux-Bacchi, Veronique

    2015-01-01

    Complement activation has a role in the pathogenesis of IgA nephropathy, an autoimmune disease mediated by pathogenic immune complexes consisting of galactose-deficient IgA1 bound by antiglycan antibodies. Of three complement-activation pathways, the alternative and lectin pathways are involved in IgA nephropathy. IgA1 can activate both pathways in vitro, and pathway components are present in the mesangial immunodeposits, including properdin and factor H in the alternative pathway and mannan-binding lectin, mannan–binding lectin–associated serine proteases 1 and 2, and C4d in the lectin pathway. Genome–wide association studies identified deletion of complement factor H–related genes 1 and 3 as protective against the disease. Because the corresponding gene products compete with factor H in the regulation of the alternative pathway, it has been hypothesized that the absence of these genes could lead to more potent inhibition of complement by factor H. Complement activation can take place directly on IgA1–containing immune complexes in circulation and/or after their deposition in the mesangium. Notably, complement factors and their fragments may serve as biomarkers of IgA nephropathy in serum, urine, or renal tissue. A better understanding of the role of complement in IgA nephropathy may provide potential targets and rationale for development of complement-targeting therapy of the disease. PMID:25694468

  7. SIGNIFICANCE OF P- SELECTIN EXPRESSION IN IGA NEPHROPATHY

    Institute of Scientific and Technical Information of China (English)

    吴珮; 周同; 李晓; 王伟铭; 陈楠; 董德长

    2000-01-01

    Objective To investigate the role of P-selectin in IgA nephropathy (IgAN). Methods Plasma P-selectin level was measured by ELISA and P-selectin expression in renal tissue was detected by immunohistochemistry and in situ hybridization in 45 patients with IgAN. Results Plasma P-selectin levels in the patients with IgAN were significantly higher than those in the controls. In IgAN, the levels in the patients with nephrotic syndrome or renal function insufficiency were much higher than those in the patients with gross hematuria, abnormal urine analyses or nephritis syndrome. P- selectin was widely expressed within renal tissue in IgAN. Glomerular P- selectin expression was remarkably up - regulated in grade Ⅳ and Ⅴ of IgAN than that in grade Ⅱ and Ⅲ. Moreover, the expression of P- selectin on tubular epithelium or within interstitium was strongly associated with the degree of tubulointerstitial lesions. Conclusion The results suggested that P- selectin might play an important role in IgAN, and the level of P - selectin in plasma and renal tissue might predict the progress of IgAN.

  8. Expression of prorenin receptor in renal biopsies from patients with IgA nephropathy.

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    Miyazaki, Nagisa; Murata, Ichijiro; Takemura, Genzou; Okada, Hideshi; Kanamori, Hiromitsu; Matsumoto-Miyazaki, Jun; Yoshida, Gakuro; Izumi, Kumiko; Kashi, Hitomi; Niimi, Kaori; Nishiwaki, Ayuko; Miyazaki, Tatsuhiko; Ohno, Michiya; Ohashi, Hiroshige; Suzuki, Fumiaki; Minatoguchi, Shinya

    2014-01-01

    Prorenin receptor (PRR) has been implicated in the onset and progression of various renal diseases, though its possible association with immunoglobulin A (IgA) nephropathy remains unclear. In the present study, we tried to clarify expression and pathophysiological significance of PRR in IgA nephropathy. We immunohistochemically assessed PRR levels in renal biopsy specimens from 48 patients with IgA nephropathy and evaluated its relevance to the clinical and pathological features of the disease. PRR was detected mainly in renal tubular cells, which was confirmed at the subcellular level using immunoelectron microscopy. The PRR-positive area (%PRR area) correlated with daily urinary protein, which is known to reflect disease severity (r=0.286, P=0.049). PRR levels were weaker in tubular cells bordering areas of severe interstitial fibrosis, where α-smooth muscle actin-positive myofibroblasts were present. We also used immunohistochemical detection of microtubule-associated protein-1 light chain 3 (LC3) and electron microscopy to assess autophagy, a cytoprotective mechanism downstream of PRR. We noted an apparent coincidence between autophagy activation in tubular cells and PRR expression in the same cells. Taken together, our findings suggest that renal expression of PRR in IgA nephropathy may be a compensatory response slowing disease progression by preventing tubular cell death and subsequent fibrosis through activation of cytoprotective autophagic machinery. Further studies using different type of kidney diseases could draw conclusion if the present finding is a generalized observation beyond IgA nephropathy.

  9. Role of glomerular proteoglycans in IgA nephropathy.

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    Ebefors, Kerstin; Granqvist, Anna; Ingelsten, Madeleine; Mölne, Johan; Haraldsson, Börje; Nyström, Jenny

    2011-04-06

    Mesangial matrix expansion is a prominent feature of the most common form of glomerulonephritis, IgA nephropathy (IgAN). To find molecular markers and improve the understanding of the disease, the gene and protein expression of proteoglycans were investigated in biopsies from IgAN patients and correlated to clinical and morphological data. We collected and microdissected renal biopsies from IgAN patients (n = 19) and from healthy kidney donors (n = 14). Patients were followed for an average time of 4 years and blood pressure was according to target guidelines. Distinct patterns of gene expression were seen in glomerular and tubulo-interstitial cells. Three of the proteoglycans investigated were found to be of special interest and upregulated in glomeruli: perlecan, decorin and biglycan. Perlecan gene expression negatively correlated to albumin excretion and progress of the disease. Abundant decorin protein expression was found in sclerotic glomeruli, but not in unaffected glomeruli from IgAN patients or in controls. Transforming growth factor beta (TGF-β), known to interact with perlecan, decorin and biglycan, were upregulated both on gene and protein level in the glomeruli. This study provides further insight into the molecular mechanisms involved in mesangial matrix expansion in IgAN. We conclude that perlecan is a possible prognostic marker for patients with IgAN. In addition, the up-regulation of biglycan and decorin, as well as TGF-β itself, indicate that regulation of TGF-β, and other profibrotic markers plays a role in IgAN pathology.

  10. Urinary uromodulin excretion predicts progression of chronic kidney disease resulting from IgA nephropathy.

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    Jingjing Zhou

    Full Text Available BACKGROUND: Uromodulin, or Tamm-Horsfall protein, is the most abundant urinary protein in healthy individuals. Recent studies have suggested that uromodulin may play a role in chronic kidney diseases. We examined an IgA nephropathy cohort to determine whether uromodulin plays a role in the progression of IgA nephropathy. METHODS: A total of 344 IgA nephropathy patients were involved in this study. Morphological changes were evaluated with the Oxford classification of IgA nephropathy. Enzyme Linked Immunosorbent Assay (ELISA measured the urinary uromodulin level on the renal biopsy day. Follow up was done regularly on 185 patients. Time-average blood pressure, time-average proteinuria, estimated glomerular filtration rate (eGFR and eGFR decline rate were caculated. Association between the urinary uromodulin level and the eGFR decline rate was analyzed with SPSS 13.0. RESULTS: We found that lower baseline urinary uromodulin levels (P = 0.03 and higher time-average proteinuria (P = 0.04 were risk factors for rapid eGFR decline in a follow-up subgroup of the IgA nephropathy cohort. Urinary uromodulin level was correlated with tubulointerstitial lesions (P = 0.016. Patients that had more tubular atrophy/interstitial fibrosis on the surface had lower urinary uromodulin levels (P = 0.02. CONCLUSIONS: Urinary uromodulin level is associated with interstitial fibrosis/tubular atrophy and contributes to eGFR decline in IgA nephropathy.

  11. Rapamycin ameliorates IgA nephropathy via cell cycle-dependent mechanisms.

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    Tian, Jihua; Wang, Yanhong; Liu, Xinyan; Zhou, Xiaoshuang; Li, Rongshan

    2015-07-01

    IgA nephropathy is the most frequent type of glomerulonephritis worldwide. The role of cell cycle regulation in the pathogenesis of IgA nephropathy has been studied. The present study was designed to explore whether rapamycin ameliorates IgA nephropathy via cell cycle-dependent mechanisms. After establishing an IgA nephropathy model, rats were randomly divided into four groups. Coomassie Brilliant Blue was used to measure the 24-h urinary protein levels. Renal function was determined using an autoanalyzer. Proliferation was assayed via Proliferating Cell Nuclear Antigen (PCNA) immunohistochemistry. Rat mesangial cells were cultured and divided into the six groups. Methylthiazolyldiphenyl-tetrazolium bromide (MTT) and flow cytometry were used to detect cell proliferation and the cell cycle phase. Western blotting was performed to determine cyclin E, cyclin-dependent kinase 2, p27(Kip1), p70S6K/p-p70S6K, and extracellular signal-regulated kinase 1/2/p- extracellular signal-regulated kinase 1/2 protein expression. A low dose of the mammalian target of rapamycin (mTOR) inhibitor rapamycin prevented an additional increase in proteinuria, protected kidney function, and reduced IgA deposition in a model of IgA nephropathy. Rapamycin inhibited mesangial cell proliferation and arrested the cell cycle in the G1 phase. Rapamycin did not affect the expression of cyclin E and cyclin-dependent kinase 2. However, rapamycin upregulated p27(Kip1) at least in part via AKT (also known as protein kinase B)/mTOR. In conclusion, rapamycin can affect cell cycle regulation to inhibit mesangial cell proliferation, thereby reduce IgA deposition, and slow the progression of IgAN.

  12. Cholestatic jaundice and IgA nephropathy induced by OTC muscle building agent superdrol.

    Science.gov (United States)

    Jasiurkowski, Beata; Raj, Jaya; Wisinger, David; Carlson, Richard; Zou, Lixian; Nadir, Abdul

    2006-11-01

    Over the counter (OTC) medicines are commonly used in the United States despite a lack of scientific evidence for clinical utility and toxicity associated with their use. A case of jaundice and IgA nephropathy as a consequence of use of a muscle enhancing OTC supplement that was advertised as innocuous with no hormonal activity is described. IgA nephropathy has not been described previously in association with the use of testosterone. The case highlights that, besides adulteration, the misrepresentation of chemicals present in OTC medications and supplements can create confusion and a false sense of security with their use.

  13. The coincidence of IgA nephropathy and Fabry disease

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    Maixnerová Dita

    2013-01-01

    Full Text Available Abstract Background IgA nephropathy (IgAN is the most common glomerulonephritis, which may also coexist with other diseases. We present two patients with an unusual coincidence of IgAN and Fabry disease (FD. Case presentation A 26 year-old man underwent a renal biopsy in February 2001. Histopathology showed very advanced IgAN and vascular changes as a result of hypertension. Because of his progressive renal insufficiency the patient began hemodialysis in August 2001. By means of the blood spot test screening method the diagnosis of FD was suspected. Low activity of alpha-galactosidase A in the patient’s plasma and leukocytes and DNA analysis confirmed the diagnosis of FD. Enzyme replacement therapy started in July 2004. Then the patient underwent kidney transplantation in November 2005. Currently, his actual serum creatinine level is 250 μmol/l. Other organ damages included hypertrophic cardiomyopathy, neuropathic pain and febrile crisis. After enzyme replacement therapy, myocardial hypertrophy has stabilized and other symptoms have disappeared. No further progression of the disease has been noted. The other patient, a 30 year-old woman, suffered from long-term hematuria with a good renal function. Recently, proteinuria (2.6 g/day appeared and a renal biopsy was performed. Histopathology showed IgAN with remarkably enlarged podocytes. A combination of IgAN and a high suspicion of FD was diagnosed. Electron microscopy revealed dense deposits in paramesangial areas typical for IgAN and podocytes with inclusive zebra bodies and myelin figures characteristic of FD. FD was confirmed by the decreased alpha-galactosidase A activity in plasma and leukocytes and by DNA and RNA analysis. Enzyme replacement therapy and family screening were initiated. Conclusions Our results emphasize the role of complexity in the process of diagnostic evaluation of kidney biopsy samples. Electron microscopy represents an integral part of histopathology, and genetic

  14. Lack of serologic evidence to link IgA nephropathy with celiac disease or immune reactivity to gluten.

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    Sina Moeller

    Full Text Available IgA nephropathy is the most common form of primary glomerulonephritis worldwide. Mucosal infections and food antigens, including wheat gluten, have been proposed as potential contributing environmental factors. Increased immune reactivity to gluten and/or association with celiac disease, an autoimmune disorder triggered by ingestion of gluten, have been reported in IgA nephropathy. However, studies are inconsistent about this association. We aimed to evaluate the proposed link between IgA nephropathy and celiac disease or immune reactivity to gluten by conducting a comprehensive analysis of associated serologic markers in cohorts of well-characterized patients and controls. Study participants included patients with biopsy-proven IgA nephropathy (n = 99, unaffected controls of similar age, gender, and race (n = 96, and patients with biopsy-proven celiac disease (n = 30. All serum specimens were tested for IgG and IgA antibodies to native gliadin and deamidated gliadin, as well as IgA antibody to transglutaminase 2 (TG2. Anti-TG2 antibody-positive nephropathy patients and unaffected controls were subsequently tested for IgA anti-endomysial antibody and genotyped for celiac disease-associated HLA-DQ2 and -DQ8 alleles. In comparison to unaffected controls, there was not a statistically significant increase in IgA or IgG antibody reactivity to gliadin in individuals with IgA nephropathy. In addition, the levels of celiac disease-specific serologic markers, i.e., antibodies to deamidated gliadin and TG2, did not differ between IgA nephropathy patients and unaffected controls. Results of the additional anti-endomysial antibody testing and HLA genotyping were corroborative. The data from this case-control study do not reveal any evidence to suggest a significant role for celiac disease or immune reactivity to gluten in IgA nephropathy.

  15. Uncoupling of glomerular IgA deposition and disease progression in alymphoplasia mice with IgA nephropathy.

    Directory of Open Access Journals (Sweden)

    Masashi Aizawa

    Full Text Available Previous clinical and experimental studies have indicated that cells responsible for IgA nephropathy (IgAN, at least in part, are localized in bone marrow (BM. Indeed, we have demonstrated that murine IgAN can be experimentally reconstituted by bone marrow transplantation (BMT from IgAN prone mice in not only normal mice, but also in alymphoplasia mice (aly/aly independent of IgA+ cells homing to mucosa or secondary lymphoid tissues. The objective of the present study was to further assess whether secondary lymph nodes (LN contribute to the progression of this disease. BM cells from the several lines of IgAN prone mice were transplanted into aly/aly and wild-type mice (B6. Although the transplanted aly/aly showed the same degree of mesangial IgA and IgG deposition and the same serum elevation levels of IgA and IgA-IgG immune-complexes (IC as B6, even in extent, the progression of glomerular injury was observed only in B6. This uncoupling in aly/aly was associated with a lack of CD4+ T cells and macrophage infiltration, although phlogogenic capacity to nephritogenic IC of renal resident cells was identical between both recipients. It is suggested that secondary LN may be required for the full progression of IgAN after nephritogenic IgA and IgA/IgG IC deposition.

  16. Corticosteroids in IgA Nephropathy: A Retrospective Analysis from the VALIGA Study

    NARCIS (Netherlands)

    Tesar, V.; Troyanov, S.; Bellur, S.; Verhave, J.C.; Cook, H.T.; Feehally, J.; Roberts, I.S.; Cattran, D.; Coppo, R.

    2015-01-01

    Current guidelines suggest treatment with corticosteroids (CS) in IgA nephropathy (IgAN) when proteinuria is persistently >/=1 g/d despite 3-6 months of supportive care and when eGFR is >50 ml/min per 1.73 m(2). Whether the benefits of this treatment extend to patients with an eGFR

  17. Combined C4d and CD3 immunostaining predicts immunoglobulin (Ig)A nephropathy progression

    NARCIS (Netherlands)

    Faria, B.; Henriques, C.; Matos, A. C.; Daha, M. R.; Pestana, M.; Seelen, M.

    2015-01-01

    A number of molecules have been shown recently to be involved in the pathogenesis and progression of immunoglobulin (Ig)A nephropathy (IgAN). Among these, we have selected C4d (complement lectin pathway involvement), CD3 (T cell marker, traducing interstitial inflammation), transglutaminase 2 (TGase

  18. Prevalence and risk factors of hyperuricemia in patients with IgA nephropathy

    Institute of Scientific and Technical Information of China (English)

    梁孟君

    2013-01-01

    Objective To evaluate the prevalence of hyperuricemia in patients with IgA nephropathy and find out the risk factors of hyperuricemia,including clinical and pathological characteristics.Methods A retrospective study enrolled 2566 adult patients,who admitted to the

  19. Cellular Signaling and Production of Galactose-Deficient IgA1 in IgA Nephropathy, an Autoimmune Disease

    Directory of Open Access Journals (Sweden)

    Colin Reily

    2014-01-01

    Full Text Available Immunoglobulin A (IgA nephropathy (IgAN, the leading cause of primary glomerulonephritis, is characterized by IgA1-containing immunodeposits in the glomeruli. IgAN is a chronic disease, with up to 40% of patients progressing to end-stage renal disease, with no disease-specific treatment. Multiple studies of the origin of the glomerular immunodeposits have linked elevated circulating levels of aberrantly glycosylated IgA1 (galactose-deficient in some O-glycans; Gd-IgA1 with formation of nephritogenic Gd-IgA1-containing immune complexes. Gd-IgA1 is recognized as an autoantigen in susceptible individuals by anti-glycan autoantibodies, resulting in immune complexes that may ultimately deposit in the kidney and induce glomerular injury. Genetic studies have revealed that an elevated level of Gd-IgA1 in the circulation of IgAN patients is a hereditable trait. Moreover, recent genome-wide association studies have identified several immunity-related loci that associated with IgAN. Production of Gd-IgA1 by IgA1-secreting cells of IgAN patients has been attributed to abnormal expression and activity of several key glycosyltransferases. Substantial evidence is emerging that abnormal signaling in IgA1-producing cells is related to the production of Gd-IgA1. As Gd-IgA1 is the key autoantigen in IgAN, understanding the genetic, biochemical, and environmental aspects of the abnormal signaling in IgA1-producing cells will provide insight into possible targets for future disease-specific therapy.

  20. Gluten exacerbates IgA nephropathy in humanized mice through gliadin-CD89 interaction.

    Science.gov (United States)

    Papista, Christina; Lechner, Sebastian; Ben Mkaddem, Sanae; LeStang, Marie-Bénédicte; Abbad, Lilia; Bex-Coudrat, Julie; Pillebout, Evangéline; Chemouny, Jonathan M; Jablonski, Mathieu; Flamant, Martin; Daugas, Eric; Vrtovsnik, François; Yiangou, Minas; Berthelot, Laureline; Monteiro, Renato C

    2015-08-01

    IgA1 complexes containing deglycosylated IgA1, IgG autoantibodies, and a soluble form of the IgA receptor (sCD89), are hallmarks of IgA nephropathy (IgAN). Food antigens, notably gluten, are associated with increased mucosal response and IgAN onset, but their implication in the pathology remains unknown. Here, an IgAN mouse model expressing human IgA1 and CD89 was used to examine the role of gluten in IgAN. Mice were given a gluten-free diet for three generations to produce gluten sensitivity, and then challenged for 30 days with a gluten diet. A gluten-free diet resulted in a decrease of mesangial IgA1 deposits, transferrin 1 receptor, and transglutaminase 2 expression, as well as hematuria. Mice on a gluten-free diet lacked IgA1-sCD89 complexes in serum and kidney eluates. Disease severity depended on gluten and CD89, as shown by reappearance of IgAN features in mice on a gluten diet and by direct binding of the gluten-subcomponent gliadin to sCD89. A gluten diet exacerbated intestinal IgA1 secretion, inflammation, and villous atrophy, and increased serum IgA1 anti-gliadin antibodies, which correlated with proteinuria in mice and patients. Moreover, early treatment of humanized mice with a gluten-free diet prevented mesangial IgA1 deposits and hematuria. Thus, gliadin-CD89 interaction may aggravate IgAN development through induction of IgA1-sCD89 complex formation and a mucosal immune response. Hence, early-stage treatment with a gluten-free diet could be beneficial to prevent disease.

  1. Identification of distinct glycoforms of IgA1 in plasma from patients with IgA nephropathy and healthy individuals

    DEFF Research Database (Denmark)

    Lehoux, Sylvain; Mi, Rongjuan; Aryal, Rajindra P;

    2014-01-01

    Immunoglobulin A nephropathy (IgAN) is the most common form of glomerulonephritis worldwide and is histologically characterized by the deposition of IgA1 and consequent inflammation in the glomerular mesangium. Prior studies suggested that serum IgA1 from IgAN patients contains aberrant, undergal......AN and suggest that in vivo inefficiency of T-synthase toward IgA1 in a subpopulation of B or plasma cells, as well as overall elevation of IgA, may contribute to IgAN pathogenesis....... there are different glycoforms of IgA1 in plasma from patients with IgAN and healthy individuals. While total plasma IgA in IgAN patients was elevated ~1.6-fold compared to that in healthy donors, IgA1 in all samples was unexpectedly separable into two distinct glycoforms: one with core 1 based O...

  2. Analysis of O-glycan heterogeneity in IgA1 myeloma proteins by Fourier transform ion cyclotron resonance mass spectrometry: implications for IgA nephropathy

    DEFF Research Database (Denmark)

    Renfrow, MB; Mackay, CL; Chalmers, MJ;

    2007-01-01

    IgA nephropathy (IgAN) is the most common form of primary glomerulonephritis. In IgAN, IgA1 molecules with incompletely galactosylated O-linked glycans in the hinge region (HR) are present in mesangial immunodeposits and in circulating immune complexes. It is not known whether the galactose...... deficiency in IgA1 proteins occurs randomly or preferentially at specific sites. We have previously demonstrated the first direct localization of multiple O-glycosylation sites on a single IgA1 myeloma protein by use of activated ion-electron capture dissociation (AI-ECD) Fourier transform ion cyclotron...

  3. Aberrant O-glycosylation and anti-glycan antibodies in an autoimmune disease IgA nephropathy and breast adenocarcinoma

    DEFF Research Database (Denmark)

    Stuchlová Horynová, Milada; Raška, Milan; Clausen, Henrik;

    2013-01-01

    Glycosylation abnormalities have been observed in autoimmune diseases and cancer. Here, we compare mechanisms of aberrant O-glycosylation, i.e., formation of Tn and sialyl-Tn structures, on MUC1 in breast cancer, and on IgA1 in an autoimmune disease, IgA nephropathy. The pathways of aberrant O...

  4. Potential association of hyperhomocysteinemia with the progression of IgA nephropathy: a retrospective study

    Institute of Scientific and Technical Information of China (English)

    Duan Shuwei; Liu Shuwen; Sun Xuefeng; Zheng Ying; Liu Linchang; Yao Feixiang; Wu Jie

    2014-01-01

    Background The high blood homocysteine (Hcy) levels found in patients with hyperhomocysteinemia (HHcy) have been implicated in an increased risk of cardiovascular disease morbidity and mortality in end-stage renal disease (ESRD).This study investigated the association of HHcy with progression of IgA nephropathy.Methods We analyzed 108 participants newly diagnosed with IgA nephropathy between August 2005 and August 2007 in the Department of Nephrology,Chinese People's Liberation Army General Hospital.The association between clinicopathological factors and the Hcy levels were analyzed by Logistic regression and those with ESRD risk were analyzed by Cox regression.Results Patients were aged (35.71±10.73) years and included 45.71% women and 12.04% patients with HHcy.In multivariate Logistic regression analysis,HHcy was associated with arterial lesions (OR 2.60; 95% CI 1.55-4.34; P<0.001) even when age,body mass index,estimated glomerular filtration rate,mean arterial pressure,and initial proteinuria were taken into account.Mean follow-up was (67.37±16.21) months.HHcy was also associated with worse ESRD-free survival (HR 4.71; 95% CI 1.45 to 15.31; P=0.010).Conclusion HHcy is associated with the risk of intrarenal arterial lesions and may be useful for estimating the prognosis of IgA nephropathy.

  5. A Clinicopathologic Study of Thrombotic Microangiopathy in IgA Nephropathy

    Science.gov (United States)

    Hill, Gary S.; Karras, Alexandre; Jacquot, Christian; Moulonguet, Luc; Kourilsky, Olivier; Frémeaux-Bacchi, Véronique; Delahousse, Michel; Van Huyen, Jean-Paul Duong; Loupy, Alexandre; Bruneval, Patrick; Nochy, Dominique

    2012-01-01

    Thrombotic microangiopathy (TMA) occurs in IgA nephropathy, but its clinical significance is not well described. We retrospectively examined a series of 128 patients diagnosed with IgA nephropathy between 2002 and 2008 who had a mean follow-up of 44±27 months. In our series, 53% presented with lesions of TMA, acute or organized, in arteries and/or arterioles. Among patients with TMA, 4% were normotensive, 25% had controlled hypertension, and 71% had uncontrolled hypertension. Of those with uncontrolled hypertension, 26% had malignant hypertension. Histologically, the group with TMA had a significantly greater percentage of sclerotic glomeruli and worse tubulointerstitial fibrosis than those of the group without TMA. However, a significant minority of patients had near-normal histology, with minimal tubular atrophy (20%) and/or <20% interstitial fibrosis (24%). TMA rarely occurred in the absence of significant proteinuria. During follow-up, a doubling of serum creatinine or ESRD occurred in all patients with laboratory evidence of TMA, in 42% of those with morphologic evidence but no laboratory evidence of TMA, and in 11% of those without TMA. In summary, lesions of TMA are frequent in IgA nephropathy and may occur in normotensive patients with near-normal renal histology. Although the pathophysiologic mechanisms involved remain undetermined, the current study rules out severe hypertension or advanced renal disease as sole causes. PMID:22052055

  6. Early pre-eclampsia unmasks underlying IgA nephropathy

    OpenAIRE

    Mona Singh; Akhenaton Pappoe; Don, Burl R.

    2010-01-01

    Mona Singh, Akhenaton Pappoe, Burl R DonDivision of Nephrology, University of California Davis Medical Center, Sacramento, CA, USAAbstract: Pre-eclampsia is the most ominous complication of pregnancy, and primary glomerular diseases can mimic pre-eclampsia in presentation. A patient presented at 21 weeks gestation with signs and symptoms of both pre-eclampsia and primary glomerular nephropathy. A critical clinical decision whether to continue or terminate the pregnancy was dependent on result...

  7. [Extracapillary IgA nephropathy associated with infection with hepatitis C virus and hepatic cirrhosis].

    Science.gov (United States)

    Cabezuelo, J B; Enríquez, R; Andrada, E; Amorós, F; Sirvent, A E; Reyes, A

    2000-01-01

    We describe a 36 year old man who was admitted to the hospital with dyspnea, edema of the lower limbs, arterial hypertension and oliguric renal failure. He had microhematuria and nephrotic range proteinuria, immunological tests were normal or negative. Renal biopsy revealed global (55%) or segmental glomeruloesclerosis, remaining glomeruli showed extracapillary proliferation (25%). Immunofluorescence study disclosed IgA mesangial deposits. He was also diagnosed as having liver cirrhosis with positive serology against hepatitis C virus. He was treated with dialysis, antihypertensive drugs and steroids with improvement of the renal function. However, ten months later maintenance hemodialysis became necessary. We emphasize two points: first IgA glomerulonephritis is rarely associated with hepatitis C infection, and second crescentic IgA nephropathy has been infrequently reported in liver cirrhosis.

  8. A simultaneous liver-kidney transplant recipient with IgA nephropathy limited to native kidneys and BK virus nephropathy limited to the transplant kidney.

    Science.gov (United States)

    Ujire, Manasa P; Curry, Michael P; Stillman, Isaac E; Hanto, Douglas W; Mandelbrot, Didier A

    2013-08-01

    Immunoglobulin A (IgA) deposition in the native kidneys of patients with liver disease is well described. Secondary IgA nephropathy usually is thought to be benign, but hematuria, proteinuria, and loss of kidney function have been reported in this context. BK virus nephropathy is an important cause of kidney transplant loss; however, BK virus nephropathy is rare in the native kidneys of patients who underwent transplantation of other organs. We report the case of a patient with alcohol-related end-stage liver disease and chronic kidney disease with hematuria who underwent simultaneous liver-kidney transplantation. His kidney function decreased over the course of several weeks posttransplantation. Biopsy of the transplant kidney showed BK virus nephropathy, but no IgA deposits. In contrast, biopsy of the native kidneys showed IgA deposits, but no BK virus nephropathy. To our knowledge, this is the first reported case of a simultaneous liver-kidney transplantation wherein both the native and transplant kidneys were biopsied posttransplantation and showed exclusively different pathologies. These findings confirm the predilection of BK virus nephropathy for transplant rather than native kidneys.

  9. Serum Galactose-Deficient IgA1 Level Is Not Associated with Proteinuria in Children with IgA Nephropathy

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    M. Colleen Hastings

    2012-01-01

    Full Text Available Introduction. Percentage of galactose-deficient IgA1 (Gd-IgA1 relative to total IgA in serum was recently reported to correlate with proteinuria at time of sampling and during follow-up for pediatric and adult patients with IgA nephropathy. We sought to determine whether this association exists in another cohort of pediatric patients with IgA nephropathy. Methods. Subjects were younger than 18 years at entry. Blood samples were collected on one or more occasions for determination of serum total IgA and Gd-IgA1. Gd-IgA1 was expressed as serum level and percent of total IgA. Urinary protein/creatinine ratio was calculated for random specimens. Spearman’s correlation coefficients assessed the relationship between study variables. Results. The cohort had 29 Caucasians and 11 African-Americans with a male : female ratio of 1.9 : 1. Mean age at diagnosis was 11.7 ± 3.7 years. No statistically significant correlation was identified between serum total IgA, Gd-IgA1, or percent Gd-IgA1 versus urinary protein/creatinine ratio determined contemporaneously with biopsy or between average serum Gd-IgA1 or average percent Gd-IgA1 and time-average urinary protein/creatinine ratio. Conclusion. The magnitude of proteinuria in this cohort of pediatric patients with IgA nephropathy was influenced by factors other than Gd-IgA1 level, consistent with the proposed multi-hit pathogenetic pathways for this renal disease.

  10. The efficacy and influencing factors of combination of prednisone and MTX in patients with IgA nephropathy

    Institute of Scientific and Technical Information of China (English)

    Xuan Cao; Jian Chen; Ya-Lin Hu

    2016-01-01

    Objective:To evaluate the efficacy and factors of prednisone combined with cyclophosphamide (CTX) treatment of primary immunoglobulin A (IgA).Methods:Ninty six cases of IgA nephropathy patients were treated with prednisone combined CTX from January 2013 to December 2014. Renal function before and after treatment was measured and the effects of prednisone combined CTX in patients with IgA nephropathy were analyzed.Results:Thirty eight cases were cured in 96 cases of IgA nephropathy patients (39.58%), 42 cases were effective (43.75%), 16 cases were ineffective (16.67%), totally, and 80 were was ffective (83.33%). After treatment, blood urea nitrogen (BUN), serum creatinine (blood Scr), 24 h urine protein (24 h Uab) were lower than before treatment, and endogenous creatinine clearance (Ccr) were significantly higher than before treatment (P<0.05). After logistic regression, 24 h urine protein levels, Ccr before treatment, LEE kidney tissue classification, hypertension were risk factors of prednisone combined with CTX treatment on prognosis of patients with IgA nephropathy.Conclusions:Prednisone combined CTX is effective in the treatment of IgA nephropathy, but clinical and pathological features of patients should be considered in the choice of the program.

  11. IgA nephropathy in systemic lupus erythematosus patients: case report and literature review

    Directory of Open Access Journals (Sweden)

    Leonardo Sales da Silva

    2016-06-01

    Full Text Available Abstract Systemic erythematosus lupus (SLE is a multisystemic autoimmune disease which has nephritis as one of the most striking manifestations. Although it can coexist with other autoimmune diseases, and determine the predisposition to various infectious complications, SLE is rarely described in association with non‐lupus nephropathies etiologies. We report the rare association of SLE and primary IgA nephropathy (IgAN, the most frequent primary glomerulopathy in the world population. The patient was diagnosed with SLE due to the occurrence of malar rash, alopecia, pleural effusion, proteinuria, ANA 1: 1,280, nuclear fine speckled pattern, and anticardiolipin IgM and 280 U/mL. Renal biopsy revealed mesangial hypercellularity with isolated IgA deposits, consistent with primary IgAN. It was treated with antimalarial drug, prednisone and inhibitor of angiotensin converting enzyme, showing good progress. Since they are relatively common diseases, the coexistence of SLE and IgAN may in fact be an uncommon finding for unknown reasons or an underdiagnosed condition. This report focus on the importance of the distinction between the activity of renal disease in SLE and non‐SLE nephropathy, especially IgAN, a definition that has important implications on renal prognosis and therapeutic regimens to be adopted in the short and long term.

  12. IgA nephropathy in systemic lupus erythematosus patients: case report and literature review.

    Science.gov (United States)

    da Silva, Leonardo Sales; Almeida, Bruna Laiza Fontes; de Melo, Ana Karla Guedes; de Brito, Danielle Christine Soares Egypto; Braz, Alessandra Sousa; Freire, Eutília Andrade Medeiros

    2016-01-01

    Systemic erythematosus lupus (SLE) is a multisystemic autoimmune disease which has nephritis as one of the most striking manifestations. Although it can coexist with other autoimmune diseases, and determine the predisposition to various infectious complications, SLE is rarely described in association with non-lupus nephropathies etiologies. We report the rare association of SLE and primary IgA nephropathy (IgAN), the most frequent primary glomerulopathy in the world population. The patient was diagnosed with SLE due to the occurrence of malar rash, alopecia, pleural effusion, proteinuria, ANA 1: 1280, nuclear fine speckled pattern, and anticardiolipin IgM and 280U/mL. Renal biopsy revealed mesangial hypercellularity with isolated IgA deposits, consistent with primary IgAN. It was treated with antimalarial drug, prednisone and inhibitor of angiotensin converting enzyme, showing good progress. Since they are relatively common diseases, the coexistence of SLE and IgAN may in fact be an uncommon finding for unknown reasons or an underdiagnosed condition. This report focus on the importance of the distinction between the activity of renal disease in SLE and non-SLE nephropathy, especially IgAN, a definition that has important implications on renal prognosis and therapeutic regimens to be adopted in both the short and long terms.

  13. IgA Nephropathy and Henoch-Schoenlein Purpura Nephritis: Aberrant Glycosylation of IgA1, Formation of IgA1-Containing Immune Complexes, and Activation of Mesangial Cells

    DEFF Research Database (Denmark)

    Novak, J.; Moldoveanu, Z.; Renfrow, M.B.;

    2007-01-01

    IgA1 in the circulation and glomerular deposits of patients with IgA nephropathy (IgAN) is aberrantly glycosylated; the hinge-region O-linked glycans are galactose-deficient. The circulating IgA1 of patients with Henoch-Schoenlein purpura nephritis (HSPN) has a similar defect. This aberrancy...... at specific sites. We sought to define the aberrant glycosylation of a galactose-deficient IgA1 myeloma protein and analyze the formation of the immune complexes and their biological activities. Supplementation of serum or cord-blood serum with this IgA1 protein resulted in formation of new IgA1 complexes...... determined the O-glycosylation sites in the hinge region of the IgA1 myeloma protein and IgA1 proteins from sera of IgAN patients. The IgA1 myeloma protein had galactose-deficient sites at residues 228 and/or 230 and 232. These sites reacted with IgG specific to galactose-deficient IgA1. IgA1 from the Ig...

  14. IgA nephropathy and tonsils--an approach from the structure of IgA1 produced by tonsillar lymphocytes.

    Science.gov (United States)

    Hiki, Yoshiyuki; Horie, Akeyo; Yasuda, Yoshinari; Iwase, Hitoo; Sugiyama, Satoshi

    2004-12-01

    Human immunoglobulin A1 (IgA1), which is the predominant subtype to be deposited in glomeruli in IgA nephropathy (IgAN), has a unique mucine-like structure in its hinge region. Namely, it contains O-glycans and proline-rich peptides We previously observed underglycosylation of the hinge region in serum and deposited IgA1 in IgAN. On the other hand, clinical development and exacerbation of IgAN are frequently preceded by episodes of upper respiratory tract infection, and palatine tonsils represent the predominant immunocompetent tissue of the upper respiratory tract. Therefore, we hypothesized that tonsils were one of the origins of glomerular IgA1 in IgAN, and investigated the O-glycan structure of IgA1 produced by tonsillar lymphocytes (tonsillar IgA1). A significant increase in asialo-agalacto type O-glycans was found in the tonsillar IgA1 hinge in IgAN. These results suggest that the tonsils produce underglycosylated IgA1 molecules, which enter the bloodstream and are then deposited in the glomeruli.

  15. Retrospective study of mycophenolate mofetil treatment in IgA nephropathy with proliferative pathological phenotype

    Institute of Scientific and Technical Information of China (English)

    Liang Yan; Zhang Junjun; Liu Dongwei; Quan Songxia; Xing Guolan; Liu Zhangsuo

    2014-01-01

    Background Mycophenolate mofetil (MMF) and cyclophosphamide (CTX) are widely used in treating various kidney diseases.However,whether they are effective and which one is better for treating IgA nephropathy patients with proliferative pathological phenotype in renal diseases,such as endocapillary proliferation,cellular crescents,and/or capillary loops fibrinoid necrosis is still unknown.We,therefore,initiated a study to compare the effects of MMF and CTX in treating IgA nephropathy with the above pathological lesions.Methods One hundred and nineteen patients with IgA nephropathy who had at least one of the three aforementioned lesions were enrolled.All patients were treated with prednisone; 48 patients received prednisone only (Pred group),40 received MMF and prednisone (MMF + Pred group),and 31 were treated with CTX and prednisone (CTX + Pred group).The median time of follow-up was 30 months (maximum:96 months).The primary endpoint was defined as renal survival.The incidence of remission of proteinuria was the secondary endpoint.Results Serum creatinine in all groups declined significantly at different follow-up times (P=0.002),and the differences among the three groups were significant (P<0.001).At 24 months of follow-up,the decline rates were 12.35%,32.95%,and 24.14% in the Pred,MMF + Pred,and CTX + Pred groups respectively.For urine protein excretion,the decline rates were 49.12% (Pred),73.67% (MMF + Pred),and 63.53% (CTX + Pred) respectively at 24 months of follow-up.The differences among the three groups were not significant (P=0.714).Renal survival (the primary endpoint) was significantly different (P=0.027); however,the sencondary endpoint was similar for all the three groups (P=0.100).Conclusions For IgA nephropathy patients with endocapillary proliferation,cellular crescents,and/or fibrinoid necrosis of capillary loops,prednisone combined with MMF was more effective in lowering the serum creatinine than with CTX.Combined MMF and orednisone

  16. The incidence of biopsy-proven IgA nephropathy is associated with multiple socioeconomic deprivation.

    Science.gov (United States)

    McQuarrie, Emily P; Mackinnon, Bruce; McNeice, Valerie; Fox, Jonathan G; Geddes, Colin C

    2014-01-01

    Chronic kidney disease is more common in areas of socioeconomic deprivation, but the relationship with the incidence and diagnosis of biopsy-proven renal disease is unknown. In order to study this, all consecutive adult patients undergoing renal biopsy in West and Central Scotland over an 11-year period were prospectively analyzed for demographics, indication, and histologic diagnosis. Using the Scottish Index of Multiple Deprivation, 1555 eligible patients were separated into quintiles of socioeconomic deprivation according to postcode. Patients in the most deprived quintile were significantly more likely to undergo biopsy compared with patients from less deprived areas (109.5 compared to 95.9 per million population/year). Biopsy indications were significantly more likely to be nephrotic syndrome, or significant proteinuria without renal impairment. Patients in the most deprived quintile were significantly more likely to have glomerulonephritis. There was a significant twofold increase in the diagnosis of IgA nephropathy in the patients residing in the most compared with the least deprived postcodes not explained by the demographics of the underlying population. Thus, patients from areas of socioeconomic deprivation in West and Central Scotland are significantly more likely to undergo native renal biopsy and have a higher prevalence of IgA nephropathy.

  17. IgA nephropathy: A clinicopathologic study from two centers in Saudi Arabia

    Directory of Open Access Journals (Sweden)

    Khawajah Azhar

    2010-01-01

    Full Text Available A total of 42 patients, who were diagnosed to have primary Immunoglobulin A neph-ropathy (IgAN at the King Abdul Aziz University Hospital and King Faisal Hospital, Jeddah over the last seven years, were studied. The objective was to analyze their clinical and pathological fea-tures and to classify them according to Hass Classification by using light, immunofluorescence and electron microscopy. Majority of the study cases were males in the second, third and fourth decades of life. Hematuria was the most common clinical complaint followed by proteinuria. There were varying degrees of mesangial proliferation. Majority of the cases presented with class-2 followed by class-3. Immunofluorescence demonstrated diffuse granular deposition of IgA in the glomerular mesangium in majority of the cases. Ultrastructural analysis showed electron dense deposits within the matrix of the mesangium and paramesangium in majority of the cases. Sub-endothelial deposits and mesangial interposition were demonstrated in few cases. Extensive effacement with fusion of the visceral epithelial foot processes was detected in only few patients while focal effacement was demonstrated in many cases. Irregularities of the glomerular basement membrane were seen in some cases. We conclude that IgA nephropathy is an immune-complex glomerular disease, which occurs at all ages and with higher frequency in males and presents mostly with hematuria and proteinuria. Public health awareness is seriously needed to perform the investigations at an early stage.

  18. NLRP3 inflammasome: Pathogenic role and potential therapeutic target for IgA nephropathy

    Science.gov (United States)

    Tsai, Yu-Ling; Hua, Kuo-Feng; Chen, Ann; Wei, Chyou-Wei; Chen, Wen-Shiang; Wu, Cheng-Yeu; Chu, Ching-Liang; Yu, Yung-Luen; Lo, Chia-Wen; Ka, Shuk-Man

    2017-01-01

    We have previously showed that IL-1β is involved in the pathogenesis of both spontaneously occurring and passively induced IgA nephropathy (IgAN) models. However, the exact causal-relationship between NLRP3 inflammasome and the pathogenesis of IgAN remains unknown. In the present study, we showed that [1] IgA immune complexes (ICs) activated NLRP3 inflammasome in macrophages involving disruption of mitochondrial integrity and induction of mitochondrial ROS, bone marrow-derived dendritic cells (BMDCs) and renal intrinsic cells; [2] knockout of NLRP3 inhibited IgA ICs-mediated activation of BMDCs and T cells; and [3] knockout of NLRP3 or a kidney-targeting delivery of shRNA of NLRP3 improved renal function and renal injury in a mouse IgAN model. These results strongly suggest that NLRP3 inflammasome serves as a key player in the pathogenesis of IgAN partly through activation of T cells and mitochondrial ROS production and that a local, kidney-targeting suppression of NLRP3 be a therapeutic strategy for IgAN. PMID:28117341

  19. Functional networks of aging markers in the glomeruli of IgA nephropathy: a new therapeutic opportunity

    Science.gov (United States)

    Jiang, Hong; Liang, Ludan; Qin, Jing; Lu, Yingying; Li, Bingjue; Wang, Yucheng; Lin, Chuan; Zhou, Qin; Feng, Shi; Yip, Shun H.; Xu, Feng; Lai, EnYin; Wang, Junwen; Chen, Jianghua

    2016-01-01

    IgA nephropathy(IgAN) is the most common primary glomerular disease in China. Primary infections always occur before IgAN. However, the pathology of IgAN is still unclear. Previously we found that LL37, a protein secreted by senescent cells, was specific for the progression of IgAN, and also played a role in the neutrophil function. So we hypothesized that the infiltration of neutrophils, inflammation factors, and aging markers, which were modulated by functional networks, induced the immune response and renal injury. RNA-Sequencing (RNA-seq) can be used to study the whole transcriptome and detect splicing variants that are expressed in a specific cell type or tissue. We separate glomerulus from the renal biopsy tissues. After RNA extraction, the sequences were analyzed with Illumina HiSeq 2000/2500. 381 genes with differential expression between the IgAN patients and the healthy controls were identified. Only PLAU, JUN, and FOS were related to DNA damage, telomere dysfunction-induced aging markers, neutrophil function and IgA nephropathy. The networks showed the possibility of these genes being connected. We conclude that DNA damage and telomere dysfunction could play important roles in IgA nephropathy. In addition, neutrophils are also important factors in this disease. The networks of these markers showed the mechanism pathways that are involved in the duration of the occurrence and progression of IgA nephropathy and might be a new therapeutic opportunity for disease treatment. PMID:27127888

  20. IgA 肾病患者 IgA1糖基化异常及其致病机制%The pathopoiesis mechanism of abnormal IgA1 glycosylation in IgA nephropathy patients

    Institute of Scientific and Technical Information of China (English)

    林淑芃

    2015-01-01

    IgA 肾病(IgAN)是导致终末期肾病最常见的原发性肾小球疾病。其病理特点为 IgA1在肾小球系膜区沉积,IgA1分子的异常糖基化是导致 IgAN 发病的关键因素。多种与 IgAN 相关的基因位点已经被发现。这些基因编码的细胞因子参与了 IgA1糖基化异常的发病机制。此外糖基化酶缺乏、分子伴侣甲基化异常都可能导致 IgA1异常糖基化。异常糖基化的 IgA1可通过自我聚集或形成免疫复合物沉积于系膜区,进而刺激系膜细胞增殖、分泌系膜基质、细胞因子、趋化因子、生长因子等,导致肾小球损伤。对 IgA1异常糖基化的深入研究有助于了解 IgA 肾病的发病机制并提供新的诊断与治疗措施。%IgA nephropathy (IgAN)is the most common primary glomerular disease that can result in end-stage renal disease,and is histologically characterized by the deposition of IgA1 in the glomerular mesangium.The abnormal IgA1 glycosylation is the key factor in the pathogenesis of IgAN.Multiple genetic loci associated with IgAN have been identified,and the cytokines coded by them are involved in the pathopoiesis mechanism of abnormal IgA1 glycosylation.In addition,the lack of glycosylase and abnormal methylation of molecular chaperone may also be involved in the aberrant glycosylation of IgA1.Abnormally glycosylated IgA1 can deposit in the mesangium through their own assembly together or formation of immunocomplex,which can subsequently stimulate mesangial cell proliferation and secretion of extracellular matrix,cytokines,chemokines,and growth factors,etc,leading to glomerular injury.In-depth research on IgA1 abnormal glycosylation will help to understand the pathogenesis of IgAN and provide new diagnosis and treatment methods.

  1. Childhood linear IgA bullous dermatosis in Tunisia.

    Science.gov (United States)

    Kenani, Nesrine; Mebazaa, Amel; Denguezli, Mohamed; Ghariani, Najet; Sriha, Badreddine; Belajouza, Colandane; Nouira, Rafia

    2009-01-01

    The objective was to determine the demographic characteristics, the clinical features, the immuno-histological findings and response to treatment of childhood linear IgA bullous dermatosis (LABD) in Tunisia. We collected all the cases of auto-immune bullous diseases of childhood, diagnosed from January 1987 to December 2006. Based on clinical, histological, and immunofluorescent features, we identified 25 cases of LABD. Sixteen male and nine female children with a mean age of 7.5 years were identified. Clinical manifestations were characterized by a vesiculo-bullous eruption in all cases associated with mucous membrane involvement in two cases. Dapsone was the main therapy in 19 cases, associated with systemic corticosteroids in eight cases. Exclusive antibiotic therapy was successful in five cases. Sixteen of those patients had resolution of disease after a mean period of 15 months and eight patients had severe clinical presentation and required a prolonged follow-up. Childhood LABD is the most frequent bullous dermatosis in Tunisia. The majority of our patients responded rapidly to dapsone treatment and were stabilized for long time. Our cases were characterized by a minimal mucosal involvement and favorable outcome. Treatment with antibiotherapy was interesting. Erythromycin and oxacillin may be considered as an alternative therapy.

  2. Controlled prospective trial of prednisolone and cytotoxics in progressive IgA nephropathy.

    Science.gov (United States)

    Ballardie, Francis W; Roberts, Ian S D

    2002-01-01

    In a single-center, multiple-referral source study, 38 patients with progressive IgA nephropathy and controlled hypertension were randomized to treatment with prednisolone and cytotoxic agents, to therapy with low-dose cyclophosphamide then azathioprine, and to control groups. The follow-up period lasted 2 to 6 yr. Renal survival, as assessed by Kaplan-Meier analysis annually to 5 yr, showed significant preservation of function from 3 yr in the treatment group and 82, 82, 72, and 72% for 2, 3, 4, and 5 yr, respectively, compared with 68, 47, 26, and 6% in controls. Rate of loss of renal function, evaluated objectively by least-squares analyses of reciprocal serum creatinine, was reduced-and in one-third of the patients, arrested-during immunosuppressive treatment. Proteinuria, present in all patients at the time of entry into the trial, was reduced by treatment from 12 mo, compared with pretreatment levels or controls; erythrocyturia was reduced from 6 mo. Histologic activity and chronicity indexes were determined in renal biopsies performed at trial entry. Multivariate analysis demonstrated that mesangial cell proliferation and matrix scores were highest in those patients with more rapidly progressive disease. No morphologic variable or residual renal function predicted response to immunosuppressive therapy at entry. Mean arterial pressures did not differ significantly between treatment and control groups. There was thus no explanation other than treatment for the improved outcome in patients who received immunosuppressive therapy. Morbidity attributable to treatment or to renal failure occurred in both groups; an audit showed that benefits of therapy outweighed expected or minor side effects of drugs in this population at risk of end-stage renal failure. Patients selected for moderately progressive IgA nephropathy benefit from treatment with prednisolone and cytotoxic agents; results are consistent with modulation of systemic immune response or nephritic injury

  3. Vibration Induces BAFF Overexpression and Aberrant O-Glycosylation of IgA1 in Cultured Human Tonsillar Mononuclear Cells in IgA Nephropathy

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    Muyao Ye

    2016-01-01

    Full Text Available Objective. To investigate the influence of in vitro vibratory stimulation of human tonsillar mononuclear cells (TMCs. Methods. Fourteen IgA nephropathy (IgAN patients with chronic tonsillitis (CT and 12 CT patients with no renal pathology were enrolled. Group A TMCs were collected after 24 hours of culture and used to determine baseline levels. TMCs in groups B, C, D, E, and F were exposed to vibratory stimulation (60 Hz for 0 (as the control group, 1, 3, 5, and 10 minutes, respectively. Results. Baseline concentrations of B-cell-activation factor (BAFF and IgA1, BAFF mRNA expression, and aberrant O-glycosylation IgA1 level were higher in the IgAN group as compared to that in the CT group, and all increased after vibratory stimulation. Baseline mRNA expressions of core β1,3-galactosyltransferase (C1GALT1 and core β1,3GalT-specific molecular chaperone (Cosmc were lower in the IgAN group; the levels decreased further after vibratory stimulation. Conclusion. In patients with IgAN, vibratory stimulation of TMCs appears to induce IgA1 secretion through activation of BAFF release and to aberrant O-glycosylation IgA1 by suppressing C1GALT1 and Cosmc expression. In vitro vibratory stimulation of human TMCs mimics the vibratory simulation of palatine tonsils produced by vocal cords during phonation.

  4. Determination of optimal blood pressure for patients with IgA nephropathy based on renal histology.

    Science.gov (United States)

    Osawa, Y; Narita, I; Imai, N; Iino, N; Iguchi, S; Ueno, M; Shimada, H; Nishi, S; Arakawa, M; Gejyo, F

    2001-03-01

    To evaluate the optimal BP control for patients with IgA nephropathy (IgAN) based on the histologic severity of the nephropathy and the degree of renal dysfunction. We analyzed 332 consecutive renal biopsy specimens and clinical data from patients with IgAN. Patients were divided into three groups based on their BP at the time of biopsy: an optimal BP (SBP or = 140 mmHg and/or DBP > or = 90 mmHg), and an intermediate BP group. Each biopsy specimen was evaluated for mesangial proliferation, degree of sclerosis and/or hyalinosis of the arterioles and the interlobular artery using a semiquantitative method. Creatinine clearance and the percentage of sclerosed glomeruli were also determined. Both the degree of renal dysfunction and the histologic changes correlated significantly with BP, even in patients with a BP <140/90 mmHg. The patients with an optimal BP at the time of biopsy had significantly less histologic damage with respect to mesangial proliferation and vessel changes than those with an intermediate or hypertensive BP. In the patients with a hypertensive BP, the percentage of sclerotic glomeruli was significantly higher and the creatinine clearance was significantly lower. The optimal BP proposed by the WHO in 1999 prevents histologic evidence of renal damage for patients with IgAN.

  5. A panel of serum biomarkers differentiates IgA nephropathy from other renal diseases.

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    Hiroyuki Yanagawa

    Full Text Available BACKGROUND AND OBJECTIVES: There is increasing evidence that galactose-deficient IgA1 (Gd-IgA1 and Gd-IgA1-containing immune complexes are important for the pathogenesis of IgA nephropathy (IgAN. In the present study, we assessed a novel noninvasive multi-biomarker approach in the diagnostic test for IgAN. MATERIALS AND METHODS: We compared serum levels of IgA, IgG, Gd-IgA1, Gd-IgA1-specific IgG and Gd-IgA1-specific IgA in 135 IgAN patients, 79 patients with non-IgAN chronic kidney disease (CKD controls and 106 healthy controls. Serum was collected at the time of kidney biopsy from all IgAN and CKD patients. RESULTS: Each serum marker was significantly elevated in IgAN patients compared to CKD (P<0.001 and healthy controls (P<0.001. While 41% of IgAN patients had elevated serum Gd-IgA1 levels, 91% of these patients exhibited Gd-IgA1-specific IgG levels above the 90th percentile for healthy controls (sensitivity 89%, specificity 92%. Although up to 25% of CKD controls, particularly those with immune-mediated glomerular diseases including lupus nephritis, also had elevated serum levels of Gd-IgA1-specific IgG, most IgAN patients had elevated levels of Gd-IgA1-specific antibody of both isotypes. Serum levels of Gd-IgA1-specific IgG were associated with renal histological grading. Furthermore, there was a trend toward higher serum levels of Gd-IgA1-specific IgG in IgAN patients with at least moderate proteinuria (≥1.0 g/g, compared to patients with less proteinuria. CONCLUSIONS: Serum levels of Gd-IgA1-specific antibodies are elevated in most IgAN patients, and their assessment, together with serum levels of Gd-IgA1, improves the specificity of the assays. Our observations suggest that a panel of serum biomarkers may be helpful in differentiating IgAN from other glomerular diseases.

  6. Intermedin ameliorates IgA nephropathy by inhibition of oxidative stress and inflammation.

    Science.gov (United States)

    Wang, Yanhong; Tian, Jihua; Guo, Haixiu; Mi, Yang; Zhang, Ruijing; Li, Rongshan

    2016-05-01

    IgA nephropathy (IgAN) is the most frequent form of glomerulonephritis worldwide. The role of oxidative stress and inflammation in the pathogenesis of IgAN has been reported. Intermedin (IMD) is a newly discovered peptide that is closely related to adrenomedullin. We have recently reported that IMD can significantly reduce renal ischemia/reperfusion injury by diminishing oxidative stress and suppressing inflammation. The present study was designed to explore whether IMD ameliorates IgAN via oxidative stress- and inflammation-dependent mechanisms. Our results showed that IMD administration resulted in the prevention of albuminuria and ameliorated renal pathomorphological changes. These findings were associated with (1) decreased renal TGF-β1 and collagen IV expression, (2) an increased SOD level and reduced MDA level, (3) the inhibition of the renal activation of NF-κB p65 and (4) the downregulation of the expression of inflammatory factors (TNF-α, MCP-1 and MMP-9) in the kidney. These results indicate that IMD in the kidney protects against IgAN by reducing oxidative stress and suppressing inflammation.

  7. IgA Nephropathy: A Twenty Year Retrospective Single Center Experience

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    Jacob Rube

    2009-01-01

    Full Text Available IgA nephropathy (IgAN is a common glomerular disease whose etiology is unknown. Previous studies have described the clinical and laboratory features but none have specifically compared patients during different time periods. This 20 year retrospective study was performed to assess trends in the severity of IgAN from 1989–2008. We reviewed 57 patient charts that contained a confirmed biopsy diagnosis of IgAN and recorded data at the time of diagnosis and the final follow-up appointment. Clinical data included physical examination, urine, and blood tests. Patients were separated into two cohorts, Cohort 1 1989–1998 and Cohort 2 1999–2008. An increase in severity was noted in Cohort 2 based on a significantly higher Up/c and lower serum albumin level. Other prognostic indicators including GFRe, hematocrit, and glomerular injury score also demonstrated a trend towards more severe disease over the past 20 years. The patients in both Cohorts received similar treatments and had comparable renal function at the last follow-up visit. Based on our findings, we suggest that although a kidney biopsy is required to diagnose IgAN, the procedure may not be necessary in patients clinically suspected of having the disease but who have normal kidney function and minimal urine abnormalities.

  8. Comparison of a Bayesian Network with a Logistic Regression Model to Forecast IgA Nephropathy

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    Michel Ducher

    2013-01-01

    Full Text Available Models are increasingly used in clinical practice to improve the accuracy of diagnosis. The aim of our work was to compare a Bayesian network to logistic regression to forecast IgA nephropathy (IgAN from simple clinical and biological criteria. Retrospectively, we pooled the results of all biopsies (n=155 performed by nephrologists in a specialist clinical facility between 2002 and 2009. Two groups were constituted at random. The first subgroup was used to determine the parameters of the models adjusted to data by logistic regression or Bayesian network, and the second was used to compare the performances of the models using receiver operating characteristics (ROC curves. IgAN was found (on pathology in 44 patients. Areas under the ROC curves provided by both methods were highly significant but not different from each other. Based on the highest Youden indices, sensitivity reached (100% versus 67% and specificity (73% versus 95% using the Bayesian network and logistic regression, respectively. A Bayesian network is at least as efficient as logistic regression to estimate the probability of a patient suffering IgAN, using simple clinical and biological data obtained during consultation.

  9. Comparison of a Bayesian network with a logistic regression model to forecast IgA nephropathy.

    Science.gov (United States)

    Ducher, Michel; Kalbacher, Emilie; Combarnous, François; Finaz de Vilaine, Jérome; McGregor, Brigitte; Fouque, Denis; Fauvel, Jean Pierre

    2013-01-01

    Models are increasingly used in clinical practice to improve the accuracy of diagnosis. The aim of our work was to compare a Bayesian network to logistic regression to forecast IgA nephropathy (IgAN) from simple clinical and biological criteria. Retrospectively, we pooled the results of all biopsies (n = 155) performed by nephrologists in a specialist clinical facility between 2002 and 2009. Two groups were constituted at random. The first subgroup was used to determine the parameters of the models adjusted to data by logistic regression or Bayesian network, and the second was used to compare the performances of the models using receiver operating characteristics (ROC) curves. IgAN was found (on pathology) in 44 patients. Areas under the ROC curves provided by both methods were highly significant but not different from each other. Based on the highest Youden indices, sensitivity reached (100% versus 67%) and specificity (73% versus 95%) using the Bayesian network and logistic regression, respectively. A Bayesian network is at least as efficient as logistic regression to estimate the probability of a patient suffering IgAN, using simple clinical and biological data obtained during consultation.

  10. Insulin-like growth factor binding protein-1 levels are increased in patients with IgA nephropathy

    Energy Technology Data Exchange (ETDEWEB)

    Tokunaga, Koki [Department of Digestive and Life-Style Related Disease, Health Research Course, Human and Environmental Sciences, Kagoshima University Graduate School of Medical and Dental Sciences, 8-35-1 Sakuragaoka, Kagoshima 890-8520 (Japan); Uto, Hirofumi, E-mail: hirouto@m2.kufm.kagoshima-u.ac.jp [Department of Digestive and Life-Style Related Disease, Health Research Course, Human and Environmental Sciences, Kagoshima University Graduate School of Medical and Dental Sciences, 8-35-1 Sakuragaoka, Kagoshima 890-8520 (Japan); Takami, Yoichiro; Mera, Kumiko; Nishida, Chika; Yoshimine, Yozo; Fukumoto, Mayumi; Oku, Manei; Sogabe, Atsushi; Nosaki, Tsuyoshi; Moriuchi, Akihiro; Oketani, Makoto; Ido, Akio; Tsubouchi, Hirohito [Department of Digestive and Life-Style Related Disease, Health Research Course, Human and Environmental Sciences, Kagoshima University Graduate School of Medical and Dental Sciences, 8-35-1 Sakuragaoka, Kagoshima 890-8520 (Japan)

    2010-08-20

    Research highlights: {yields} IGFBP-1 mRNA over express in kidneys obtained from mice model of IgA nephropathy. {yields} Serum IGFBP-1 levels are high in patients with IgA nephropathy. {yields} Serum IGFBP-1 levels correlate with renal function and the severity of renal injury. -- Abstract: The mechanisms underlying the pathogenesis of immunoglobulin A (IgA) nephropathy (IgAN) are not well understood. In this study, we examined gene expression profiles in kidneys obtained from mice with high serum IgA levels (HIGA mice), which exhibit features of human IgAN. Female inbred HIGA, established from the ddY line, were used in these experiments. Serum IgA levels, renal IgA deposition, mesangial proliferation, and glomerulosclerosis were increased in 32-week-old HIGA mice in comparison to ddY animals. By microarray analysis, five genes were observed to be increased by more than 2.5-fold in 32-week-old HIGA in comparison to 16-week-old HIGA; these same five genes were decreased more than 2.5-fold in 32-week-old ddY in comparison to 16-week-old ddY mice. Of these five genes, insulin-like growth factor (IGF) binding protein (IGFBP)-1 exhibited differential expression between these mouse lines, as confirmed by quantitative RT-PCR. In addition, serum IGFBP-1 levels were significantly higher in patients with IgAN than in healthy controls. In patients with IgAN, these levels correlated with measures of renal function, such as estimated glomerular filtration rate (eGFR), but not with sex, age, serum IgA, C3 levels, or IGF-1 levels. Pathologically, serum IGFBP-1 levels were significantly associated with the severity of renal injury, as assessed by mesangial cell proliferation and interstitial fibrosis. These results suggest that increased IGFBP-1 levels are associated with the severity of renal pathology in patients with IgAN.

  11. UNUSUAL CLINICOPATHOLOGICAL AND IMMUNOLOGICAL PRESENTATION OF CHRONIC BULLOUS DERMATOSIS OF CHILDHOOD (LINEAR IGA DERMATOSIS)

    OpenAIRE

    Al-Saif Fahad; Al-Rikabi Ammar

    2011-01-01

    Linear IgA bullous dermatosis is a rare sulfone-responsive subepidermal blistering disorder of unknown etiology in which smooth linear deposits of IgA are found in the basement membrane zone. Chronic bullous dermatosis of childhood is equivalent to linear IgA disease of adulthood and is characterized by an abrupt onset of large, widespread and tense bullae on a normal or erythematous base. In this case, we describe an unusual presentation of chronic bullous dermatosis in a 14-month-old Saudi ...

  12. Race/ethnicity and disease severity in IgA nephropathy

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    Chertow Glenn M

    2004-09-01

    Full Text Available Abstract Background Relatively few U.S.-based studies in chronic kidney disease have focused on Asian/Pacific Islanders. Clinical reports suggest that Asian/Pacific Islanders are more likely to be affected by IgA nephropathy (IgAN, and that the severity of disease is increased in these populations. Methods To explore whether these observations are borne out in a multi-ethnic, tertiary care renal pathology practice, we examined clinical and pathologic data on 298 patients with primary glomerular lesions (IgAN, focal segmental glomerulosclerosis, membranous nephropathy and minimal change disease at the University of California San Francisco Medical Center from November 1994 through May 2001. Pathologic assessment of native kidney biopsies with IgAN was conducted using Haas' classification system. Results Among individuals with IgAN (N = 149, 89 (60% were male, 57 (38% white, 53 (36% Asian/Pacific Islander, 29 (19% Hispanic, 4 (3% African American and 6 (4% were of other or unknown ethnicity. The mean age was 37 ± 14 years and median serum creatinine 1.7 mg/dL. Sixty-six patients (44% exhibited nephrotic range proteinuria at the time of kidney biopsy. The distributions of age, gender, mean serum creatinine, and presence or absence of nephrotic proteinuria and/or hypertension at the time of kidney biopsy were not significantly different among white, Hispanic, and Asian/Pacific Islander groups. Of the 124 native kidney biopsies with IgAN, 10 (8% cases were classified into Haas subclass I, 12 (10% subclass II, 23 (18% subclass III, 30 (25% subclass IV, and 49 (40% subclass V. The distribution of Haas subclass did not differ significantly by race/ethnicity. In comparison, among the random sample of patients with non-IgAN glomerular lesions (N = 149, 77 (52% patients were male, 51 (34% white, 42 (28% Asian/Pacific Islander, 25 (17% Hispanic, and 30 (20% were African American. Conclusions With the caveats of referral and biopsy biases, the race

  13. Association of Megsin Gene Variants With IgA Nephropathy in Northwest Chinese Population

    Science.gov (United States)

    Wei, Lin-Ting; Fu, Rong-Guo; Gao, Jie; Yu, Qiao-Ling; Dong, Feng-Ming; Wang, Zhe; Wang, Meng; Liu, Xing-Han; Dai, Zhi-Jun

    2016-01-01

    Abstract Megsin is a mesangial cell-predominant gene that encodes a serpin family protein which is expressed in the renal mesangium. Overexpression of megsin has been observed in the glomeruli of patients with IgA nephropathy (IgAN). The aim of this study was to evaluate the association of megsin polymorphisms (rs1055901 and rs1055902) with IgAN in a Chinese population. We examined 351 patients with histologically proven IgAN and compared them with 310 age, sex, and ethnicity-matched healthy subjects. Two single nucleotide polymorphisms (SNPs) in megsin were genotyped by Sequenom MassARRAY. SPSS 18.0 was used for statistical analyses, and SNP Stats to test for associations between these polymorphisms and IgAN risk. Odds ratios with 95% confidence intervals were used to assess the relationships. We found that rs1055901 and rs1055902 SNPs were not correlated with susceptibility to IgAN in Northwest Chinese population. Analyses of the relationship between genotypes and clinical variables indicated that in patients with IgAN, rs1055901 was associated with 24-hour proteinuria, an increase in blood pressure, and Lee's grade (P = 0.04, 0.02, and 0.04, respectively), and rs1055902 was associated with 24-hour proteinuria and Lee's grade (P = 0.03 and 0.01, respectively). However, the results showed no association between these gene variants and sex of the patients. These results indicate that megsin gene variants may play a role in the severity, development, and/or progression of IgAN in Northwest Chinese population. PMID:26871801

  14. Identification of new susceptibility loci for IgA nephropathy in Han Chinese.

    Science.gov (United States)

    Li, Ming; Foo, Jia-Nee; Wang, Jin-Quan; Low, Hui-Qi; Tang, Xue-Qing; Toh, Kai-Yee; Yin, Pei-Ran; Khor, Chiea-Chuen; Goh, Yu-Fen; Irwan, Ishak D; Xu, Ri-Cong; Andiappan, Anand K; Bei, Jin-Xin; Rotzschke, Olaf; Chen, Meng-Hua; Cheng, Ching-Yu; Sun, Liang-Dan; Jiang, Geng-Ru; Wong, Tien-Yin; Lin, Hong-Li; Aung, Tin; Liao, Yun-Hua; Saw, Seang-Mei; Ye, Kun; Ebstein, Richard P; Chen, Qin-Kai; Shi, Wei; Chew, Soo-Hong; Chen, Jian; Zhang, Fu-Ren; Li, Sheng-Ping; Xu, Gang; Tai, E Shyong; Wang, Li; Chen, Nan; Zhang, Xue-Jun; Zeng, Yi-Xin; Zhang, Hong; Liu, Zhi-Hong; Yu, Xue-Qing; Liu, Jian-Jun

    2015-06-01

    IgA nephropathy (IgAN) is one of the most common primary glomerulonephritis. Previously identified genome-wide association study (GWAS) loci explain only a fraction of disease risk. To identify novel susceptibility loci in Han Chinese, we conduct a four-stage GWAS comprising 8,313 cases and 19,680 controls. Here, we show novel associations at ST6GAL1 on 3q27.3 (rs7634389, odds ratio (OR)=1.13, P=7.27 × 10(-10)), ACCS on 11p11.2 (rs2074038, OR=1.14, P=3.93 × 10(-9)) and ODF1-KLF10 on 8q22.3 (rs2033562, OR=1.13, P=1.41 × 10(-9)), validate a recently reported association at ITGAX-ITGAM on 16p11.2 (rs7190997, OR=1.22, P=2.26 × 10(-19)), and identify three independent signals within the DEFA locus (rs2738058, P=1.15 × 10(-19); rs12716641, P=9.53 × 10(-9); rs9314614, P=4.25 × 10(-9), multivariate association). The risk variants on 3q27.3 and 11p11.2 show strong association with mRNA expression levels in blood cells while allele frequencies of the risk variants within ST6GAL1, ACCS and DEFA correlate with geographical variation in IgAN prevalence. Our findings expand our understanding on IgAN genetic susceptibility and provide novel biological insights into molecular mechanisms underlying IgAN.

  15. Corticosteroid Treatment Influences TA-Proteinuria and Renal Survival in IgA Nephropathy.

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    Cristina Sarcina

    Full Text Available The clinical course of IgA nephropathy (IgAN and its outcome are extremely variable. Proteinuria at baseline has been considered one of the most important risk factors. More recently, mean proteinuria of follow-up (time-average proteinuria: TAp was described as a stronger marker of renal survival, suggesting to consider it as a marker of disease activity and response to treatment. We evaluated predictors of renal survival in IgAN patients with different degrees of renal dysfunction and histological lesions, focusing on the role of the therapy in influencing TAp. We performed a retrospective analysis of three prospective, randomized, clinical trials enrolling 325 IgAN patients from 1989 to 2005. Patients were divided into 5 categories according to TAp. The primary endpoint of the 100% increase of serum creatinine occurred in 54 patients (16.6% and renal survival was much better in groups having lower TAp. The median follow up was 66.6 months (range 12 to 144. The primary endpoint of the 100% increase of serum creatinine occurred in 54 patients (16,6% and renal survival was much better in groups having lower TA proteinuria. At univariate analysis plasma creatinine and 24h proteinuria, systolic (SBP and diastolic (DBP blood pressure during follow-up and treatment with either steroid (CS or steroid plus azathioprine (CS+A were the main factors associated with lower TAp and renal survival. At multivariate analysis, female gender, treatment with S or S+A, lower baseline proteinuria and SBP during follow-up remained as the only variables independently influencing TAp. In conclusion, TA-proteinuria is confirmed as one of the best outcome indicators, also in patients with a severe renal insufficiency. A 6-month course of corticosteroids seems the most effective therapy to reduce TAp.

  16. Long-Term Survival of Patients with IgA Nephropathy After Dialysis Therapy

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    Hiroyuki Komatsu

    2013-12-01

    Full Text Available Background/Aims: How dialysis affects the survival of patients with biopsy-proven IgA nephropathy (IgAN is not fully understood. The present long-term cohort study quantifies the survival rates and incidence of cardio-cerebrovascular diseases (CCVDs among such patients in Japan. Methods: Fifty-two of 433 patients with IgAN who had reached end-stage kidney disease underwent renal replacement therapy (RRT between 1981 and 2010. The overall survival rate and incidence of CCVDs in these patients were evaluated during follow-up for 11.3 ± 6.4 years. Results: The mean age at starting RRT was 42.8 ± 13.3 years. Only seven patients died during follow-up (mortality rate, 1.2/100 person-years and Kaplan-Meier analysis revealed favorable survival rates of 93.3% and 65.1% at 10 and 20 years, respectively, compared with that of patients with glomerulonephritis in the registry of the Japanese Society for Dialysis Therapy who required RRT. Malignancy and CCVDs were causes of death at 13.6 ± 4.8 and 3.9 ± 1.3 years, respectively, after starting RRT. Fatal and non-fatal CCVDs developed in 15 (incidence, 2.7/100 person-years patients and acute coronary syndrome and cerebral hemorrhage developed relatively soon after starting RRT. Cox proportional hazards models revealed that age at the time of starting RRT was a significant factor affecting the onset of CCVDs. Meanwhile, a history of having had corticosteroid as an initial treatment did not affect the onset of events. Conclusion: Although the survival of patients with IgAN is favorable after dialysis, the onset of CCVDs during the early phase of dialysis should be carefully monitored.

  17. Activation of podocytes by mesangial-derived TNF-alpha: glomerulo-podocytic communication in IgA nephropathy.

    Science.gov (United States)

    Lai, Kar Neng; Leung, Joseph C K; Chan, Loretta Y Y; Saleem, Moin A; Mathieson, Peter W; Lai, Fernand M; Tang, Sydney C W

    2008-04-01

    We have previously documented that human mesangial cell (HMC)-derived TNF-alpha is an important mediator involved in the glomerulo-tubular communication in the development of interstitial damage in IgA nephropathy (IgAN). With the strategic position of podocytes, we further examined the role of mesangial cells in the activation of podocytes in IgAN. There was no binding of IgA from patients with IgAN to podocytes. Podocytes cultured with IgA from patients with IgAN did not induce the release of growth factors or cytokines. Furthermore, podocytes did not express mRNA of known IgA receptors. In contrast, IgA-conditioned medium (IgA-HMC medium) prepared by culturing HMC with IgA from patients with IgAN for 48 h significantly increased the gene expression and protein synthesis of TNF-alpha by podocytes with a 17-fold concentration above that of IgA-HMC medium. The upregulation of TNF-alpha expression by podocyte was only abolished by a neutralizing antibody against TNF-alpha but not by other antibodies. Exogenous TNF-alpha upregulated the synthesis of TNF-alpha by podocytes in an autocrine fashion. IgA-HMC medium prepared with IgA from patients with IgAN also significantly upregulated the expression of both TNF-alpha receptor 1 and 2 in podocytes. Our in vitro finding suggests podocytes may play a contributory role in the development of interstitial damage in IgAN by amplifying the activation of tubular epithelial cells with enhanced TNF-alpha synthesis after inflammatory changes of HMC.

  18. [Linear IgA bullous dermatosis of childhood: case report].

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    Reyes-Baraona, Francisco; Andino, Romina; Carrasco, Juan Eduardo; Arriagada, Camila; Guerrero, Silvia

    2014-04-01

    Linear IgA bullous dermatosis is a rare acquired autoinmune vesiculobullous disease characterized by linear IgA deposit on the dermo-epidermal basement membrane observed with direct inmunofluorescence. The characteristic lesions are vesicles and tense serous bullae, which most often are grouped giving a "cluster of jewels" appearance. Differential diagnosis must be established with other autoimmune dermatosis, such as dermatitis herpetiformis and bullous pemphigoid. Dapsone is the first line therapy, with excellent response in a short period. This is a benign disease that tends to wax and wane in severity until it disappears spontaneously. We report the case of a 5-year-old girl presenting with bullous lesions, being diagnosed a linear IgA bullous dermatosis, with excellent response to dapsone in less than 2 weeks.

  19. Unusual clinicopathological and immunological presentation of chronic bullous dermatosis of childhood (linear IgA dermatosis

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    Al-Saif Fahad

    2011-01-01

    Full Text Available Linear IgA bullous dermatosis is a rare sulfone-responsive subepidermal blistering disorder of unknown etiology in which smooth linear deposits of IgA are found in the basement membrane zone. Chronic bullous dermatosis of childhood is equivalent to linear IgA disease of adulthood and is characterized by an abrupt onset of large, widespread and tense bullae on a normal or erythematous base. In this case, we describe an unusual presentation of chronic bullous dermatosis in a 14-month-old Saudi girl. Histopathological examination revealed subepidermal cell poor blisters with linear deposition of IgA, IgG, IgM, and C3 along the dermoepidermal junction. The unusual clinical, histopathological and immunofluorescence findings in this patient are discussed, with an account on the differential diagnosis in such cases along with a detailed review of the relevant literature.

  20. Unusual clinicopathological and immunological presentation of chronic bullous dermatosis of childhood (linear IgA dermatosis).

    Science.gov (United States)

    Fahad, Al-Saif; Ammar, Al-Rikabi

    2011-01-01

    Linear IgA bullous dermatosis is a rare sulfone-responsive subepidermal blistering disorder of unknown etiology in which smooth linear deposits of IgA are found in the basement membrane zone. Chronic bullous dermatosis of childhood is equivalent to linear IgA disease of adulthood and is characterized by an abrupt onset of large, widespread and tense bullae on a normal or erythematous base. In this case, we describe an unusual presentation of chronic bullous dermatosis in a 14-month-old Saudi girl. Histopathological examination revealed subepidermal cell poor blisters with linear deposition of IgA, IgG, IgM, and C3 along the dermoepidermal junction. The unusual clinical, histopathological and immunofluorescence findings in this patient are discussed, with an account on the differential diagnosis in such cases along with a detailed review of the relevant literature.

  1. Proliferation and Cytokine Production of Human Mesangial Cells Stimulated by Secretory IgA Isolated from Patients with IgA Nephropathy

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    Yan Liang

    2015-07-01

    Full Text Available Background/Aims: IgA nephropathy (IgAN is the most common form of primary glomerulonephritis, and often aggravates by mucosal infection. Secretory IgA (SIgA is the dominant immunoglobulin in mucosal immunity, and is deposited in the mesangium in IgAN. The biological effects of SIgA on mesangial cells are poorly understood. Methods: Deposition of SIgA in frozen renal sections from IgAN patients was detected and the association between deposition of SIgA and patients characteristics was analyzed. The biological effects of SIgA and polymeric IgA (pIgA on human renal mesangial cells were compared. We also studied the molecular mechanism of microRNA regulating the inflammatory effects of SIgA on mesangial cells. Results: Fifty-five of 176 patients had SIgA deposition with higher incidence of infection history and hematuria, lower serum cystatin C, β2 microglobulin, blood urea nitrogen and T-grade in the Oxford classification, compared with patients without SIgA deposition. SIgA stimulated mesangial cells at a higher ratio of proliferation and higher production of interleukin (IL-6, IL-8, monocyte chemotactic protein 1, transforming growth factor-β1 and fibronectin, compared with SIgA from healthy volunteers. The proliferation and cytokines production in mesangial cells stimulated by SIgA were significantly lower than that stimulated by pIgA. miR-16 targeted the 3′-untranslated region of IL-6 and suppressed its translation in mesangial cells induced by SIgA. Conclusions: The biological effects of SIgA on mesangial cells differ from those of pIgA. SIgA stimulates mesangial cell proliferation and production of proinflammatory cytokines. IL-6 production is regulated by miR-16 in mesangial cells.

  2. Mortality of IgA nephropathy patients: a single center experience over 30 years.

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    Hajeong Lee

    Full Text Available Research on the prognosis of IgA nephropathy (IgAN has focused on renal survival, with little information being available on patient survival. Hence, this investigation aimed to explore long-term patient outcome in IgAN patients. Clinical and pathological characteristics at the time of renal biopsy were reviewed in 1,364 IgAN patients from 1979 to 2008. The outcomes were patient death and end stage renal disease (ESRD progression. Overall, 71 deaths (5.3% and 277 cases of ESRD (20.6% occurred during 13,916 person-years. Ten-, 20-, and 30-year patient survival rates were 96.3%, 91.8%, and 82.7%, respectively. More than 50% patient deaths occurred without ESRD progression. Overall mortality was elevated by 43% from an age/sex-matched general population (GP (standardized mortality ratio [SMR], 1.43; 95% confidence interval [CI], 1.04-1.92. Men had comparable mortality to GP (SMR, 1.22; 95% CI, 0.82-1.75, but, in women, the mortality rate was double (SMR, 2.17; 95% CI, 1.21-3.57. Patients with renal risk factors such as initial renal dysfunction (estimated glomerular filgration rate <60 ml/min per 1.73 m(2; SMR, 1.70; 95% CI, 1.13-2.46, systolic blood pressure ≥ 140 mmHg (SMR, 1.88; 95% CI, 1.19-2.82 or proteinuria ≥ 1 g/day (SMR, 1.66; 95% CI, 1.16-2.29 had an elevated mortality rate. Patients with preserved renal function, normotension, and proteinuria <1 g/day, however, had a similar mortality rate to GP. When risk stratification was performed by counting the number of major risk factors present at diagnosis, low-risk IgAN patients had a mortality rate equal to that of GP, whereas high-risk patients had a mortality rate higher than that of GP. This investigation demonstrated that overall mortality in IgAN patients was higher than that of GP. Women and patients with renal risk factors had a higher mortality than that of GP, Therefore, strategies optimized to alleviate major renal risk factors are warranted to reduce patient mortality.

  3. Clinicopathological Features and Outcomes of IgA Nephropathy with Hematuria and/or Minimal Proteinuria

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    Min Tan

    2015-04-01

    Full Text Available Background/Aims: Information regarding the clinical and histological prognostic factors of IgA nephropathy (IgAN is mostly derived from patients in whom diagnostic renal biopsies were performed because their proteinuria levels were higher than 1-2 g/d. The clinicopathological features and outcomes of IgAN patients presenting with normal blood pressure, normal renal function, hematuria and minimal or no proteinuria are not well described. We therefore conducted a study of the clinicopathological features and outcomes in IgAN patients with these characteristics. Methods: The clinical, laboratory, and pathological manifestations and long-term outcomes of all IgAN patients with the above-mentioned characteristics were collected. The relationships between renal pathology, injury, long-term outcomes and clinical factors were studied, and the risk factors of IgAN were analyzed using multivariate logistic regression. Results: Of all of the renal biopsy cases, IgAN with the above features accounted for 8.9%. Among these patients, 67.2% (253 showed simultaneous hematuria and proteinuria, 23.1% (87 showed only hematuria, and 9.7% (36 showed only proteinuria. Additionally, 33.8% (127 patients showed macroscopic hematuria and 65.1% (245 had a prodromal infection. Regarding renal pathological changes, 45.5% (171 of the patients were unexpectedly classified as Grade II to IV (Hass classification. Proteinuria at the time of renal biopsy was an independent predictor of more severe renal pathological injury. After a median follow-up of 75 months, 61 (16.2% patients experienced adverse events. Among these patients, 28 (7.45% exhibited hypertension, 22 (5.85% presented proteinuria levels >1 g/24 h, and 11 (2.9% developed impaired renal function. Conclusions: Severe renal histological injury may be observed in some IgAN patients with benign clinical characteristics. Proteinuria is an independent predictor of severe renal pathological injury in IgAN patients with

  4. [A clinicopathological study of IgA nephropathy after tonsillectomy--with emphasis on renal histology and the timing of tonsillectomy].

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    Koichi, K; Yamaji, S; Kimura, T; Yosizawa, T; Tujie, H

    1993-08-01

    We studied the effects and the timing of tonsillectomy on IgA nephropathy by urinalysis, serum Cr, IgA, ASLO and renal histology. The renal histology was classified into three groups according to its progression. Tonsillectomy was carried out in 31 patients (12 males and 19 females) with IgA nephropathies diagnosed by renal biopsy. The average age at tonsillectomy was 30.4 years old. The effects of tonsillectomy were followed up from more than 1 year (average 36.9 months) after operation. An improvement in urinary findings was observed in 77% and in 22 cases (71%) the tonsillectomy was evaluated as having been effective based on urinary findings and renal function. The difference between the effective group and the ineffective group, in terms of the tonsillectomy, was significant for serum Cr but not for urinary findings, serum IgA or ASLO. The effective rate in groups I and II, based on renal histological classification, was 96%. In group III (advanced stage), no case was considered to show an effective response. We conclude that tonsillectomy in IgA nephropathies should be carried out at an early stage, while renal function is normal and before renal histology has progressed to that of group III.

  5. Periodontal disease bacteria specific to tonsil in IgA nephropathy patients predicts the remission by the treatment.

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    Yasuyuki Nagasawa

    Full Text Available BACKGROUND: Immunoglobulin (IgA nephropathy (IgAN is the most common form of primary glomerulonephritis in the world. Some bacteria were reported to be the candidate of the antigen or the pathogenesis of IgAN, but systematic analysis of bacterial flora in tonsil with IgAN has not been reported. Moreover, these bacteria specific to IgAN might be candidate for the indicator which can predict the remission of IgAN treated by the combination of tonsillectomy and steroid pulse. METHODS AND FINDINGS: We made a comprehensive analysis of tonsil flora in 68 IgAN patients and 28 control patients using Denaturing gradient gel electrophoresis methods. We also analyzed the relationship between several bacteria specific to the IgAN and the prognosis of the IgAN. Treponema sp. were identified in 24% IgAN patients, while in 7% control patients (P = 0.062. Haemophilus segnis were detected in 53% IgAN patients, while in 25% control patients (P = 0.012. Campylobacter rectus were identified in 49% IgAN patients, while in 14% control patients (P = 0.002. Multiple Cox proportional-hazards model revealed that Treponema sp. or Campylobactor rectus are significant for the remission of proteinuria (Hazard ratio 2.35, p = 0.019. There was significant difference in remission rates between IgAN patients with Treponema sp. and those without the bacterium (p = 0.046, and in remission rates between IgAN patients with Campylobacter rectus and those without the bacterium (p = 0.037 by Kaplan-Meier analysis. Those bacteria are well known to be related with the periodontal disease. Periodontal bacteria has known to cause immune reaction and many diseases, and also might cause IgA nephropathy. CONCLUSION: This insight into IgAN might be useful for diagnosis of the IgAN patients and the decision of treatment of IgAN.

  6. Oxidative damages in tubular epithelial cells in IgA nephropathy: role of crosstalk between angiotensin II and aldosterone

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    Lim Ai-Ing

    2011-10-01

    Full Text Available Abstract Background Inhibition of the renin-angiotensin-aldosterone system (RAAS slows down the progression of chronic renal diseases (CKD including IgA nephropathy (IgAN. Herein, we studied the pathogenetic roles of aldosterone (Aldo in IgAN. Methods Human mesangial cells (HMC was activated with polymeric IgA (pIgA from IgAN patients and the effects on the expression of RAAS components and TGF-β synthesis examined. To study the roles of RAAS in the glomerulotubular communication, proximal tubular epithelial cells (PTEC was cultured with conditioned medium from pIgA-activated HMC with eplerenone or PD123319, the associated apoptotic event was measured by the generation of nicotinamide adenine dinucleotide phosphate (NADPH oxidase and reactive oxygen species (ROS. Results Polymeric IgA up-regulated the Aldo synthesis and aldosterone synthase expression by HMC. The release of TGF-β by HMC was up-regulated synergistically by AngII and Aldo and this was inhibited by incubation of HMC with losartan plus eplerenone. Cultured PTEC express the mineralocorticoid receptor, but not synthesizing aldosterone. Apoptosis, demonstrated by cleaved PARP expression and caspase 3 activity, was induced in PTEC activated by conditioned medium prepared from HMC cultured with pIgA from IgAN patients. This apoptotic event was associated with increased generation of NADPH oxidase and ROS. Pre-incubation of PTEC with PD123319 and eplerenone achieved complete inhibition of PTEC apoptosis. Conclusions Our data suggest that AngII and Aldo, released by pIgA activated HMC, served as mediators for inducing apoptosis of PTEC in glomerulo-tubular communications. Crosstalk between AngII and Aldo could participate in determining the tubular pathology of IgAN.

  7. Clinical Experience of Professor Zhai Wen-sheng Treatment of Primary IgA Nephropathy%翟文生教授治疗免疫球蛋白A沉积性肾病(IgA nephropathy)的经验

    Institute of Scientific and Technical Information of China (English)

    赵小静

    2011-01-01

    目的:总结翟文生教授治疗原发性免疫球蛋白A沉积性肾病(IgA nephropathy)临床经验.方法:探讨该病的病因病机,提出热、瘀、虚是IgA肾病病机、辨证论治的关键;重视清热解毒利咽、活血化瘀、扶正补虚在治疗中的作用.结果与结论:翟文生教授治疗Isa肾病的经验对临床有重要的指导意义.%Objective:To summarize the clinical experience of professor ZhaiWenSheng treatment of primary IgA nephropathy. Methods:To explore the causes and pathogenesis f the disease,proposed heat, blood stasis, virtual is pathogenesis of IgA nephropathy, syndrome differentiation and treatment key, Attention clearing heat and the pharynx, promoting blood circulation to remove blood stasis, centralizer fill in the treatment of virtual function. Results and Conclusion: Professor ZhaiWenSheng treatment experience of clinical IgA nephropathy have important significance and deserves further study.

  8. Obesity-Related Nephropathy Associated with IgA Nephropathy:a Clinicopathological Analysis of Two Cases%肥胖相关性肾病伴IgA肾病2例临床病理分析

    Institute of Scientific and Technical Information of China (English)

    陈霓; 郝丽; 梁兴澜; 郑智勇; 余英豪

    2011-01-01

    Objective : To study the histomorphological characteristics, diagnosis and differential diagnosis of obesity - related glomerulopathy ( ORG ) associated with IgA nephropathy.Methods : Two cases of ORG associated with IgA nephropathy were reported and the literatures were reviewed.Results:The two cases occurred in 1 woman and 1 man, median age 35.5 years, the main symptoms of urinary abnormalities, overweight, BMI surpassed the normal value.Microscopic showed that ORG associated with IgA nephropathy included : diffuse glomeruli hypertrophy, mesangial and paramesangial deposition and a certain number of glomerular sclerosis.Immunohistochemistry showed that the IgA deposits in the mesangial area deposition.Ultrastructurally showed that mesangial deposits with a small amount of fusion of foot processes and microvilli degeneration.Conclusion: ORG associated with IgA Nephropathy is extremely rare.Its diagnosis is mainly relying on its clinical manifestation, pathology, phenotype, patients with obesity and overweight BMI.Differential diagnosis between IgA deposition associated nephropathy and glomerular sclerosis associated nephropathy is necessary.Treatments are diverse, while the prognosis is difficult to predict.%目的:探讨肥胖相关性肾病(ORG)伴IgA肾病的组织形态学特征、诊断、鉴别诊断、治疗及预后.方法:回顾性分析2例ORG伴IgA肾病的临床病理资料,并结合文献进行复习.结果:2例均为成年人,男女各1例,中位年龄35.5岁;以尿检异常为主要症状,体型肥胖,BMI均超正常值.光镜示:肾小球显著肥大,系膜区及系膜旁区沉积物伴一定数量的肾小球硬化.免疫组化显示以IgA沉积为主的系膜区沉积.电镜显示系膜区少量沉积物伴足突融合及微绒毛变性.结论:ORG伴IgA肾病非常罕见,依赖病理形态学和免疫表型,结合患者有肥胖及BMI超标即可确诊.病理学上需与IgA沉积及肾小球硬化相关肾病加以鉴别.ORG伴IgA肾病治疗方法多种,其预后较难预测.

  9. Anti-Glomerular Basement Membrane Disease Combined with IgA Nephropathy Complicated with Reversible Posterior Leukoencephalopathy Syndrome: An Unusual Case

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    Ge, Ya-ting; Liao, Jin-lan; Liang, Wei; Xiong, Zu-ying

    2015-01-01

    Patient: Male, 24 Final Diagnosis: Crescentic glomerulonephritis (type I) with IgA nephropathy Symptoms: Headache • gross hematuria • nocturia • seizures Medication: Cyclophosphamide Clinical Procedure: Dignosis to treatment Specialty: Nephrology Objective: Rare co-existance of disease or pathology Background: Anti-glomerular basement membrane disease (anti-GBM disease) is an autoimmune glomerulonephritis disease that is characterized by IgG linear deposition along the non-collagen domain of α3 chains of type IV collagen on the GBM. Although anti-GBM disease accompanied with IgA linear deposition along GBMs was discussed previously in some papers, anti-GBM disease combined with IgA granular deposition in the mesangial area, especially complicated with reversible posterior leukoencephalopathy syndrome (RPLS), was rarely reported. RPLS is usually caused by hypertensive encephalopathy, renal decompensation, fluid retention, and adverse effects of immunosuppressive drugs. Case Report: A male patient with the chief complaints of headache, gross hematuria, and nocturia was referred to our hospital. Based on renal biopsy, the diagnosis was finally confirmed as anti-GBM disease combined with IgA nephropathy and, the patient received comprehensive treatment, including cyclophosphamide (CTX), which led to symptom improvement. Two days after the third impulse CTX was given, he suddenly experienced headache and dizziness, which eventually developed into a tonic-clonic seizure. RPLS was identified by cranial magnetic resonance imaging (MRI) with reversible neuroimaging. After diazepam and antihypertension management, seizures were controlled. RPLS, a neurological complication, was found in anti-GBM disease with IgA nephropathy during our immunosuppressants therapy for the first time. Conclusions: It is worth paying more attention to patients with rapidly progressive glomerulonephritis (RPGN), as they might be complicated with RPLS during intravenous administration of CTX

  10. The Clinical Significance of Uric Acid and Complement Activation in the Progression of IgA Nephropathy

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    Yasar Caliskan

    2016-02-01

    Full Text Available Background/Aims: The aim of this study is to investigate the utility of clinical [age, gender, mean arterial pressure (MAP] and laboratory parameters [eGFR, hemoglobin (Hgb, serum levels of creatinine, uric acid, albumin, proteinuria, hematuria] and also histopathological lesions (Oxford classification parameters, crescents, intensity and pattern of staining for C3, C1Q, IgA, IgG, IgM as progression markers in patients with IgA Nephropathy (IgAN. Methods: A total of 111 IgAN patients with a follow-up period >1 year or who reached kidney failure [GFR category G5 chronic kidney disease (CKD] Results: Mean follow-up period was 33±29 months. Thirty-seven (33.3% patients progressed to kidney failure and 4 (3.6% patients developed eGFR decline ≥50% from the baseline after a median of 23 and 65 months, respectively. In multivariate Cox regression analysis, baseline levels of Hgb (HR:0.782, 95% CI 0.559-0.973, p=0.037, serum uric acid (HR:1.293, 95% CI 1.023-1.621, p=0.046, eGFR (HR:0.966, 95% CI 0.947-0.984, p=0.004 and intensity of C3 staining (HR:1.550, 95% CI 1.198-1.976, p=0.049 predicted primary endpoint. Serum uric acid level was associated independently with T score (β=0.303, p=0.005 in patients with eGFR>30 ml/min/m2. Conclusions: Hyperuricemia and the deposition of C3 are independent risk factors for IgAN progression.

  11. Atypical response to treatment in linear IgA bullous dermatosis of childhood: Revision of literature.

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    Moleiro, Susana; Santos, Vera; Calha, Manuela; Pessoa, Graça

    2011-06-15

    A three-year-old boy presented with 2 months of worsening skin lesions characterized by multiple clear vesicles and bullae. The histopathological and immunohistochemical examinations revealed changes consistent with linear IgA bullous dermatosis of childhood. Treatment with dapsone and prednisolone resulted in gradual clinical improvement. However, within a week of therapy he presented with diabetic ketoacidosis, the onset of type I diabetes mellitus. Since then, keeping this child asymptomatic has been a challenge. This case emphasizes the importance of close monitoring of patients taking systemic corticosteroids; the coexistence of other immune mediated conditions may influence the success of treatment.

  12. Discovery of new risk loci for IgA nephropathy implicates genes involved in immunity against intestinal pathogens

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    Kiryluk, Krzysztof; Li, Yifu; Scolari, Francesco; Sanna-Cherchi, Simone; Choi, Murim; Verbitsky, Miguel; Fasel, David; Lata, Sneh; Prakash, Sindhuri; Shapiro, Samantha; Fischman, Clara; Snyder, Holly J.; Appel, Gerald; Izzi, Claudia; Viola, Battista Fabio; Dallera, Nadia; Vecchio, Lucia Del; Barlassina, Cristina; Salvi, Erika; Bertinetto, Francesca Eleonora; Amoroso, Antonio; Savoldi, Silvana; Rocchietti, Marcella; Amore, Alessandro; Peruzzi, Licia; Coppo, Rosanna; Salvadori, Maurizio; Ravani, Pietro; Magistroni, Riccardo; Ghiggeri, Gian Marco; Caridi, Gianluca; Bodria, Monica; Lugani, Francesca; Allegri, Landino; Delsante, Marco; Maiorana, Mariarosa; Magnano, Andrea; Frasca, Giovanni; Boer, Emanuela; Boscutti, Giuliano; Ponticelli, Claudio; Mignani, Renzo; Marcantoni, Carmelita; Di Landro, Domenico; Santoro, Domenico; Pani, Antonello; Polci, Rosaria; Feriozzi, Sandro; Chicca, Silvana; Galliani, Marco; Gigante, Maddalena; Gesualdo, Loreto; Zamboli, Pasquale; Maixnerová, Dita; Tesar, Vladimir; Eitner, Frank; Rauen, Thomas; Floege, Jürgen; Kovacs, Tibor; Nagy, Judit; Mucha, Krzysztof; Pączek, Leszek; Zaniew, Marcin; Mizerska-Wasiak, Małgorzata; Roszkowska-Blaim, Maria; Pawlaczyk, Krzysztof; Gale, Daniel; Barratt, Jonathan; Thibaudin, Lise; Berthoux, Francois; Canaud, Guillaume; Boland, Anne; Metzger, Marie; Panzer, Ulf; Suzuki, Hitoshi; Goto, Shin; Narita, Ichiei; Caliskan, Yasar; Xie, Jingyuan; Hou, Ping; Chen, Nan; Zhang, Hong; Wyatt, Robert J.; Novak, Jan; Julian, Bruce A.; Feehally, John; Stengel, Benedicte; Cusi, Daniele; Lifton, Richard P.; Gharavi, Ali G.

    2014-01-01

    We performed a genome-wide association study (GWAS) of IgA nephropathy (IgAN), the most common form of glomerulonephritis, with discovery and follow-up in 20,612 individuals of European and East Asian ancestry. We identified six novel genome-wide significant associations, four in ITGAM-ITGAX, VAV3 and CARD9 and two new independent signals at HLA-DQB1 and DEFA. We replicated the nine previously reported signals, including known SNPs in the HLA-DQB1 and DEFA loci. The cumulative burden of risk alleles is strongly associated with age at disease onset. Most loci are either directly associated with risk of inflammatory bowel disease (IBD) or maintenance of the intestinal epithelial barrier and response to mucosal pathogens. The geo-spatial distribution of risk alleles is highly suggestive of multi-locus adaptation and the genetic risk correlates strongly with variation in local pathogens, particularly helminth diversity, suggesting a possible role for host-intestinal pathogen interactions in shaping the genetic landscape of IgAN. PMID:25305756

  13. Presence of Streptococcus mutans strains harbouring the cnm gene correlates with dental caries status and IgA nephropathy conditions

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    Misaki, Taro; Naka, Shuhei; Hatakeyama, Rina; Fukunaga, Akiko; Nomura, Ryota; Isozaki, Taisuke; Nakano, Kazuhiko

    2016-01-01

    Streptococcus mutans is a major pathogen of human dental caries. Strains harbouring the cnm gene, which encodes Cnm, a collagen-binding protein, contribute to the development of several systemic diseases. In this study, we analysed S. mutans strains isolated from the oral cavity of immunoglobulin (Ig)A nephropathy (IgAN) patients to determine potential relationships between cnm and caries status as well as IgAN conditions. Saliva specimens were collected from 109 IgAN patients and the cnm status of isolated S. mutans strains was determined using PCR. In addition, the dental caries status (decayed, missing or filled teeth [DMFT] index) in patients who agreed to dental consultation (n = 49) was evaluated. The DMFT index and urinary protein levels in the cnm-positive group were significantly higher than those in the cnm-negative group (p S. mutans strains from the oral cavity may be associated with urinary protein levels in IgAN patients, especially those with a high dental caries status. PMID:27811984

  14. Corticotherapy response in primary IgA nephropathy Resposta à corticoterapia na nefropatia da IgA primária

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    Natália Novaretti

    2013-03-01

    Full Text Available INTRODUCTION: Some beneficial effects from long-term use of corticosteroids have been reported in patients with IgA nephropathy. OBJECTIVE: This retrospective study aimed to evaluate the outcome of proteinuria and renal function according to a protocol based on a 6-month course of steroid treatment. METHOD: Twelve patients were treated with 1 g/day intravenous methylprednisolone for 3 consecutive days at the beginning of months 1, 3, and 5 plus 0.5 mg/kg oral prednisone on alternate days for 6 months (treated group. The control group included 9 untreated patients. RESULTS: Proteinuria (median and 25th and 75th percentiles at baseline in the treated group was 1861 mg/24h (1518; 2417 mg/24h and was 703 mg/24h (245; 983 and 684 mg/24h (266; 1023 at the 6th (p INTRODUÇÃO: Tem sido sugerido que tratamento mais prolongado com corticosteroides pode ser benéfico em pacientes com nefropatia da IgA primária. OBJETIVO: Neste estudo retrospectivo avaliamos os efeitos na proteinúria e na função renal após 12 meses do protocolo baseado no uso por 6 meses de corticosteroides. MÉTODO: Doze pacientes receberam pulsos de 1 g/dia de metilprednisolona intravenosa por 3 dias consecutivos no início dos meses 1, 3 e 5, seguidos por prednisona (0,5 mg/kg por via oral em dias alternados após cada pulso durante 6 meses (grupo tratado. O grupo controle foi composto por nove pacientes não tratados. RESULTADOS: A proteinúria (mg/24h; mediana; 25º; 75º percentis no período basal no grupo tratado foi de 1861 (1518; 2417 e de 703 (245; 983 e de 684 (266; 1023 nos 6º (p < 0,05 vs. basal e 12º (p < 0,05 vs. basal meses, respectivamente. No grupo controle, a proteinúria foi de 1900 (1620; 3197 no período basal e de 2290 (1500; 2975 e de 1600 (1180; 2395 nos 6º e 12º meses, respectivamente (não significantes vs. basal. Comparado com o grupo controle, o grupo tratado teve menor proteinúria (p < 0,05 e número maior de pacientes em remissão (p < 0,05 nos 6º

  15. The Akt/mTOR/p70S6K pathway is activated in IgA nephropathy and rapamycin may represent a viable treatment option.

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    Tian, Jihua; Wang, Yanhong; Guo, Haixiu; Li, Rongshan

    2015-12-01

    IgA nephropathy (IgAN) is one of the most frequent forms of glomerulonephritis, and 20 to 40% of patients progress to end-stage renal disease (ESRD) within 20 years of disease onset. However, little is known about the molecular pathways involved in the altered physiology of mesangial cells during IgAN progression. This study was designed to explore the role of mTOR signaling and the potential of targeted rapamycin therapy in a rat model of IgAN. After establishing an IgA nephropathy model, the rats were randomly divided into four groups: control, control+rapamycin, IgAN and IgA+rapamycin. Western blotting and immunohistochemistry were performed to determine phospho-Akt, p70S6K and S6 protein levels. Coomassie Brilliant Blue was utilized to measure 24-h urinary protein levels. The biochemical parameters of the rats were analyzed with an autoanalyzer. To evaluate IgA deposition in the glomeruli, FITC-conjugated goat anti-rat IgA antibody was used for direct immunofluorescence. Cellular proliferation and the mesangial matrix in glomeruli were assayed via histological and morphometric procedures. Our results showed that p70S6K, S6 and Akt phosphorylation were significantly upregulated in IgAN rats, and rapamycin effectively inhibited p70S6K and S6 phosphorylation. A low dose of the mTOR inhibitor rapamycin reduced proteinuria, inhibited IgA deposition, and protected kidney function in an IgAN rat model. Low-dose rapamycin treatment corresponded to significantly lower cellular proliferation rates and a decreased mesangial matrix in the glomeruli. In conclusion, the Akt/mTOR/p70S6K pathway was activated in IgAN, and our findings suggested that rapamycin may represent a viable option for the treatment of IgAN.

  16. Research progress of microRNA in IgA nephropathy%microRNA在IgA肾病发病机制中的研究进展

    Institute of Scientific and Technical Information of China (English)

    孙嫱; 沈颖

    2014-01-01

    IgA肾病发病机制复杂,病因不明,一些microRNA可能参与其致病过程,并且可能与其关键酶——核心β1、3半乳糖基转移酶相关.现介绍表观遗传学的定义,microRNA的概念、基本结构和功能.结合文献总结、概述目前在IgA肾病患者中已经发现的异常表达的microRNA,以及其可能的作用方式.%IgA nephropathy is a complex disease with unclear mechanism,microRNAs may involve in the disease,and some of them may be associated with the key enzyme of IgA nephropathy-core β1 3-galactosyl-transferase.The concept of epigenetics and the structures and functions of microRNA were introduced.Through reviewing the related researches,several microRNAs expressing abnormally in IgA nephropathy was found.

  17. IgA肾病遗传学的研究进展%Research Progress of IgA Nephropathy Genetics

    Institute of Scientific and Technical Information of China (English)

    齐尔

    2012-01-01

    IgA nephropathy( IgAN ) is the most common form of primary glomerulonephritis worldwide. It is a polygenic, multifactorial and complex diseases. The discovery of familial IgAJN and diverse prevalence between different populations indicate a genetic mechanism in the development of IgAJN. By linkage analysis and candidate-gene association studies to find IgAJN susceptibility gene mapping and clear disease-causing mutation has been a hotspot of genetic studies recently. Defects in IgA, glycosylation lead to formation of immune complexes which constitute a heritable risk factor for IgAJN. Continuous improvement of genomics( including genome-wide association studies )offers promising tools for elucidating the genetic basis of IgAN. In the past few years, animal models of IgAJN have made a lot of advances and have reflected the pathogenesis of IgAJN from different aspects.%IgA肾病(IgAN)是世界范围内最常见的原发性肾小球肾炎,是一种多基因、多因素决定的复杂性疾病.家族聚集起病及发病率的人种差异均提示遗传因素为重要致病机制之一.通过连锁分析和候选基因关联研究的方法找到IgAN致病易患基因图谱和明确致病突变基因已成为当前研究的热点.血清IgA1糖基化的缺陷导致免疫复合物的形成,构成了遗传性IgAN的危险因素.不断完善的基因组学方法(包括全基因组关联研究)为阐明IgAN的遗传基础提供了有力的工具.近年来IgAN动物模型研究有了许多新的进展,从不同侧面反映了IgAN的发病机制.

  18. Cyclosporine A combined with medium/low dose prednisone in progressive IgA nephropathy.

    Science.gov (United States)

    Xu, Lin; Liu, Zhong-Cheng; Guan, Guang-Ju; Lv, Xue-Ai; Luo, Qing

    2014-08-01

    The aim of the present study was to evaluate the efficacy of cyclosporine A (CsA) combined with medium/low dose prednisone in the treatment of progressive immunoglobulin A nephropathy (IgAN). Ninety-six patients who satisfied the inclusion criteria were enrolled in a prospective controlled clinical study. They were assigned into two groups and initially given either 0.6-0.8 mg/kg/day prednisone (maximum 40 mg/day) plus 3 mg/kg/day CsA (CsA group), or 1 mg/kg/day prednisone (maximum 60 mg/day) alone (steroid group). During therapy, the dose of prednisone was reduced in both groups and the dose of CsA was gradually tailed off over the first 3 months and maintained at 2 mg/kg/day in the CsA group. Urinary protein excretion, serum biochemical indexes, clinical efficacy and side effects of CsA were assayed. A significant decline in mean 24-hour urinary protein excretion (p prednisone and CsA (p 0.05). CsA at a dose of 2-3 mg/kg/day in combination with medium/low dose prednisone was effective in inducing remission of IgAN, especially for patients with Lee's Grade III IgAN, and is a safe and effective choice for short-term treatment of patients with progressive IgAN.

  19. Circulating TNF receptors 1 and 2 are associated with the severity of renal interstitial fibrosis in IgA nephropathy.

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    Yuji Sonoda

    Full Text Available The current study aimed to examine whether the levels of TNF receptors 1 and 2 (TNFR1 and TNFR2 in serum and urine were associated with other markers of kidney injury and renal histological findings, including TNFR expression, in IgA nephropathy (IgAN. The levels of the parameters of interest were measured by immunoassay in 106 biopsy-proven IgAN patients using samples obtained immediately before renal biopsy and in 34 healthy subjects. Renal histological findings were evaluated using immunohistochemistry. The levels of serum TNFRs were higher in IgAN patients than in healthy subjects. The levels of both TNFRs in serum or urine were strongly correlated with each other (r > 0.9. Serum TNFR levels were positively correlated with the urinary protein to creatinine ratio (UPCR and four markers of tubular damage of interest (N-acetyl-β-D-glucosaminidase [NAG], β2 microglobulin [β2m], liver-type fatty acid-binding protein [L-FABP], and kidney injury molecule-1 [KIM-1] and negatively correlated with estimated glomerular filtration rate (eGFR. Patients in the highest tertile of serum TNFR levels showed more severe renal interstitial fibrosis than did those in the lowest or second tertiles. The tubulointerstitial TNFR2-, but not TNFR1-, positive area was significantly correlated with the serum levels of TNFRs and eGFR. Stepwise multiple regression analysis revealed that elevated serum TNFR1 or TNFR2 levels were a significant determinant of renal interstitial fibrosis after adjusting for eGFR, UPCR, and other markers of tubular damage. In conclusion, elevated serum TNFR levels were significantly associated with the severity of renal interstitial fibrosis in IgAN patients. However, the source of TNFRs in serum and urine remains unclear.

  20. Glomerulo-tubular junction stenosis as a factor contributing to glomerular obsolescence in IgA nephropathy.

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    Sato, Mitsuhiro; Hotta, Osamu; Taguma, Yoshio

    2002-05-01

    Periglomerular fibrosis (PF) is an interstitial injury observed in various renal diseases. It is speculated that this lesion, by occluding the glomerulo-tubular junction (GTJ) and causing atubular glomeruli, may result functionally in a reduction of the glomerular filtration rate (GFR) and may be a factor contributing to the progression of renal disease. In the present study, 340 renal biopsy specimens were analysed to determine whether or not there was nephron injury derived from such a mechanism, as well as direct glomerular injury, in IgA nephropathy (IgAN). The patients were divided into five groups according to the degree of glomerular sclerosis. The average age was lower in groups with milder sclerosis and serum creatinine (Cr) was elevated in groups with more severe sclerosis. Because the GTJ was assumed to disappear when an atubular glomerulus was formed, the ratio of the number of glomeruli with discernible GTJ to the total number of glomeruli was evaluated. As glomerular sclerosis progressed, discernible GTJ reduced significantly (p <0.001) and the degree of PF increased significantly (p <0.05). By serial section study in cases with pronounced PF, transitions between the stages of stenosis of the GTJ and atubular glomeruli were observed. It is speculated that the occlusion of the GTJ eventually hyalinizes the glomerulus; in such cases, glomerular obsolescence of the collapse type might be formed. On the other hand, obsolescence of the mesangial proliferative type might be formed in the hyalinization derived from direct glomerular injury. In this context, glomerular obsolescence of the collapse type was observed more frequently and was accompanied by more increased PF than obsolescence of the mesangial proliferative type (p <0.001). These results suggest that in addition to direct glomerular injury, nephron injury derived from interstitial damage of this type plays an important contributory role in the progression of IgAN.

  1. Circulating Tumor Necrosis Factor α Receptors Predict the Outcomes of Human IgA Nephropathy: A Prospective Cohort Study.

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    Yun Jung Oh

    Full Text Available The circulating tumor necrosis factor receptors (TNFRs could predict the long-term renal outcome in diabetes, but the role of circulating TNFRs in other chronic kidney disease has not been reported. Here, we investigated the correlation between circulating TNFRs and renal histologic findings on kidney biopsy in IgA nephropathy (IgAN and assessed the notion that the circulating TNFRs could predict the clinical outcome. 347 consecutive biopsy-proven IgAN patients between 2006 and 2012 were prospectively enrolled. Concentrations of circulating TNFRs were measured using serum samples stored at the time of biopsy. The primary clinical endpoint was the decline of estimated glomerular filtration rate (eGFR; ≥ 30% decline compared to baseline. Mean eGFR decreased and proteinuria worsened proportionally as circulating TNFR1 and TNFR2 increased (P < 0.001. Tubulointerstitial lesions such as interstitial fibrosis and tubular atrophy were significantly more severe as concentrations of circulating TNFRs increased, regardless of eGFR levels. The risks of reaching the primary endpoint were significantly higher in the highest quartile of TNFRs compared with other quartiles by the Cox proportional hazards model (TNFR1; hazard ratio 7.48, P < 0.001, TNFR2; hazard ratio 2.51, P = 0.021. In stratified analysis according to initial renal function classified by the eGFR levels of 60 mL/min/1.73 m2, TNFR1 and TNFR2 were significant predictors of renal progression in both subgroups. In conclusion, circulating TNFRs reflect the histology and clinical severity of IgAN. Moreover, elevated concentrations of circulating TNFRs at baseline are early biomarkers for subsequent renal progression in IgAN patients.

  2. Outcome results in children with IgA nephropathy: a single center experience

    Science.gov (United States)

    Bulut, Ipek Kaplan; Mir, Sevgi; Sozeri, Betul; Bulut, Mustafa Orhan; Sen, Sait; Dincel, Nida

    2012-01-01

    Background Immunoglobulin A nephropathy (IgAN) is the most common primary glomerulonephritis. Patients manifest variable clinical symptoms (eg, microhematuria) with preserved or progressive deterioration of renal function resulting in end-stage renal disease. The aim of this study was to evaluate patients from a single center to describe the clinical features, treatments, and follow-up results of those with the disease. Methods This is a retrospective data study of all children with IgAN. Patients who had a histopathologically proven diagnosis of IgAN and were followed up for at least 5 years were included in the study. Renal biopsy, graded as Hass classification, was performed on all patients. A total of 39 patients were included in the study. Results The mean follow-up time (± standard deviation) was 10.4 ± 3.51 (range 5–16) years. Twenty-nine patients (74.4%) were male and ten (25.6%) were female. Nineteen (48.7%) patients presented with recurrent macroscopic hematuria, ten (25.6%) with microscopic hematuria ± proteinuria, six (15.4%) with nephritic syndrome, and four (10.3%) with nephrotic syndrome. All patients underwent a renal biopsy, which was graded according to the Hass classification. At the end of follow-up time, 18 (46.1%) patients were normal, 15 (38.5%) had minor urinary abnormalities, three (7.7%) had active renal disease, and three (7.7%) developed renal failure. Conclusion The results of the present study are better than those from most other series. The majority of children with IgAN in this study were admitted with recurrent macroscopic hematuria and found to have a good prognosis. We suggest that children with IgAN have a good prognosis in the first 5-year follow-up period. PMID:22334797

  3. Pregnancy in patients with IgA nephropathy%IgA 肾病与妊娠相关的研究进展

    Institute of Scientific and Technical Information of China (English)

    史亚男; 叶文玲

    2016-01-01

    IgA nephropathy ,as one of the most common types of glomerulonephritis ,often occurs in women of child‐bearing age .The mutual influence between IgA nephropathy and pregnancy ,and the appropriate timing of pregnancy in patients with IgA nephropathy were summarized in this review .Most studies showed that pregnancy in IgA nephropathy patients with CKD 1‐2 was not associated with increased risk of kidney disease progression .Howev‐er ,higher frequencies of caesarean delivery ,increased preterm delivery rate and lower average birth weight of new‐borns were observed in those patients .Renal insufficiency ,proteinuria and high blood pressure during pregnancy were risk factors for the adverse outcomes of pregnancy .We therefore suggest that pregnancy should be considered only for patients with blood pressure maintained within the normal range ,proteinuria being less than 1 .0 g/24 h and normal renal function or mild renal dysfunction .%Ig A肾病是最常见的原发性肾小球肾炎之一,好发于育龄期,本文主要概述了Ig A肾病与妊娠的相互影响及IgA肾病患者妊娠的时机选择和妊娠前准备。研究表明,慢性肾脏病(CKD)1~2期的IgA肾病患者妊娠不增加肾功能下降的风险,但Ig A肾病患者的剖腹产比例、早产发生率及低体质量儿发生率均高于正常人群。肾功能不全、蛋白尿、高血压是不良妊娠的危险因素。建议Ig A肾病患者具备血压维持正常、尿蛋白排泄率<1.0 g/24 h、肾功能正常或仅轻度损伤等条件时开始妊娠。

  4. Recent Research Progress of Pathogenesis of IgA Nephropathy%IgA肾病发病机制的新进展

    Institute of Scientific and Technical Information of China (English)

    郭燕

    2012-01-01

    Deposition of IgA in the glomeruli,the hallmark of IgA nephropathy,is a quite common phenomenon. Aberrant O-linked galactosylation of IgA subclass ( IgA1 ) appears to play a central role in the process and auto-immunity to a conformational epitope related to glycans at the hinge region of IgA1 is apparently required. Both circulating immune complex and in-situ immune complex mechanism have been proven to exist. Mediator systems, such as complement activation and engagement of innate immune system, also play prominent roles in determining the clinical onset and severity of disease. Progress in understanding the details of the pathogenesis of IgA nephropathy will lead to diagnosis improvement,more accurate individualized prognosis and personalized treatment regimens for this globally distributed and very common primary disease.%免疫球蛋白A(IgA)沉积于肾小球是IgA肾病的特点,是非常普遍的现象.IgA亚类(IgA1)的异常O-联半乳糖基化在这个过程中起到主要作用,针对IgA1铰链区聚多糖相关表位的自身免疫是必要的发病条件.已证明既存在循环免疫复合物也存在原位免疫复合物.先天免疫素质及中介系统,如补体系统也在临床发病及病情演变中起重要作用.对于IgA肾病详细机制的理解有助于改善诊断方法,也有助于对这一常见的原发性全球分布疾病做出更加精确的个性化预后预测及个性化治疗方法 的改进.

  5. Geographic differences in genetic susceptibility to IgA nephropathy: GWAS replication study and geospatial risk analysis.

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    Krzysztof Kiryluk

    Full Text Available IgA nephropathy (IgAN, major cause of kidney failure worldwide, is common in Asians, moderately prevalent in Europeans, and rare in Africans. It is not known if these differences represent variation in genes, environment, or ascertainment. In a recent GWAS, we localized five IgAN susceptibility loci on Chr.6p21 (HLA-DQB1/DRB1, PSMB9/TAP1, and DPA1/DPB2 loci, Chr.1q32 (CFHR3/R1 locus, and Chr.22q12 (HORMAD2 locus. These IgAN loci are associated with risk of other immune-mediated disorders such as type I diabetes, multiple sclerosis, or inflammatory bowel disease. We tested association of these loci in eight new independent cohorts of Asian, European, and African-American ancestry (N = 4,789, followed by meta-analysis with risk-score modeling in 12 cohorts (N = 10,755 and geospatial analysis in 85 world populations. Four susceptibility loci robustly replicated and all five loci were genome-wide significant in the combined cohort (P = 5×10⁻³²-3×10⁻¹⁰, with heterogeneity detected only at the PSMB9/TAP1 locus (I² = 0.60. Conditional analyses identified two new independent risk alleles within the HLA-DQB1/DRB1 locus, defining multiple risk and protective haplotypes within this interval. We also detected a significant genetic interaction, whereby the odds ratio for the HORMAD2 protective allele was reversed in homozygotes for a CFHR3/R1 deletion (P = 2.5×10⁻⁴. A seven-SNP genetic risk score, which explained 4.7% of overall IgAN risk, increased sharply with Eastward and Northward distance from Africa (r = 0.30, P = 3×10⁻¹²⁸. This model paralleled the known East-West gradient in disease risk. Moreover, the prediction of a South-North axis was confirmed by registry data showing that the prevalence of IgAN-attributable kidney failure is increased in Northern Europe, similar to multiple sclerosis and type I diabetes. Variation at IgAN susceptibility loci correlates with differences in disease prevalence

  6. The urine albumin-to-creatinine ratio is a reliable indicator for evaluating complications of chronic kidney disease and progression in IgA nephropathy in China

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    Lu Huan

    2016-05-01

    Full Text Available OBJECTIVE: This study investigated the correlation between the albumin-to-creatinine ratio in the urine and 24-hour urine proteinuria and whether the ratio can predict chronic kidney disease progression even more reliably than 24-hour proteinuria can, particularly in primary IgA nephropathy. METHODS: A total of 182 patients with primary IgA nephropathy were evaluated. Their mean urine albumin-to-creatinine ratio and 24-hour proteinuria were determined during hospitalization. Blood samples were also analyzed. Follow-up data were recorded for 44 patients. A cross-sectional study was then conducted to test the correlation between these parameters and their associations with chronic kidney disease complications. Subsequently, a canonical correlation analysis was employed to assess the correlation between baseline proteinuria and parameters of the Oxford classification. Finally, a prospective observational study was performed to evaluate the association between proteinuria and clinical outcomes. Our study is registered in the Chinese Clinical Trial Registry, and the registration number is ChiCTR-OCH-14005137. RESULTS: A strong correlation (r=0.81, p<0.001 was found between the ratio and 24-hour proteinuria except in chronic kidney disease stage 5. First-morning urine albumin-to-creatinine ratios of ≥125.15, 154.44 and 760.31 mg/g reliably predicted equivalent 24-hour proteinuria ‘thresholds’ of ≥0.15, 0.3 and 1.0 g/24 h, respectively. In continuous analyses, the albumin-to-creatinine ratio was significantly associated with anemia, acidosis, hypoalbuminemia, hyperphosphatemia, hyperkalemia, hypercholesterolemia and higher serum cystatin C. However, higher 24-hour proteinuria was only associated with hypoalbuminemia and hypercholesterolemia. Higher tubular atrophy and interstitial fibrosis scores were also associated with a greater albumin-to-creatinine ratio, as observed in the canonical correlation analysis. Finally, the albumin

  7. Expression and clinical significance of NF-κB, CTGF and OPN in mononuclear cells in peripheral blood as well as renal tissues in patients with IgA nephropathy

    Institute of Scientific and Technical Information of China (English)

    Cheng-Luo Hao; Chang-Bin Liao

    2016-01-01

    Objective:To study the expression and clinical significance of NF-κB, CTGF and OPN in mononuclear cells in peripheral blood as well as renal tissues in patients with IgA nephropathy. Methods:A total of 25 nephropathy patients diagnosed with IgA nephropathy and 25 patients receiving nephrectomy due to trauma or tumor in our hospital were studied. Peripheral blood and kidney tissues were collected to test NF-κB, CTGF, OPN, T-bet, GATA-3, RORγT and Foxp3 expressions.Results:CTGF and OPN percentages in peripheral blood mononuclear cells and kidney tissues of nephropathy patients were higher than those of the control group. NF-κB, CTGF and OPN expressions were significantly higher in M1, E1, S1 group patients’ peripheral blood mononuclear cells and renal tissues than those in M0, E1 and S1 group. T-bet, GATA-3 and RORγT expressions in nephropathy patients’ peripheral blood were significantly higher than those in the control group, and were positively correlated with NF-κB, CTGF and OPN expressions. The expression of Foxp3 was significantly lower than that of control group, and was negatively correlated with NF-κB, CTGF and OPN expressions.Conclusions: The expression of NF-κB, CTGF and OPN in peripheral blood mononuclear cells and renal tissue in patients with IgA nephropathy is abnormally high and can evaluate the prognosis of the disease and the differentiation of CD4+T cells.

  8. IgA肾病湿热证的血清蛋白质组学研究%Serum proteomics study on IgA nephropathy with Shire syndrome

    Institute of Scientific and Technical Information of China (English)

    刘垠浩; 王丽萍

    2014-01-01

    目的:应用表面加强激光解析电离-飞行时间-质谱(SELDI-TOF-MS)技术,对IgA肾病(IgAN)湿热证患者进行血清蛋白质指纹图谱检测,分析探讨该人群中所表达的特异蛋白,试图从蛋白质组层面寻找与IgA肾病湿热证相关的血清标志物。方法:采集IgA肾病患者的血清样本共29例(湿热证14例,非湿热证15例),同时选择非IgA肾病的湿热证肾病患者血清样本10例和健康人血清样本15例。采用表面增强激光解析离子化蛋白质芯片分析仪实现各组血清蛋白质表达谱的检测,后运用Biomarker WizardTM和Biomarker PatternsTM软件进行数据分析,最终识别IgA肾病湿热证特异表达的蛋白质,并建立证候决策树模型。结果:IgA肾病湿热证组与非湿热证组之间共检出有7个蛋白峰具有显著差异(P<0.05);IgA肾病湿热证组与非IgA肾病湿热证组之间共检出有4个蛋白峰具有显著差异(P<0.05);IgA肾病湿热证组与健康对照组之间共检出有5个蛋白峰具有显著差异(P<0.05);综合以上各组对比结果,并结合统计学分析证实,M/Z(质荷比)为4987.92所代表的“Beta-defensin 33蛋白”可能是IgA肾病湿热证的特异血清蛋白标志物。经筛选以M/Z为1092.71等11个蛋白峰组成的证候决策模型能很好地将IgA肾病湿热证区分出来,该模型的敏感性为92.86%,特异性为87.50%。进一步对此决策模型进行盲法验证,结果其敏感性为90.00%,特异性为86.67%。结论:IgA肾病湿热证的发生发展,可能是以M/Z为4987.92所代表的“Beta-defensin 33蛋白”的差异表达为物质基础的,同时建立了分子生物学证候决策树模型。%Objective: By applying the technology of SELDI-TOF-MS to detect the serum protein fingerprints of IgA nephropathy(IgAN) patients, analyze the serum specific proteins expressed by the IgA nephropathy patients who are diagnosed with

  9. MiR-100-3p and miR-877-3p regulate overproduction of IL-8 and IL-1β in mesangial cells activated by secretory IgA from IgA nephropathy patients.

    Science.gov (United States)

    Liang, Yan; Zhao, Guoqiang; Tang, Lin; Zhang, Junjun; Li, Tianfang; Liu, Zhangsuo

    2016-10-01

    IgA nephropathy (IgAN) is the most common type of primary glomerulonephritis, characterized by mesangial deposition of pathogenic IgA and the injury to mesangial cells. Our previous studies indicate that secretory IgA (SIgA) plays an important role in the pathogenesis of IgAN, and miR-16 is involved in destructive process in mesangial cells mediated by the SIgA from IgAN patients. Our current study aimed to study the role of miRNAs in the effect of SIgA from IgAN patients on mesangial cells. MicroRNA microarray and cytokines assay were performed to obtain the differential microRNAs expression profile in human renal mesangial cells stimulated by SIgA from IgAN patients (P-SIgA) with the cells treated by SIgA from healthy subjects (N-SgA) as control. The microRNAs with the most significant differences in microarray analysis were validated by quantitative RT-PCR. Among them, miR-100-3p and miR-877-3p were selected to predict target gene related to cytokines detecting in this study. Fifty-six differentially expressed microRNAs were chosen and 17 microRNAs with the most prominent changes were validated. Compared with N-SIgA, P-SIgA increased the production of interleukin (IL)-1β, IL-8, monocyte chemotactic protein-1 and transforming growth factor-β1. In addition, we for the first time demonstrated that over-production of IL-8 induced by the SIgA was regulated by down-expression of miR-100-3p in mesangial cells. Similarly, IL-1β over-production was regulated by down-expression of miR-877-3p. Our findings represent a pathogenic microRNAs expression profiling in human mesangial cells activated by P-SIgA. Furthermore, we provide a new explanation characterizing the molecular mechanism responsible for the regulation of IL-1β and IL-8 production in P-SIgA-triggered mesangial cells.

  10. Mesangial C3 deposition and decreased serum C3 levels in patients with IgA nephropathy

    Institute of Scientific and Technical Information of China (English)

    章晓炎

    2014-01-01

    Objective To explore the clinical significance of complement activation in Ig A nephropathy(IgAN)patients and provide new potential therapy targets.Methods Biopsy-proven IgAN patients admitted in our renal center were retrospectively recruited.Demographic,baseline clinical and pathological data were recorded as well as the follow-up results.Patients were divided into three groups,negative,weak positive and strong positive

  11. The role of Chlamydia Trachomatis in renal tissue in the pathogenesis IGA-nephropathy related to age

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    I. A. Rakityanskaya

    2012-01-01

    Full Text Available IgA-nephropathy is the most common form of primary glomerulonephritis in the world and therefore the mechanisms of this isease are actively explored. In our study, an analysis of renal biopsy issue from 117 patients IgA-nephropathy in the presence of Chlamydia trachomatis antigen related to age (before and after 60 years. It was shown that the presence of C. trachomatis in the glomerular zone influence on the severity of segmental sclerosis (p <0.05, and its presence in the interstitium affect on the size of the glomeruli (p <0.02 and severity of degeneration of epithelial tubules (p <0.02 regardless of patient age. It was shown the effect of C. trachomatis on the expression of local immune response of kidney tissue. In patients under 60 years: C. trachomatis in the glomeruli affects the number of cells of the phenotype CD25 (p = 0,04 and CD19 / κ (p = 0,034 in the glomerular infiltration and the presence of antigen in the interstitium affect the expression of CD95 (APO-1/Fas (p = 0,038 by mononuclear cells infiltration and formation of deposits S5b-C9 (p = 0,042 in the interstitial space. In patients older than 60 years of presence C. trachomatis in the glomerular zone impacts on the expression of TNF-α (p = 0,039 in the glomeruli, the presence of antigen in interstitium affect the number of cells CD71 (p = 0,025 in the interstitial infiltrate. Based on these results, we concluded that the presence of Chlamydia trachomatis antigen has an impact on the development and course of the disease and is the etiologic agent in patients with IgA-nephropathy, regardless of age.

  12. Decreased Serum C3 Levels in Immunoglobulin A (IgA) Nephropathy with Chronic Kidney Disease: A Propensity Score Matching Study

    Science.gov (United States)

    Yang, Xi; Wei, Ri-bao; Wang, Yang; Su, Ting-Yu; Li, Qing-Ping; Yang, Ting; Huang, Meng-Jie; Li, Kun-Ying; Chen, Xiang-Mei

    2017-01-01

    Background The effects of low serum C3 levels and the activation of the complement system on the development and the prognosis of IgAN are unclear. The present study aimed to determine whether decreased levels of complement C3 influence the prognosis of IgAN patients with chronic kidney disease. Material/Methods We enrolled a total of 1564 patients with primary IgAN diagnosed by renal biopsy at the Chinese PLA General Hospital from January 2011 to March 2015. The endpoint was end-stage renal disease (ESRD) or a doubling of the baseline serum creatinine (D-SCr) level. All patients were using 1: 1 propensity score matching (PSM), and the baseline values were not significantly different between these 2 groups (P>0.05). Results During a follow-up period, 14 patients in the group with decreased C3 levels reached the endpoint, with 12 patients with normal C3 levels. There was no significant difference between the 2 groups in achieving D-SCr or ESRD (P=0.676). In multivariate Cox analysis, adjusted for demographic and laboratory examination, the risk of reaching the endpoint was comparable in the 2 groups (HR, 0.70; 95% CI, 0.27–1.78; P=0.449;). Furthermore, the risk of reaching ESRD (HR, 0.83; 95% CI, 0.25–2.75; P=0.757) and D-SCr (HR, 1.45; 95% CI, 0.20–10.60; P=0.718) did not differ between the 2 groups. Conclusions Decreased serum C3 levels in IgA nephropathy with chronic kidney disease did not play a decisive role in renal progression. PMID:28166191

  13. Baseline proteinuria, urinary osmotic pressure, and renal function as positive predictors of corticosteroids plus cyclophosphamide treatment efficacy in IgA nephropathy

    Institute of Scientific and Technical Information of China (English)

    Fang Jing; Li Wenge; Li Duo; Tan Zhao

    2014-01-01

    Background Very limited data are available on factors predictive of corticosteroids plus cyclophosphamide treatment efficacy on IgA nephropathy (IgAN).The aim of the study was to research the clinical factors predictive of treatment efficacy in IgAN.Methods One hundred and fifty-nine patients with IgAN (proteinuria ≥2 g/d and estimated glomerular filtration rate 30-89 ml·min-1·1.73 m-2) were treated with corticosteroids/cyclophosphamide followed by a 12-month follow-up.According to their response,these patients were divided into remission group (proteinuria <0.5 g/d) and non-remission group (proteinuria ≥0.5 g/d),and their clinical data collected.Results In the present study,72.96% of the individuals underwent a complete remission,and their response was related to baseline proteinuria,urinary osmotic pressure,and renal function (P <0.05).Patients with baseline proteinuria more than 3 g/d,urinary osmotic pressure greater than 600 mOsm/L,and eGFR 60-89 ml·min-1·1.73 m-2 responded well to the combination of corticosteroids and cyclophosphamide (86.90% vs.57.33%,P=0.000; 81.48% vs.64.10%,P=0.014; 83.17% vs.55.17%,P=0.000).Conclusion The response to the combination of corticosteroids and cyclophosphamide might be well associated with baseline proteinuria,urinary osmotic pressure,and renal function in patients with IgAN.

  14. Osthole mitigates progressive IgA nephropathy by inhibiting reactive oxygen species generation and NF-κB/NLRP3 pathway.

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    Kuo-Feng Hua

    Full Text Available Renal reactive oxygen species (ROS and mononuclear leukocyte infiltration are involved in the progressive stage (exacerbation of IgA nephropathy (IgAN, which is characterized by glomerular proliferation and renal inflammation. The identification of the mechanism responsible for this critical stage of IgAN and the development of a therapeutic strategy remain a challenge. Osthole is a pure compound isolated from Cnidiummonnieri (L. Cusson seeds, which are used as a traditional Chinese medicine, and is anti-inflammatory, anti-apoptotic, and anti-fibrotic both in vitro and in vivo. Recently, we showed that osthole acts as an anti-inflammatory agent by reducing nuclear factor-kappa B (NF-κB activation in and ROS release by activated macrophages. In this study, we examined whether osthole could prevent the progression of IgAN using a progressive IgAN (Prg-IgAN model in mice. Our results showed that osthole administration resulted in prevention of albuminuria, improved renal function, and blocking of renal progressive lesions, including glomerular proliferation, glomerular sclerosis, and periglomerular mononuclear leukocyte infiltration. These findings were associated with (1 reduced renal superoxide anion levels and increased Nrf2 nuclear translocation, (2 inhibited renal activation of NF-κB and the NLRP3 inflammasome, (3 decreased renal MCP-1 expression and mononuclear leukocyte infiltration, (4 inhibited ROS production and NLRP3 inflammasome activation in cultured, activated macrophages, and (5 inhibited ROS production and MCP-1 protein levels in cultured, activated mesangial cells. The results suggest that osthole exerts its reno-protective effects on the progression of IgAN by inhibiting ROS production and activation of NF-κB and the NLRP3 inflammasome in the kidney. Our data also confirm that ROS generation and activation of NF-κB and the NLRP3 inflammasome are crucial mechanistic events involved in the progression of the renal disorder.

  15. Individuals of Pacific Asian origin with IgA nephropathy have an increased risk of progression to end-stage renal disease.

    Science.gov (United States)

    Barbour, Sean J; Cattran, Daniel C; Kim, S Joseph; Levin, Adeera; Wald, Ron; Hladunewich, Michelle A; Reich, Heather N

    2013-11-01

    IgA nephropathy (IgAN) accounts for a far higher proportion of end-stage renal disease (ESRD) in Asia compared with North America. It is not known whether this is entirely because of differences in disease prevalence or a higher risk of disease progression. The lack of a racially diverse population cohort followed longitudinally has previously precluded the ability to address this question. To determine whether Asians in North America with IgAN are at higher risk for ESRD, we analyzed a cohort of 202 patients of self-reported Pacific Asian origin and 467 of other origin from the Toronto GN Registry followed up for a median of 46.4 months. The primary outcome of ESRD (dialysis, transplantation, or eGFR below 15) was analyzed using Cox regression analysis. Baseline eGFR was 59.6 ml/min/1.73 m(2), and median proteinuria was 1.8 g/day. ESRD occurred in 213 patients. By univariable analysis, the risk of ESRD was similar between the two groups (hazard ratio 0.98, 95% CI 0.73, 1.31); however, after adjusting for age, gender, eGFR, medication use, blood pressure, and proteinuria, the risk of ESRD was significantly higher in Pacific Asian individuals (hazard ratio 1.56, 95% CI 1.10, 2.22). This was supported by a significant 1.62 ml/min/1.73 m(2)/year faster rate of eGFR decline (95% CI -3.19, -0.5) and an increased risk of a reduction in eGFR by half (hazard ratio 1.81, 95% CI 1.25, 2.62). Thus, in a large multiracial cohort of patients with IgAN, individuals of Pacific Asian origin have a higher risk of progression to ESRD.

  16. Genetic polymorphisms of the renin-angiotensin-aldosterone system in Chinese patients with end-stage renal disease secondary to IgA nephropathy

    Institute of Scientific and Technical Information of China (English)

    HUANG Hai-dong; LIN Fu-jun; LI Xin-juan; WANG Li-rui; JIANG Geng-ru

    2010-01-01

    Background Genetic variability in the renin-angiotensin-aldosterone system may modify renal responses to injury and disease progression. The angiotensin l-converting enzyme (ACE) gene insertion/deletion (l/D), the angiotensinogen (AGT) gene, M235T, the aldosterone synthase (CYP11B2) gene, C-344T, and the angiotensin Ⅱ type 1 receptor (AT1 R)gene, A1166C, have been shown to be associated with IgA nephropathy (IgAN) and its progression. We determined the presence of these polymorphisms in 130 Chinese patients with IgAN, including 47 patients with end-stage renal disease (ESRD) and 120 healthy Chinese subjects, to assess their impact on the susceptibility to disease and the liability of progression to ESRD.Methods Genotyping was performed with DNA isolated from peripheral leucocytes using polymerase chain reaction amplification of the polymorphic sequence, restriction enzyme digestion, and separation and identification of DNA fragments. Clinical data from renal biopsies were collected.Results ACE, AGT, CYP and AT1R genotype distributions were similar in patients with lgAN and in controls. Comparing patients with ESRD (IgAN-ESRD) and those without ESRD (IgAN-non ESRD), there was a significant increase only in the ACE DD genotype (P <0.05) among the four gene polymorphisms. There was significant dominance of the male (P <0.05), more marked hypertension (P <0.01), proteinuria (P <0.01) and increased serum creatinine during renal biopsy (P <0.01) in the IgAN-ESRD group.Conclusion Among the ACE, AGT, AT1R and CYP gene polymorphisms, only the DD genotype may predispose the individual to increased risk of progression to ESRD in the Chinese population.

  17. Nationwide epidemiological survey of childhood IgA vasculitis associated hospitalization in the USA.

    Science.gov (United States)

    Okubo, Yusuke; Nochioka, Kotaro; Sakakibara, Hiroshi; Hataya, Hiroshi; Terakawa, Toshiro; Testa, Marcia; Sundel, Robert P

    2016-11-01

    At the national level, IgA vasculitis-related hospitalizations among children in the USA are scarce. Furthermore, nationwide epidemiology and hospital course of children with IgA vasculitis have not been fully described in the USA, and disparities by race/ethnicity remain unknown. Hospital discharge records of patients aged 19 years or younger were obtained from the 2003, 2006, 2009, and 2012 Kids' Inpatient Database, and they were weighted to estimate the annual hospitalization rates with respect to age, gender, and race/ethnicity in the USA. Annual hospitalization rates were calculated using weighted case estimates and US census data. Negative binomial regression was used to ascertain the factors associated with length of hospital stay. Total annual hospitalization rates showed a significant decreasing trend, ranging from 2.45 per 100,000 children in 2003 to 1.89 per 100,000 children in 2012 (p USA across multiple age groups. GI and renal manifestations are associated with increased length of hospital stay.

  18. Clinicopathological study of IgA nephropathy patients with microalbuminuria%微量白蛋白尿对于IgA肾病患者的临床、病理研究

    Institute of Scientific and Technical Information of China (English)

    郭宗运; 吴玉梅

    2014-01-01

    目的:总结表现为微量白蛋白尿的IgA肾病( IgAN)患者的临床及病理的特点,探讨微量白蛋白尿对于IgAN的病理改变及预后的影响。方法对90例微量白蛋白尿IgAN患者的临床和病理资料进行回顾性分析,探讨其相关性。结果90例临床表现为微量白蛋白尿的IgAN患者中,14例(15.6%)存在肾功能异常,18.4%的肾功能正常的患者中存在LeeⅢ级以上的病理损害,多因素logistic回归分析显示微量白蛋白尿是IgAN病理损伤的独立危险因素。结论微量白蛋白尿是IgAN病理损伤的独立危险因素,临床表现为微量白蛋白尿的IgAN患者的病理损伤亦可能较重,微量白蛋白尿可影响IgAN患者的预后。%Objective:To Summarize the clinicopathological features of IgA nephrology( IgAN)patients with microalbu-minuria and investigate the influence of microalbuminuria to IgA nephropathy pathological changes and prognosis. Methods:The clinical and pathological data of all IgAN patients with microalbuminuria was retrospectively analyzed,to discuss the correlation. Results:14 cases(15. 6%)of IgA nephrology(IgAN)patients with clinical manifestations included microalbu-minuria existed abnormal renal function,18. 4% of patients with normal renal function existed pathological damage accord-ing with Lee’s grade Ⅲ or more severe. Multifactor logistic regression analysis revealed that microalbuminuria was inde-pendent risk factors for renal pathological lesions of IgA nephropathy. Conclusions:Microalbuminuria is independent risk factors for renal pathological lesions of IgA nephropathy,severe renal histological injury may be seen in some IgAN patients with microalbuminuria,and microalbuminuria effects the prognosis of these patients.

  19. Research progress of galactose - deficient IgA1 antibodies in diagnosis and treatment of IgA nephropathy%异常糖基化IgA1抗体在IgA肾病诊治中的作用研究进展

    Institute of Scientific and Technical Information of China (English)

    张红; 周楠; 沈颖

    2016-01-01

    研究发现,异常糖基化 IgA1抗体是导致 IgA 肾病(IgAN)发病的重要机制之一。该抗体水平与IgAN 患者蛋白尿水平及肾脏病变程度有显著相关性,检测该抗体对 IgAN 的诊断具有指导价值,其敏感性和特异性可分别达到88%~89%及92%~95%。该抗体对评估临床预后具有指导意义,现对其在 IgAN 诊治的作用进行综述,为治疗 IgAN 提供新方向。%In recent years,studies have shown that galactose - deficient IgA1(Gd - IgA1)antibodies are im-portant in the pathogenesis of IgA nephropathy(IgAN). Serum levels of Gd - IgA1 antibodies are associated with levels of proteinuria and renal histological grading. Measuring the antibodies has guidance value in the diagnosis of IgAN,for its sensitivity and specificity can be up to 88% - 89% and 89% - 92% respectively. In addition,the antibodies play an important role in clinical prognosis,and it may provide a new direction for treatment in IgAN. Now,its role in prognosis of IgAN was reviewed.

  20. 37例儿童IgA肾病临床与病理分析%Clinical and pathological features of 37 children with IgA nephropathy

    Institute of Scientific and Technical Information of China (English)

    周纬; 陈难

    2001-01-01

    为了探讨IgA肾病的临床与病理改变的关系,对37例IgA肾病进行临床分型并与肾小球、肾小管间质改变及免疫病理特点的关系进行比较。结果:临床分型中单纯血尿(血尿)18例占49%,肾病综合征(肾病)14例占38%,血尿和蛋白尿3例占8%,肾炎综合征(肾炎)2例占5%。肾小球病理损害以III级为主占54%,临床各型与肾小球病理损害无相关性。肾小管间质改变24例,血尿组7例占39%,其中I级为43%,II级为57%。肾病组均有改变,其中II级11例占78%,III级3例占22%。血尿和蛋白尿组2例占66%,肾炎组1例占50%。免疫病理改变为IgA 16例,IgAG 6例,IgAM 10例,IgAGM 5例。血尿组以单纯IgA沉积为主占66%,肾病组则以IgAM型为主占50%。提示IgA肾病临床以单纯血尿为主,其次为肾病综合征;肾小球病理损害程度与临床分型无相关性,但肾病组肾小管间质均有改变且程度也较血尿组为重。免疫病理血尿组以单纯IgA为主,而肾病组以IgAM为主。%To explore the relationship between clinical findings and pathological changes of IgA nephropathy (IgAN), 37 children with IgAN were undertaken clinical classification and renal-pathological comparison including glomerulus change, renal tubule-interstitial change and immunopathology. The results showed that there were 18 (49%) cases with hematuria, 14 (38%) cases with nephrotic syndrome, 3(8%) cases with both of hematuria and proteineuria, and 2 (5%) cases with nephritic syndrome in the clinical classification. 54% of cases with glomerulus changes was as class III. No significant relationship was found between clinical classification and glomerulus changes. There were 24 cases with renal-tubule interstitial changes and 7 cases with hematuria. 43% of them were classified as class I and 57% as class II.All cases with nephrotic syn-drome developed renal tubule-interstitial change. 78% (11 cases) of them were as class II and 22

  1. Low Birth Weight and Risk of Progression to End Stage Renal Disease in IgA Nephropathy-A Retrospective Registry-Based Cohort Study.

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    Paschal Ruggajo

    Full Text Available Low Birth Weight (LBW is a surrogate for fetal undernutrition and is associated with impaired nephron development in utero. In this study, we investigate whether having been born LBW and/or small for gestational age (SGA predict progression to ESRD in IgA nephropathy (IgAN patients.Retrospective registry-based cohort study.The Medical Birth Registry has recorded all births since 1967 and the Norwegian Renal Registry has recorded all patients with ESRD since 1980. Based on data from the Norwegian Kidney Biopsy Registry we included all patients diagnosed with IgAN in Norway from 1988-2013. These registries were linked and we analysed risk of progression to ESRD associated with LBW (defined as birth weight less than the 10th percentile and/or SGA (defined as birth weight less than the 10th percentile for gestational week by Cox regression statistics.We included 471 patients, of whom 74 developed ESRD. As compared to patients without LBW, patients with LBW had a hazard ratio (HR of 2.0 (95% confidence interval 1.1-3.7 for the total cohort, 2.2 (1.1-4.4 for males and 1.3 (0.30-5.8 for females. Corresponding HRs for SGA were 2.2 (1.1-4.2, 2.7 (1.4-5.5 and 0.8 (0.10-5.9. Further analyses showed that as compared to patients with neither LBW nor SGA, patients with either SGA or LBW did not have significantly increased risks (HRs of 1.3-1.4 but patients who were both LBW and SGA had an increased risk (HR 3.2 (1.5-6.8.Mean duration of follow-up only 10 years and maximum age only 46 years.Among IgAN patients, LBW and/or SGA was associated with increased risk for progression to ESRD, the association was stronger in males.

  2. Tubule-interstitial lesions in children with IgA nephropathy%肾小管间质损害在儿童IgA肾病中的临床意义

    Institute of Scientific and Technical Information of China (English)

    李艳红; 汪清

    2013-01-01

    目的 探讨肾小管间质损害在儿童IgA肾病中的临床意义.方法 分析住院的IgA肾病患儿的临床与病理资料,探讨IgA肾病患儿的肾小管间质损害与临床、实验室指标及其他病理参数的关系.结果 98例符合IgA肾病诊断标准的患儿中,23例未见肾小管间质损害,56例患儿存在肾小管间质轻度损害,19例存在重度损害.与无肾小管间质损害的患儿比较,肾小管间质损害患儿的病情加重,表现为血清白蛋白水平下降和血肌酐及尿素氮水平增高(P<0.05).病理可见随着肾小管间质损害程度的加重,肾小球损害程度亦加重,表现为肾小球硬化发生率逐渐增多,以及评价肾小球总体损害的积分逐渐增高(P<0.05).结论 IgA肾病患儿的肾小管间质损害与肾小球病变程度相平行,随着肾小管间质损害程度的加重,IgA肾病患儿的病情亦逐渐加重,提示肾小管间质损害与肾小球病变直接相关,肾小管间质损害可能是影响儿童IgA肾病预后不良的因素之一.%Objective To explore the clinical significance of tubule-interstitial lesions (TIL) in children with IgA nephropathy. Methods 98 children with biopsy-proven IgA nephropathy were enrolled in this study, 56 with mild TIL, 19 with severe, and 23 without TIL. Data regarding clinical and laboratory features were obtained. Semi-quantitative scores for glomerular and tubulointerstitial damage were used to evaluate renal histological lesions. Results There was a significant decrease in serum creatinine and urea nitrogen, but an increase in serum albumin, in patients with TIL compared with patients without TIL. TIL was significantly associated with increased severity of glomerular damage. Conclusion TIL correlates well with clinical and histological features in children with IgA nephropathy, which suggests TIL may be one of the factors that predispose children with IgA nephropathy to develop into end-stage renal failure.

  3. 他克莫司在难治性IgA肾病中的疗效观察%Tacrolimus in treatment of refractory IgA nephropathy:a clinical observation

    Institute of Scientific and Technical Information of China (English)

    廖丹; 肖欢; 姜丹; 张林

    2013-01-01

    Object:To observe the efficacy and safety of the combined application of Tacrolimus and moderate dose glucocorticoid in the treatment of refractory IgA nephropathy.Method:Forty-three patients with refractory IgA nephropathy were randomized to the single Glucocorticoids group(Prednisone 30 mg•d-1) and drug combination group(combined medication of Prednisone 30 mg•d-1 and Tacrolimus 1 mg•d-1). The blood trough concentration of tacrolimus was maintained at 3-7ng•dl-1. Before and after treatment of 4 weeks, 12 weeks and 24 weeks, the detection result including 24-hour urinary protein excretion, Urinary red blood cellcount, Urine specific gravity, CCr, BUN, Cr, TP, ALB, AST, ALT, GLU and the Adverse Reaction, were recorded and analyzed.Result:To the contrast, in BUN、Cr、TP、AST、ALT and GLU, there were no difference between the groups (P >0.05). Compared with the single Glucocorticoids group, 24-hour urinary protein excretion, Urinary red blood cellcount, Onset time, Complete remission rate and ALB were significantly bet er in drug combination group (P0.05)。联合治疗组24小时尿蛋白定量、尿红细胞计数、起效时间、完全缓解率、ALB和单纯激素组比较具有统计学意义(P<0.01)。结论:采用他克莫司联合中等剂量糖皮质激素治疗难治性IgA肾病的治疗效果更为显著,无明显不良反应发生,为难治性IgA肾病的治疗提供了新的方向。

  4. 伴有肉眼血尿的IgA肾病患者临床特点分析%Study on clinical features of idiopathic IgA nephropathy with gross hematuria

    Institute of Scientific and Technical Information of China (English)

    刘玺; 沈明静; 缪蕙

    2012-01-01

    Objective To study the clinical features of idiopathic IgA nephropathy with gross hematuria. Methods Seventy six patients with IgA nephropathy diagnosed by renal biopsy were divided into study group and control group according to whether with gross hematuria or not. Twenty two patients with gross hematuria were allocated in study group; and 54 patients without gross hematuria were allocated in control group. Katafuchi semi - quantitative analysis had been applied for determination of the severity of renal lesions in patients, and it combined with clinical features had been applied to analyze the characteristics of pathological changes and clinical characteristics of patients. Results ①Patients in study group were significantly higher in precursory infection rate, incidence of hypertension was lower than that of control group, serum level of creatinine was lower than that of control group, and mean duration of disease was shorter than that of control group ( P 0. 05 ). ② Scores of renal pathology, vascular scores, and scores of glomerular sclerosis and thickening of vessel wall were lower than those of control group ( P 0.05).②研究组患者肾脏病理积分、血管积分、球硬化积分及血管壁增厚积分低于对照组(P<0.05).结论 伴有肉眼血尿的IgA肾病患者发病期多伴有前驱感染,病程中高血压发生率、血肌酐水平较不伴有肉眼血尿的IgA肾病患者低.病理改变较不伴有肉眼血尿的IgA肾病轻,预后相对较好.

  5. Preliminary application of virtual touch tissue quantification imaging in diagnosis of IgA nephropathy%声触诊组织定量技术在系膜增生型IgA肾病诊断中的应用

    Institute of Scientific and Technical Information of China (English)

    梁晓宁; 郭瑞君; 李硕; 张颖; 张岩; 孙宏

    2015-01-01

    目的:探讨声触诊组织定量技术(VTQ)对系膜增生型IgA肾病的诊断价值。方法收集2013年12月至2014年7月在首都医科大学附属北京朝阳医院肾内科住院,被诊断为IgA肾病的患者85例,排除病情危重者、无法配合检查者、病理类型为非系膜细胞增生性IgA肾病者,最终入组54例系膜细胞增生型IgA肾病患者,108个肾脏。选取体检的健康志愿者54例,共计108个肾脏,作为健康对照组。使用VTQ技术分别测量健康对照组与系膜细胞增生型IgA肾病患者双肾中部肾实质及集合系统VTQ的SWV值,并进行比较。结果健康对照组双肾中部肾实质与集合系统的SWV值分别为(2.13±0.13)m/s、(1.15±0.02) m/s;IgA肾病组双肾中部肾实质与集合系统的SWV值分别为(3.07±0.62) m/s、(1.12±0.29) m/s。健康对照组肾实质与IgA肾病组肾实质SWV值比较,差异有统计学意义(t=-14.481,P0.05);健康对照组肾实质与集合系统SWV值比较,差异有统计学意义(t=-54.01,P<0.01);IgA肾病组肾实质与集合系统SWV值比较,差异有统计学意义(t=26.09,P<0.01)。伴随肾功能不全的加重,慢性肾病患者肾实质SWV值呈递增趋势(F=798.70, P<0.001)。叶间动脉阻力指数随慢性肾病1、2、3、4期逐渐增大。结论 VTQ技术对评价IgA肾病肾功能损害程度有一定诊断价值,为早期提示肾功能减低及判断其临床分期提供一个新的观察指标。%Objective To evaluate the value of virtual touch quantization (VTQ) imaging in diagnosis of IgA nephropathy. Methods The clinical data of 85 patients with IgA nephropathy were analyzed, who were treated in Capital Medical University Affiliate Beijing Chaoyang Hospital from December 2013 to July 2014. The patients who was with critical condition, unable to cooperate and with other pathological types were excluded. Finally 108 kidneys of IgA nephropathy with

  6. Expression of monocyte chemoattractant protein-1 in IgA nephropathy and its significance%IgA 肾病患者肾脏 MCP-1的表达及意义

    Institute of Scientific and Technical Information of China (English)

    钱白音; 吴锡信; 麦美芳; 张桦; 李中和; 崔彤霞

    2014-01-01

    Objective To evaluate the role of monocyte chemoattractant protein-1(MCP-1) in the mechanism of pro-gression of IgA nephropathy.Methods A total of 34 patients with biopsy proven IgA nephropathy were studied.The ex-pression of MCP-1 in renal tissues were detected by immunohistochemical staining method; the levels of serum creatinine ( Scr) were examined by sarcosine oxidase method;MCP-1 expression in renal tissue in patients with different degree of tu-bulointerstitial lesions and different levels of Scr were compared.Molecular weight of urinary protein were detected by sodi-um dodecyl sulfate-polyacrylamide gel electrophoresis( SDS-PAGE) , and were further typed;MCP-1 expression in renal tis-sue in patients with different molecular weight of urinary protein were compared.And the relationship between the MCP-1 expression and the twenty-four-hour urine protein quantitation in patients with IgA nephropathy was analyzed by pearson cor-relation analysis.Results MCP-1 expression was mainly in renal tubular epithelial cells of IgA nephropathy patients, and was in positive correlation to twenty-four-hour urine protein quantitation (r=0.34,P140μmol/L group than Scr≤140μmol/L group (P<0.05), and was significantly higher in 10 kD proteinuria group than 23 kD proteinuria group (P<0.05).Conclusion MCP-1 may play an important role in the progression of IgA nephropathy.Low molecular weight urinary protein such as 10 kD protein may have close relationship with the expression of MCP-1 in renal tissue of IgA nephropathy.%目的:观察IgA肾病患者肾脏单核细胞趋化蛋白-1(MCP-1)表达变化,并探讨其作用。方法用免疫组织化学染色法检测34例同步行肾活检诊断明确的IgA肾病患者肾脏MCP-1表达,用肌氨酸氧化酶法检测血清肌酐( Scr),比较不同程度肾小管间质病变及不同血清肌酐水平患者MCP-1的差异。用SDS-PAGE法检测尿蛋白分子量,并进一步分型,对不同

  7. Interstitial nephritis and interstitial nephropathy

    Institute of Scientific and Technical Information of China (English)

    1992-01-01

    920710 A study of the cytomegalovirus-DNA in serum and renal tissue of patients-with IgA nephropathy. LIU Zhihong(刘志红),et al. Dept Nephrol, Jinling Hosp,Nanjing,210002. Nail Med J China 1992; 72(4): 198-200. Cytomegalovirus (CMV) has been suspectedto be involved in the pathogenesis of IgA nep-

  8. 试论IgA肾病临床表现与肾脏病理的关系%the Relationship between IgA Nephropathy Clinical Manifestationand Renal Pathology

    Institute of Scientific and Technical Information of China (English)

    何东; 廖静

    2013-01-01

    目的临床分析IgA肾病患者的临床表现及肾脏病理关系。方法选取我院2011年8月至2012年8月收治的200例肾穿刺活检患者,给予B超肾活检、免疫检查、光镜检查,分析确诊的IgA临床特点与病理。结果经过检查后得知,有70例患者确诊为IgA肾病,其中15例肾病综合征、2例肾小球肾炎、9例血尿、17例隐匿性肾小球肾炎、25例慢性肾小球肾炎、1例肾功能不全、1例妊娠伴随慢性肾炎。病理类型:12例轻微病变、15例系膜增殖型、25例局灶节段性硬化、9例FSGS型、5例增生硬化型。结论针对IgA肾病患者,其临床表现和病理表现之间的严重程度不完全相符,实施肾活检,可有有效判断肾脏疾病病理类型,有利于确定治疗方案,改善预后。%Objective Clinical analysis of the relationship between theclinical manifestation and renal pathology in patients with IgA nephropathy. Methods In our hospital from 2011 August to 2012August were treated 200 cases of renal biopsy in patients with B-mode ultrasonography, renal biopsy, immunological examination, light microscopy, IgA analysis of clinical characteristics andpathologic diagnosis. Results After examination after that, of 70 patients diagnosed as IgA nephropathy, of which 15 cases of nephrotic syndrome, 2 cases of glomerulonephritis, 9 cases of hematuria, 17 cases of Latent Glomerulonephritis, 25 cases of chronic glomerulonephritis, 1 cases of renal insufifciency, 1 cases of pregnancy with chronic nephritis. Pathological types:12 cases ofmild lesion, 15 cases of mesangial proliferative type, 25 cases of focal segmental sclerosis, 9 cases of type FSGS, 5 cases ofproliferative sclerosing. Conclusion Patients with IgA nephropathy, the severity of the clinical manifestations andpathological findings between the not entirely consistent, implementation of renal biopsy, can effectively judge thepathological type of kidney disease, to determine the

  9. Pulmonary Limited MPO-ANCA Microscopic Polyangiitis and Idiopathic Lung Fibrosis in a Patient with a Diagnosis of IgA Nephropathy

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    Alwin Tilanus

    2015-01-01

    Full Text Available We present a case of a male patient with chronic renal insufficiency, due to crescentic glomerulonephritis with IgA deposits, who successively developed (idiopathic thrombocytopenic purpura (ITP and MPO-ANCA microscopic polyangiitis (MPA with pulmonary fibrosis. The patient presented with cough, weight loss, and dyspnea on exertion. CT imaging and pulmonary function tests were compatible with interstitial pneumonitis with pulmonary fibrosis. Laboratory results showed high MPO-ANCA titers; the urinary sediment was bland. The patient was treated successfully with cyclophosphamide and methyl-prednisolone. This unique case illustrates the diagnostic and therapeutic challenges of an unusual presentation of microscopic polyangiitis presenting first as isolated kidney disease with recurrence in the form of pneumonitis without renal involvement, in association with renal IgA deposits and ITP as coexisting autoimmune conditions.

  10. 来氟米特联合雷公藤多苷治疗IgA肾病的临床研究%Clnical study of Leflunomide combined with Tripterygium Wilfordii in treatment of IgA nephropathy

    Institute of Scientific and Technical Information of China (English)

    隋剑颖

    2011-01-01

    Objective: To observe the clinical efficacy of Leflunomide combined with Tripterygium Wilfordii on IgA nephropathy. Methods: 80 patients were randomly divided into treatment group and control group, each group contained 40 cases. The treatment group was given Leflunomide combined with Tripterygium Wilfordii, and the control group with given Enalapril, and both group continued for therapeutical period of 12 months. Results: Total effective rate of treatment group with 87.5% was obviously higher than the control group's 62.5%, and there was statistical difference (x2=12.56, P< 0.05). After treatment, urine protein of treatment group was (0.63±0.21) g/24h, and serum albumin was (41.32±13.42) g/L, both compared with those of control group has statistical difference (all P<0.05). Conclusion: Leflunomide combined with Tripterygium Wilfordii could difinitely reduce urine protein and raise serum albumin of IgA nephropathy, with less side effect.%目的:观察来氟米特联合雷公藤多苷治疗IgA肾病的临床效果.方法:将IgA肾病患者80例随机分为治疗组和对照组,各40例.治疗组给予来氟米特联合雷公藤多苷,对照组给予依那普利,疗程均为12个月.结果:治疗组总有效率为87.5%(35/40),对照组为62.5%(25/40),差异有统计学意义(X2=12.56,P<0.05).治疗组治疗后尿蛋白为(0.63±0.21)g/24 h,血清白蛋白为(41.32±13.42)g/L,均较治疗前及与对照组治疗后差异有统计学意义(均P<0.05).结论:两药联用治疗IgA肾病患者能提高患者的血清白蛋白,显著降低患者的尿蛋白,副作用少.

  11. 查表型饮食疗法在IgA肾病患者饮食管理中的应用%The application of chart diet therapy on nutrition management in patients with IgA nephropathy

    Institute of Scientific and Technical Information of China (English)

    孙莉莉; 娄小平; 王荟苹; 张军军

    2016-01-01

    目的:评价查表型饮食疗法在IgA肾病患者饮食管理中的应用效果。方法:将72例IgA肾病患者随机分为实验组与对照组,实验组采用查表型饮食疗法制定食谱,只需要查表即可找到符合患者特点的每天食谱,对照组采用传统细算法,随访6个月,评价住院第1天、随访3个月、随访6个月饮食达标情况、SGA评分、血清白蛋白、肾小球滤过率等生理指标。结果:实验组与对照组比较,饮食达标较高,SGA评分较低,血清白蛋白、血红蛋白、24小时尿蛋白定量、BMI等生理指标得到改善。结论:查表型饮食疗法操作简便,可提高患者饮食治疗的依从性,改善患者营养状况。%Objective: To evaluate the effect of chart diet therapy on nutrition management in patients with IgA nephropathy. Methods: Totally 72 IgA nephropathy patients were randomly divided into experimental group and control group. The experimental group used the chart diet therapy to create meal plans, while the control group used the traditonal calories calculation method following up in 6 month and evaluating the nutrition status, SGA, Alb, TG, CHOLand other physiological indexes at three different times, first day of admission, 3 month follow up and 6 month follow up. Result: Compared with control group, the nutrition statu, SGA, Alb, Hb, 24-UP, BMI of experimental group were better. Conclusion: The chart diet therapy was easy to operate, and could improve the compliance and nutrition status.

  12. Progress of Toll-like receptors and the pathogenesis of IgA nephropathy%Toll样受体与IgA肾病发病机制的研究进展

    Institute of Scientific and Technical Information of China (English)

    王云

    2014-01-01

    IgA肾病是儿童和青少年时期最常见的原发性肾小球肾炎,是引起终末期肾功能衰竭的重要原因之一,其病理特征是以IgA或IgA为主的免疫复合物在肾小球系膜区沉积.近年来免疫因素异常成为其研究热点,但具体发病机制尚未被完全阐明.Toll样受体是进化中比较保守的一个受体家族,它能特异地识别病原相关的分子模式,不仅在激活天然免疫中发挥重要作用,而且还调节获得性免疫,是连接天然免疫和获得性免疫的桥梁.研究显示Toll样受体在IgA肾病发生、发展过程中起着重要作用.该文就其生物学特征及IgA肾病发病机制进行综述.%Immunoglobulin A nephropathy (IgAN),characterized pathologically by deposition of IgA complexes in the glomerular mesangium,is the most common form of primary glomerulo-nephritis in children and adolescents and is considered to be one of the important cause of end-stage renal failure.Abnormal immune factors become the research hotspot in recent years,but the specific pathogenesis has not yet been fully elucidated.Toll-like receptors are a family of receptors,which have been evolutionarily conserved to recognize pathogen-associated molecular patterns.Toll-like receptors do not only play an important role in activation of innate immune,but also regulate acquired immune,which is a bridge connecting the natural immune and acquired immune.Studies have shown that Toll-like receptors plays an important role in the process of occurrence and development of IgAN.This paper reviews its biological characteristics and the pathogenesis of IgA nephropathy.

  13. Dual renin-angiotensin system blockade plus oral methylprednisone for the treatment of proteinuria in IgA nephropathy Doble bloqueo del sistema renina-angiotensina más metilprednisona oral para el tratamiento de la proteinuria en la nefropatía por IgA

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    Hernán Trimarchi

    2007-10-01

    Full Text Available Renin-angiotensin system inhibition is a widely accepted approach to initially deal with proteinuria in IgA nephropathy, while the role of immunosuppressants remains controversial in many instances. A prospective, uncontrolled, open-label trial was undertaken in patients with biopsy-proven IgA nephropathy with proteinuria > 0.5 g/day and normal renal function to assess the efficacy of a combination treatment of angiotensin converting enzyme inhibitors plus angiotensin receptor blockers enalapril valsartan coupled with methylprednisone to decrease proteinuria to levels below 0.5 g/day. Twenty patients were included: Age 37.45 ± 13.26 years (50% male; 7 patients (35% were hypertensive; proteinuria 2.2 ± 1.86 g/day; serum creatinine 1.07 ± 0.29 mg/dl; mean follow-up 60.10 ± 31.47 months. IgA nephropathy was subclassified according to Haas criteria. Twelve patients (60% were class II; seven (35% were class III and one (5% class V. All patients received dual reninangiotensin system blockade as tolerated. Oral methylprednisone was started at 0.5 mg/kg/day for the initial 8 weeks and subsequently tapered bi-weekly until the maintenance dose of 4 mg was reached. Oral steroids were discontinued after 24 weeks (6 months of therapy but renin-angiotensin inhibition remained unchanged. At 10 weeks of therapy proteinuria decreased to 0.15 ± 0.07 g/day (P El doble bloqueo del sistema renina-angiotensina con inhibidores de la enzima convertidora de angiotensina junto a bloqueadores del receptor tipo I de angiotensina II es aceptado como tratamiento en la proteinuria de la nefropatía por IgA, ya que el rol de los inmunosupresores continúa siendo controvertido. Estudio prospectivo, no controlado, abierto para pacientes con nefropatía por IgA con proteinurias >0.5 g/día y creatininas séricas <1.4 mg/dl, para evaluar la eficacia de tratamiento de enalapril más valsartán asociado a metilprednisona vía oral para disminuir las proteinurias a <0.5 g

  14. Nefropatia por IgA em portadores de espondiloartrites acompanhados no Serviço de Reumatologia do Hospital das Clínicas da UFMG IgA nephropathy in patients with spondyloarthritis followed-up at the Rheumatology Service of Hospital das Clínicas/UFMG

    Directory of Open Access Journals (Sweden)

    Daniela Castelo Azevedo

    2011-10-01

    -B27 positivity, creatinine and urea serum levels, major comorbidities, hematuria and proteinuria. Patients with hematuria were subsequently assessed for the presence of dysmorphic red blood cells in urine, and those with proteinuria underwent 24-hour urine protein measurement. Renal biopsy was performed in patients with glomerular hematuria and/or proteinuria over 3.5 g/24-hour. RESULTS: Seventy-six patients were assessed. Microscopic hematuria was the most frequently found abnormality in urinalysis (44.7%, usually intermittent and in spot urine samples during patients' follow-up. In eight patients (10.5%, glomerular hematuria was suspected. Renal biopsy was performed in fi ve of them, showing IgA nephropathy in four (5.3% and thin membrane disease in one patient. CONCLUSIONS: A high frequency of urinalysis alterations was observed in that subgroup of patients, as well as a high prevalence of IgA nephropathy. Although further studies on this subject are needed to better clarify these results, periodic urinalysis of patients with spondyloarthritis should be recommended.

  15. The role of activation of the lectin pathway of complement in pathogenesy of IgA nephropathy%补体活化的凝集素途径在IgA肾病发病中的作用

    Institute of Scientific and Technical Information of China (English)

    张伟; 王墨; 李秋

    2011-01-01

    To investigate the mechanism of activation the lectin pathway of complement in IgA nephropathy,enzyme-linked immunosorbent assay was performed to detect mining level of serumal mannose-binding lectin (MBL); immunohistochemical method was performed to detect expression of MBL、MASP-1、C3、Clq、IgA and C5b-9 on renal specimens. Patients of IgAN were characterized into MBL-positive cases and MBL-negative cases by glomerular co-deposition of MBL and MASP, and were analyzed distinction of couch expressionition retrospectively. Glomerular deposition of MBL was observed in 22 of 53 cases (41.5%) with IgAN and showed a mesangial pattern. All MBL-positive cases, but none of the MBL-negative cases showed glomerular co-deposition of MBLassociated serine proteases, C3 and C5b-9. Among patients of IgAN, serum level of MBL did not show significant difference than unimpaired patients. Patients of IgAN with glomerular MBL deposition, pathology classification was not correlation with intension of MBL deposition. Intension of MBL deposition was positive correlation with IgA in patients of IgAN. MBL-positive cases have significantly more proteinuria and more attacking macroscopic hematuria as MBL-negative cases, which shows activation of the mero-lectin pathway of complement is significant in IgA nephropathy.%目的 探讨补体活化的凝集素途径在IgA1肾病发病中的作用.方法 用ELISA方法 检测20例IgAN患儿和正常儿童血清的MBL水平,免疫组化检测53例IgA肾病、23例非IgA沉积肾小球疾病患儿1肾组织甘露聚糖结合凝集素(MBL)、MBL相关的丝氨酸蛋白酶(MASP-1)、C3、C1q、IgA和C5b-9的表达,了解有无MBL-MASP-1途径补体活化参与IgAN发病;回顾性分析MBL-MASP-1沉积阳性组和MBL-MASP-1沉积阴性组患儿不同临床表现的差异;分析IgAN的MBL沉积强度与病理分级的相关性,了解不同程度补体活化与IgAN病理损害的关系.结果 22例IgAN患儿肾小球有MBL、MASP-1、C3和C5b-9

  16. leflunomide对IgA肾病大鼠肾脏保护作用的机制研究%Study on protective effects and its mechanism of leflunomide on renal tissue in rat IgA nephropathy model

    Institute of Scientific and Technical Information of China (English)

    汤颖; 娄探奇; 王成; 彭晖; 刘迅; 唐骅

    2008-01-01

    目的 观察leflunomide对实验性IgA肾病(IgAN)大鼠肾脏病理及肾组织转化生长因子-β1(TGF-β1)、单核细胞趋化因子-1(MCP-1)表达的影响.方法 建立IgAN大鼠模型,随机分成模型组、泼尼松组、leflunomide组,同时设立正常对照组.用免疫荧光和光镜观察免疫复合物在肾脏的沉积及系膜区基质增生程度;用免疫组化和逆转录-聚合酶链反应(RT-PCR)方法分别检测肾组织TGF-β1、MCP-1的蛋白和基因表达水平.结果 与模型组比较,leflunomide组免疫复合物在肾脏的沉积明显减少,系膜区基质增生程度显著减轻(P均<0.01);leflunomide在基因和蛋白水平均能够有效抑制TGF-β1和MCP-1在肾组织中的表达(P<0.05或P<0.01).结论 leflunomide能减少免疫复合物在肾脏的沉积,并且下调TGF-β1、MCP-1在肾脏的表达,减少局部炎症反应,减轻系膜区基质增生,延缓肾脏纤维化的进程,保护肾脏.%Objective To observe the effects of leflunomide on renal pathology and expression of transforming growth factor-β1 (TGF-β1), monocyte chemotaxis peptidel (MCP-1) in renal tissue of experimental IgA nephropathy in rat. Methods IgA nephropathy model was reproduced in rats. They were randomly divided into leflunomide group, prednisone group, nephropathy control group, and normal control group. The deposition of immunocomplex in renal tissue and degree of mesangial matrix hyperplasia in mesangial region were detected by immunofluorescence and light microscope;the level of expression of gene and protein of TGF-β1 and MCP-1 in renal tissue were determined by immunohistochemistry and reverse transcription-polymerase chain reaction (RT-PCR) methods. Results Compared with model group,leflunomide lessened the deposit of immunocomplex in renal tissue, alleviated the hyperplasia of mesangial matrix (all P<0. 01). Leflunomide could also inhibit the expression of TGF-β1, MCP-1 at the level of gene and protein in renal tissue (P<0

  17. Value of urine NGAL and N - acetyl - β - D - glucosaminidase in early diagnosis of IgA nephropathy%尿NGAL和N-乙酰-β-D-氨基葡萄糖苷酶对IgA肾病早期诊断的价值研究

    Institute of Scientific and Technical Information of China (English)

    王寅; 童俊容; 何凤; 罗正茂; 张虹

    2011-01-01

    Objective:To explore the value of urine NGAL and N - acetyl - p - D - glucosaminidase in diagnosing early injury of IgA nephropathy. Methods: 85 patients with lgA nephropathy were categorized into two groups ( Hass I - II group and Hass III~ V group). 30 healthy subjects were recruited as controls. Ungal and Unag were detected by commercial available ELISA kit according to the manufacturer's instructions. And the ratio of Ungal/Cr and Unag/Cr were calculated. Receiver operating characteristic (ROC) curve analysis was used to evaluat the two on the sensitivity and specificity of diagnosis on IgA nephropathy. The area under the ROC curve and the best diagnostic cut -off value were calculated. Results: The levels of Ungal,Unag,Un-GAL/Cr and Unag/Cr are significantly higher in IgAN patients than in control group. The area under ROC curve of Ungal/Cr and Unag/Cr were 0.941 and 0.846 respectively. The best diagnostic cut - off value were 3.50 g /mmol Cr and 1.20 U /mmol Cr. Taking the boundary value to diagnose IgA nephropathy, the sensitivity and specificity for Ungal/Cr and Unag/Cr were 91.2% , 93.7% and 83.3% , 84.9% respectively. Conclusion: Ungal is an objective indicator of early renal damage in IgA nephropathy. Ungal/Cr has higher sensitivity and specificity for the diagnosis of IgA nephropathy than Unag /Cr,and it is expected to be diagnostic indicator for IgA nephropathy.%目的:探讨尿中性粒细胞明胶酶相关脂蛋白(neutrophil gelatinase associated lipocalin,NGAL)和N-乙酰-β-D-氨基葡萄糖苷酶(N - acetyl -β-D - glucosaminidase,NAG)对IgA肾病早期诊断价值.方法:选择IgA肾病患者85例,根据Hass分级Hass Ⅰ~Ⅱ(A组)40例、Hass Ⅲ~V(B组)45例;正常对照组30例.应用ELISA法检测尿液NGAL(uNGAL)和uNAG的浓度,并计算uNGAL/Cr比值和uNAG/Cr比值,进而对uNGAL/Cr和uNAG/Cr进行受试者工作特征(ROC)曲线分析,计算ROC曲线下面积和最佳诊断界值.结果:IgA肾病患者的uNGAL、uNAG、uNGAL/Cr

  18. Using Two Kinds of BSA Dose Establish IgA Nephropathy Rats Model Bservation%运用两种BSA剂量建立IgA肾病大鼠模型观察

    Institute of Scientific and Technical Information of China (English)

    张静; 李静; 桑晓红; 刘健; 王丽娜; 苗娜

    2013-01-01

    目的:目前IgA肾病(IgAN)动物模型并无经典造模方法,本实验选择国内较常用方法建立大鼠IgAN模型,分两个时间点观察不同剂量牛血清白蛋白(bovine serum albumin,BSA)灌胃联合注射脂多糖(lipopolysaccharide,LPS)+四氯化碳(carbon terachloride,CCL4)方法建立IgA肾病模型效果.方法:将SPF级健康雄性SD大鼠60只(平均体重200~240 g)随机分为3组,每组20只.低剂量组(A组):BSA400 mg/kg(100 g/L)隔天灌胃,持续12周;皮下注射蓖麻油0.3 ml+CCL4 0.10 ml,每周1次持续12周,第8周LPS 0.05 mg尾静脉注射.高剂量组(B组):BSA增加至600 mg/kg(100 g/L),余干预同低剂量组.对照组(C组)生理盐水4 ml/kg灌胃,0.4 ml皮下注射.建模后第8周、12周每组各处死10只大鼠,留取血、尿、肾组织标本检测.观察指标:一般情况、蛋白尿、血尿、肝肾功能、肾组织病理.结果:模型组大鼠血肌酐、尿素氮及尿蛋白均明显增高(P<0.05),高剂量组显著高于低剂量组(P<0.05),且肾组织病理8周后均出现轻中度系膜细胞及系膜基质的增生,12周末显著伴肾间质轻度纤维化,高剂量组病变较重;12周末均有荧光出现,均强于第8周,低剂量组荧光表达不典型,高剂量组表达强于低剂量组(P<0.05).电镜报告与病理表现一致.结论:运用BSA+LPS+CCL4联合造模方法在12周左右可建立快速IgA肾病动物模型,而且高剂量BSA(600 mg/kg)组肾组织病变更显著,模型更典型.此模型的建立及运用可以为IgA肾病临床及基础研究提供良好动物模型基础.%Objective:At present there is no classic method to construct IgAN model in SD rats. To observe IgA nephropa-thy model effect, the experiment divided into two time points at different concentrations bovine serum albumin lavage combined injection lipopolysaccharide + carbon tetrachloride method to establish IgA nephropathy model effect. Methods: Sixty health male SD rats weighing 200 ~ 240 g were randomized

  19. Correlation of the clinical features and the Oxford pathological type of primary IgA nephropathy%原发性IgA肾病临床与IgA肾病牛津分类的相关性

    Institute of Scientific and Technical Information of China (English)

    周宏伟; 苏震; 朱慧萍; 林海霞; 许菲菲; 吕吟秋; 陈波

    2013-01-01

    目的:探讨IgA肾病牛津分类与临床指标之间的相关性,为进一步指导治疗、判断预后提供依据.方法:选取2010年7月至2012年6月在温州医科大学附属第一医院经肾活检确诊的原发性IgA肾病患者205例,结合其临床和病理资料,按不同条件分组,进行临床病理分析.结果:按IgA肾病牛津分类对患者的病理资料进行评价,病理损伤以局灶节段性肾小球硬化(S1)多见,病理类型以系膜细胞增多(M0型)节段性肾小球硬化(S1型)毛细血管内细胞增多(E0型)肾小管萎缩/间质纤维化(T0型)即M0S1E0T0多见.患者临床表现与病理之间的相关性分析:①系膜细胞增多(M)、毛细血管内细胞增多(E)、肾小管萎缩/间质纤维化(T)与肾活检时蛋白尿高度相关(P<0.05);②毛细血管内细胞增多(E)、肾小管萎缩/间质纤维化(T)、肾微小动脉病变与肾活检时平均动脉压(MAP)升高相关(P< 0.05);③系膜细胞增多(M)、肾小管萎缩/间质纤维化(T)与肾活检时血肌酐升高相关(P< 0.01).结论:原发性IgA肾病临床表现多样,病理类型以M0S1E0T0多见.原发性IgA肾病临床表现与IgA肾病牛津分类之间具有相关性,IgA肾病牛津分类与临床特点有关,可以有效地指导临床.%Objective:To study the correlation between clinical features and the Oxford pathological classification in IgA nephropathy (IgAN).Methods:Two hundred and five IgAN patients from the First Affiliated Hospital of Wenzhou Medical College which had been diagnosed by renal biopsy from July 2010 to June 2012 were reviewed.All of the pathological and clinical data were analyzed with SPSS 16.0.Results:Segmental glomerulosclerosis was the main pathological damage and M0S1E0T0 was the major type in renal histology.During the patient biopsy,the correlation was presence as follows:(① The proteinuria was related to renal pathological findings (including the mesangial proliferation,endocapillary proliferation

  20. 浅析活性维生素D联合血管紧张素受体拮抗剂治疗IGA肾病有效性%Analysis of the Effectiveness of Active Vitamin D Combined With Angiotensin Receptor Antagonist in the Treatment of IGA Nephropathy

    Institute of Scientific and Technical Information of China (English)

    郭陆晋

    2015-01-01

    目的:分析活性维生素D联合血管紧张素受体拮抗剂治疗IGA肾病有效性。方法随机抽取2010年4月~2014年11月诊治的80例IgA肾病患者,分为研究组和对比组。对比组服用厄贝沙坦进行治疗。而研究组在对比组的基础上增加骨化三醇胶丸进行治疗,分析两组治疗效果。结果在治疗结束之后,两组患者的尿蛋白的数值低于治疗(P 0.05)。结论活性维生素D联合血管紧张素受体拮抗剂治疗IGA肾病效果显著,其安全性高。%Objective To analyze the effectiveness of active vitamin D combined with angiotensin receptor antagonist in the treatment of IGA nephropathy. Methods 80 patients with IgA nephropathy in April 2010~November 2014 were randomly divided into study group and control group. Contrast group took irbesartan treatment. The research group on the basis of the comparison group increased Calcitriol Soft Capsules treatment, analysis of the treatment effect of the two groups.ResultsAfter the treatment, the urine protein of the two groups was significantly lower than that of the treatment (P 0.05). Conclusion Active vitamin D combined with angiotensin receptor antagonist in the treatment of IGA nephropathy is remarkable, and its safety is high, it is worthy of clinical application.

  1. Effects and safety of low doses of leflunomide combined with the adefovir dipivoxilin in the treatment of patients with IgA nephropathy and hepatitis B virus%小剂量来氟米特联合阿德福韦酯治疗合并慢性乙肝的IgA肾炎临床观察

    Institute of Scientific and Technical Information of China (English)

    郭永兵

    2011-01-01

    目的 观察小剂量(10mg)来氟米特联合阿德福韦酯治疗合并慢性乙肝的IgA肾炎的临床疗效和安全性.方法 22例伴乙肝的IgA肾炎和24例不伴乙肝的IgA肾炎分成两组,分别给予小剂量和全剂量(20 mg)的来氟米特进行为期6个月的临床观察.结果 两组蛋白尿均明显减轻(P<0.01)),两组总有效率差异无统计学意义(P>0.05);血清白蛋白均明显提高(P<0.01);血脂紊乱改善且两组均没有严重的不良反应.结论 小剂量的来氟米特治疗伴乙肝的IgA肾炎疗效肯定,无严重的不良反应.%Objective To investigate the effects and safety of low doses of leflunomide (10 mg/d) combined with the adefovir dipivoxil in the treatment of patients with IgA nephropathy and hepatitis B virus.Methods Twenty -two patients with IgA nephropathy and hepatitis B virus were treated with low doses of leflunomide combined with the adefovir dipivoxil.Another 24 cases of patients with IgA nephropathy no hepatitis B virus were treated with normal doses of leflunomide(20 mg/d) combined with the adefovir dipivoxil.Their therapeutic effects and safety were recorded and analysed through six months clinical observations respectively.Results Urinary protein of two group patients were both decreased obviously (P < 0.01 ) ).Serum albumin of two group patients were both obviously improved (P < 0.01 ).The total effectiveness of the two groups were not statistically significant (P >0.05 ).Only mild tolerable adverse effects were observed.Conclusions Low doses of leflunomide combined with adefovir dipivoxil therapy can be one of the safe and effective choices for treatment of patients with IgA nephropathy and hepatitis B virus.

  2. Concurrent Drug-Induced Linear Immunoglobulin A Dermatosis and Immunoglobulin A Nephropathy

    OpenAIRE

    Kim, Ji Seok; Choi, Misoo; Nam, Chan Hee; Kim, Jee Young; Park, Byung Cheol; Kim, Myung Hwa; Hong, Seung Phil

    2015-01-01

    Diseases associated with immunoglobulin A (IgA) antibody include linear IgA dermatosis, IgA nephropathy, Celiac disease, Henoch-Schönlein purpura, etc. Although usually idiopathic, IgA antibody is occasionally induced by drugs (e.g., vancomycin, carbamazepine, ceftriaxone, and cyclosporine), malignancies, infections, and other causes. So far, only a few cases of IgA bullous dermatosis coexisting with IgA nephropathy have been reported. A 64-year-old female receiving intravenous ceftriaxone an...

  3. 泼尼松联合霉酚酸酯结合中药治疗重症 IgA 肾病%Prednisone and Mycophenolate Mofetil Combined with Traditional Chinese Medicine for Treatment of Severe IgA Nephropathy

    Institute of Scientific and Technical Information of China (English)

    王丽伟

    2013-01-01

    目的:比较泼尼松联合霉酚酸酯(MMF)结合中药汤剂与泼尼松联合环磷酰胺(CYC)治疗重症IgA肾病的临床疗效。方法:84例重症IgA肾病患者根据入院先后顺序按随机原则分别给予泼尼松联合MMF结合中药汤剂治疗(MMF组,n=42),或采用泼尼松联合CYC治疗(CYC组n=42),持续12个月。两组患者基础病情无差异,随访时间18个月,观察两组患者临床缓解率以及相应实验室指标如24 h尿蛋白定量、血肌酐、血浆白蛋白、总蛋白、血脂变化,并比较两组治疗的不良反应。结果:1)临床缓解率:治疗18个月时MMF组临床总有效率高于CYC组,分别为85.7% VS 61.8%(P<0.05);2)观察期末,MMF组患者24 h尿蛋白定量(0.6±0.3)明显低于CYC组(1.4±0.5)(P<0.05),血浆白蛋白和总蛋白(43.2±4.3和70.2±8.1)均显著高于CYC组患者(36.9±3.6和60.3±7.6)(P<0.05);3)血脂变化:MMF组血脂较治疗前明显降低,而CYC组无变化(P<0.05);4)不良反应发生率:MMF组不良反应发生率(4.76%)明显低于CYC组(19.0%)。结论:泼尼松联合MMF治结合中药汤剂疗重症IgA肾病,临床缓解率高于泼尼松联合CYC静脉滴注疗法,能更有效降低患者24 h尿蛋白定量,改善患者血脂和血浆白蛋白水平,维持患者肾功能稳定,并且MMF不良反应发生率显著低于CYC疗法。%Objective:To compare efficacy of 1) Prednisone and mycophenolate mofetil (MMF) and herbal decoctions with 2) predni-sone and cyclophosphamide ( CYC ) for treating severe IgA nephropathy .Methods:Eighty-four patients with severe IgA nephropathy were randomly given prednisone and MMF and herbal decoction (MMF group, n=42), or treated with prednisone and CYC (CYC group n=42), for 12 months.Patients were of no difference in terms of basic illness severity .In 18-month followups, observe clinical effective rate and the

  4. Concurrent Drug-Induced Linear Immunoglobulin A Dermatosis and Immunoglobulin A Nephropathy.

    Science.gov (United States)

    Kim, Ji Seok; Choi, Misoo; Nam, Chan Hee; Kim, Jee Young; Park, Byung Cheol; Kim, Myung Hwa; Hong, Seung Phil

    2015-06-01

    Diseases associated with immunoglobulin A (IgA) antibody include linear IgA dermatosis, IgA nephropathy, Celiac disease, Henoch-Schönlein purpura, etc. Although usually idiopathic, IgA antibody is occasionally induced by drugs (e.g., vancomycin, carbamazepine, ceftriaxone, and cyclosporine), malignancies, infections, and other causes. So far, only a few cases of IgA bullous dermatosis coexisting with IgA nephropathy have been reported. A 64-year-old female receiving intravenous ceftriaxone and metronidazole for liver abscess had purpuric macules and papules on her extremities. One week later, she had generalized edema and skin rash with bullae and was diagnosed with concurrent linear IgA dermatosis and IgA nephropathy. After steroid treatment, the skin lesion subsided within two weeks, and kidney function slowly returned to normal. As both diseases occurred after a common possible cause, we predict their pathogeneses are associated.

  5. Clinical differential diagnosis value of detecting serum underglycosylated IgA1 in IgA nephropathy%血清低半乳糖化IgA1测定对鉴别IgA肾病的临床价值

    Institute of Scientific and Technical Information of China (English)

    邱强; 列才华; 曹翠明; 谢院生; 陈香美

    2008-01-01

    目的 确定血清低半乳糖化IgA1对鉴别诊断IgA肾病的临床价值.方法 以原发性肾小球疾病患者91例为研究对象,接受肾活检并留取血清;以健康体检者20例血清作为对照.血清标本先用装有耦联蚕豆凝集素的微球进行微量离心柱法分离并洗脱,获得低半乳糖化IgA1.再以凝集素HAA(Helix aspersa)用ELISA法定量检测异常糖基化IgA1(HAA-IgA1).分析血清低半乳糖IgA1升高在鉴别诊断IgA肾病方面的临床价值.结果 48例IgA肾病患者HAA-IgA1水平[(83.7±41.0)U]高于健康对照组[(52.6±22.9)U]及43例其他原发性肾小球疾病患者组[(49.2±27.3)U](均P<0.01).而该43例中,非IgA系膜增殖性肾炎患者22例(51%)的HAA-IgA1水平[(47.6±21.5)U]亦显著低于IgA肾病患者.以肾穿刺病理诊断为金标准,所绘制ROC曲线面积为0.797,面积的标准误为0.047(P<0.01);鉴别诊断IgA肾病的灵敏度为72.9%,特异度为72.1%,准确度为72.5%.结论 应用微量离心柱法联合ELISA法检测IgA肾病患者血清低半乳糖IgA1对于鉴别诊断IgA肾病具有一定临床价值.%Objective To evaluate the clinical value of detecting serum underglycosylated IgA1 in diagnosis and differentiation of lgA nephropathy (IgAN). Methods Serum underglycosylated IgA1 was isolated by microspincolumn coupled with vicia villosa lectin (VVL) from 48 cases with IgAN and 43 cases with other primary glomemlonephritis. All the patients were diagnosed by renal biopsy. Sera from 20 healthy persons were used as control group. After isolation, the eluant with rich underglycosylated lgAl was detected by incubation with biotin- labeled horseradish peroxidase (HRP) and Helix aspersa (HAA, recognizing N-acetylgalactosamine specifically)in enzyme-linked immunosorbent assay (ELISA). The sensitivity and specificity of diagnosis and differentiation of IgAN with elevated serum underglycosylated IgA1 were analyzed. Results The level of serum underglycosylated IgA1 in IgAN patients [(83

  6. Clinical and pathological analysis of IgA nephropathy with acute kidney injury%原发性IgA肾病患儿伴发急性肾损伤19例临床分析

    Institute of Scientific and Technical Information of China (English)

    陈敏广; 叶晓华; 梁海燕; 杨青

    2016-01-01

    Objective To investigate the incidence,etiology,clinico-pathological characteristics and prognosis in primary IgA nephropathy (IgAN) children with acute kidney injury (AKI).Method Retrospective analysis of the clinical and pathological manifestations and follow-up results of 19 Chlidren,who were associated with AKI in 196 cases of children with IgA nephropathy treated in our department from January,1996 to Jun,2012 was performed.Result (1) The 19 cases associated with AKI accounted for 9.7% of all 196 Chlidren with IgAN.Within the 19 cases,there were gross hematuria in 17 cases,massive proteinuria in 16 cases,hypoalbuminemia in 10 cases,edema in 10 cases and hypertension in one case.The peak serum creatinine was from 94.5 μmol/ L to 282 μmol/L.(2) Histological changes:with the formation of crescent in 10 cases,diffuse endocapillary proliferation in 5 cases,15 cases had renal tubular injury,10 cases had red blood cell and protein cast,1 case with acute interstitial nephritis.(3) The cause of IgA nephropathy with AKI:13 patients had severe glomerular damage,including crescentic glomerulonephritis and diffuse endocapillary proliferation;1 case was complicated with acute interstitial nephritis after being treated with antibiotics,2 patients had decreased glomerular filtration rate because of taking benazepril or oral indomethacin,1 case with renal tubular injury induced by gross hematuria,and the other two cases the reason was not clear.(4) Multivariate Logistic regression analysis showed that massive proteinuria was independent risk factor of IgAN in children with AKI (OR =27.370,95% confidence interval was 3.151-237.740,P < 0.01).(5) None of the patients were on dialysis,steroid therapy was used in 13 cases (including 7 cases of methylprednisolone pulse therapy),6 cases were treated with combined cyclophosphamide treatment.Except 1 cases no significant improvement,the renal functiones of all patients recovered or improved within 1-2 months after treatment

  7. 六味地黄汤对IgA肾病大鼠α-SMA及FN表达的影响%Effects of Liuwei Dihuang Decoction onthe Expression of α-SMA and FN in IgA Nephropathy Rats

    Institute of Scientific and Technical Information of China (English)

    彭亚军; 李旭华; 何泽云; 廖春来; 何雅琴; 胡淑娟

    2015-01-01

    〔Abstract〕 Objective To observe the effects of Liuwei Dihuang Decoction on the expression of α-SMA and fibronectin (FN) in IgA nephropathy rats, and further investigate its possible mechanism. Methods Forty SD rats were equally randomly divided into 4 groups: the normal group, model group, tripterygium glycosides tablets group and Liuwei Dihuang Decoction group. IgAN rat models were established with bovine serum albumin (BSA), lipopolysaccharide (LPS) and carbon tetrachloride (CCl4). The successfully established models were given 4 weeks of continuous perfusion. The proteinuria and urinary red blood cell count were detected every week, respectively. At fourth weekend, all the rats were killed for kidney function test. The α-SMA and FN protein expression of each group were detected by using immunohistochemistry and Western blot, and semi-quantitative analysis was made. Results The urine protein and urine red blood cell countsin each group were compared, the model group was higher significantly than that of normal group (P <0.05), while compared with mode group, Liuwei Dihuang Decoction group was decreased significantly (P<0.05), the difference was statistically significant.The α-SMA and FN expression in model group were increased than that in the normal group, while the expression of Liuwei Dihuang decoction group was decreased comparing with the model group (P<0.05), the difference was statistically significant. Conclusion Liuwei Dihuang Decoction can reduce the urine protein and urine red blood cell count of IgA nephropathy rats and improve the kidney function, which may be related with down-regulation of α-SMA and FN expression.%目的:观察六味地黄汤对IgA肾病大鼠α-平滑肌肌动蛋白(α-smooth muscle actin,α-SMA)及纤维连接蛋白(fibronectin, FN)表达的影响,进一步探讨其可能作用机制。方法 SD 大鼠40只随机分为正常对照组、模型组、雷公藤多苷片组,六味地黄汤组。除正常对

  8. Hepatic-associated immunoglobulin-A nephropathy in a child with liver cirrhosis and portal hypertension.

    Science.gov (United States)

    Alghamdi, Sharifa A; Saadah, Omar I; Almatury, Nesreen; Al-Maghrabi, Jaudah

    2012-01-01

    Hepatic-associated immunoglobulin A (IgA) nephropathy is a relatively common condition that occurs in adults with liver cirrhosis and portal hypertension. However, it is rare in children. This condition is characterized by the deposition of IgA in the renal glomeruli. The present report describes a 14-year-old boy with cryptogenic liver cirrhosis and portal hypertension who presented with hematuria and proteinuria associated with histological changes of IgA nephropathy.

  9. Hepatic-associated immunoglobulin-A nephropathy in a child with liver cirrhosis and portal hypertension

    Directory of Open Access Journals (Sweden)

    Sharifa A Alghamdi

    2012-01-01

    Full Text Available Hepatic-associated immunoglobulin A (IgA nephropathy is a relatively common condition that occurs in adults with liver cirrhosis and portal hypertension. However, it is rare in children. This condition is characterized by the deposition of IgA in the renal glomeruli. The present report describes a 14-year-old boy with cryptogenic liver cirrhosis and portal hypertension who presented with hematuria and proteinuria associated with histological changes of IgA nephropathy.

  10. Idiopathic erythrocytosis in IgA nephropathy

    Science.gov (United States)

    Mahesh, E.; Madhyastha, P. R.; Kalashetty, M.; Gurudev, K. C.; Bande, S.; John, M. M.

    2017-01-01

    We report a case of idiopathic erythrocytosis in a 31-year-old male who was incidentally detected to have hypertension during his preemployment checkup. Urine routine showed proteinuria and hematuria. Biochemical parameters revealed raised serum creatinine, and histological findings of the renal biopsy showed IgAN.

  11. 不同性别原发性IgA肾病患者的临床与病理特点研究%Study of the Clinical and Pathological Features of Patients with Primary IgA Nephropathy of Different Genders

    Institute of Scientific and Technical Information of China (English)

    吴小群; 廖学渊

    2013-01-01

    目的 分析不同性别原发性IgA肾病(IgAN)患者的临床、病理特点,了解性别与该病临床、病理特点的关系.方法 选择2009年1月至2011年12月梅州市人民医院经肾穿刺活检确诊的原发性IgAN患者158例的临床表现、检测指标及病理分级数据进行统计分析.结果 临床特点中,仅水肿在性别上差异有统计学意义(P<0.05);临床指标中,收缩压、舒张压、血尿酸、三酰甘油、白蛋白及补体C3在性别上分析差异均有统计学意义(P<0.05);病理分级中,男性组与女性组比较,差异无统计学意义(P>0.05).结论 性别对IgAN的临床表现及病理分级影响不大,但是临床指标水平在性别上有统计学意义,应加强男性相关指标监测,控制病情.%Objective To analyze the clinical and pathological features of primary IgA nephropathy patients of different genders,find out the relationship between gender and clinical and pathological features of the disease. Methods The clinical manifestations,indicators and pathological classification of 158 IgA nephropathy patients confirmed by renal biopsy were collected for statistical analysis. Results As for the clinical manifestations, there was significant difference in edema between different genders( P 0. 05 ). Conclusion The clinical manifestations and pathological grading of IgA nephropathy are affected less by the gender,but there are statistically significant difference in the clinical indicators. We should strengthen the monitoring of the related indicators of the male patients to control the disease.

  12. The recent research of pathogenesis of IgA nephropathy%IgA 肾病的遗传易感性和黏膜免疫应答异常

    Institute of Scientific and Technical Information of China (English)

    王金泉

    2016-01-01

    众所周知,遗传风险因素和黏膜免疫异常在IgA肾病致病机制中起重要作用。近年来,系列研究已发现新的IgA肾病易感遗传位点,以及遗传风险因素和IgA肾病各方面,包括起病、临床表现、组织病理学、对治疗反应和预后之间的新关系。与此同时,肠道黏膜免疫异常在IgA肾病致病中的作用重新引起了人们的关注。肠道免疫耐受缺陷会削弱机体对微生物肠道屏障功能、增加食物抗原和细菌毒素的吸收、激活黏膜相关淋巴组织导致亚临床肠道炎症。遗传致病机制的进一步阐明和“肠道-肾关系”学说赋予IgA肾病新的治疗理念,如治疗亚临床肠道炎症或调控肠道微生物菌群,也利于进一步探索干预IgA的新靶目标和更加个体化的治疗。%It is known that genetic risk factors and mucosal immunity play vary important role in the pathogenesis of IgA ne-phropathy ( IgAN) .Recent years, new susceptibility loci of IgAN and relationships among genetic risk factors and each field of IgAN, which includes onset, clinical manifestation, histopathology, response to treatment and prognosis, have been found.Meanwhile, a re-surgence of interest has addressed the role of intestinal immunity facing dietary components, like gluten or the complex intestinal flora, the microbiota.A defective immune tolerance might favor an abnormal response to microbiota with alterations of the intestinal barrier, including increased alimentary antigens and bacterial toxins absorption, triggering mucosal-associated lymphoid tissue ( MALT) activa-tion and subclinical intestinal inflammation.The genetic pathogenesis and the new hypothesis for a strong intestine-kidney connection in IgAN are tempting, because they offer new treatment options, such as targeted to subclinical intestinal inflammation or microbiota modi-fications, and favor for further exploring new targets of IgAN intervention.

  13. Location and distribution of bone marrow mesenchymal stem cells in the kidney of IgA nephropathy rats%骨髓间充质干细胞在IgA肾病大鼠肾脏中的定位与分布

    Institute of Scientific and Technical Information of China (English)

    彭伟; 刘郑荣

    2011-01-01

    背景:由于骨髓间充质干细胞缺乏特异性表面抗原,常根据其贴壁生长、成骨成脂分化等生物学特点来鉴定.目的:观察骨髓间充质干细胞在IgA肾病大鼠肾脏中的定位与分布情况.方法:以牛血清白蛋白+葡萄球菌肠毒素B+皮下注射四氯化碳的改良法建立SD大鼠IgA肾病模型.造模成功分别经尾静脉移植大鼠骨髓间充质干细胞或生理盐水,并设立不造模的正常对照组.移植后1,4周观察各组大鼠的尿蛋白、肾功能、肾脏病理变化、IgA荧光沉积变化,并通过免疫组织化学观察BrdU标记的骨髓间充质干细胞在肾组织中的分布情况.结果与结论:移植后1周,骨髓间充质干细胞组尿蛋白、血肌酐明显低于生理盐水组;移植后4周,骨髓间充质干细胞组肾脏病理变化、IgA荧光沉积与生理盐水组比较差异有显著性意义.提示骨髓间充质干细胞输注可促进大鼠IgA肾病的修复,并可定位于肾小球系膜区及少数肾小管间质区,但随时间延长,BrdU标记的髓间充质干细胞在肾组织中分布却逐渐减少.%BACKGROUND: Bone marrow mesenchymal stem cells (BMSCs) without specific surface antigens are often identified according to adhesive growth, osteogenic differentiation, adipogenic differentiation and other biological characteristics. OBJECTIVE: to observe the location and distribution of BMSCs in IgA nephropathy rats.METHODS: Sprague-Dawley rats were randomly divided into three groups: BMSCs group, saline group and control group. IgA nephropathy model was established by the improving method with bovine serum albumin+staphylococcal enterotoxin B+subcutaneous injection of carbon tetrachloride in the former two groups. At the 1st and 4th weeks after BMSCs injection, the changes of urine protein, renal function, histopathology and IgA immunofluorescence were observed. BrdU-labeled BMSCs were detected by immunohistochemistry to observe the disposition in the kidney

  14. Light chain immunofluorescence in various nephropathies

    Directory of Open Access Journals (Sweden)

    Megha S Uppin

    2011-01-01

    Full Text Available Context: Light chain immunofluoresence (IF in renal biopsy is routinely used in the diagnosis of light chain deposition disease (LCDD, amyloidosis and cast nephropathy. Light chain predominance has also been reported in certain glomerulopathies like IgA nephropathy. However, pathogenesis of this pattern of deposition in various glomerulopathies is uncertain. Aim: To discuss the pathogenesis and utility of light chain IF in nephropathies. Setting and Design: Retrospective study. Materials and Methods: The pattern of light chain IF and light microscopic diagnosis in 306 cases of various nephropathies was reviewed. Direct IF was done in all these cases with commercial fluorescence (Fluoresciene Isothiocynate conjugated polyclonal rabbit anti-human antisera against IgM, IgG, IgA, C3, C1q, kappa and lambda light chains. Results: Light chain deposits were seen in 240 (78.43% cases. In IgA nephropathy, lupus nephritis and post-infectious glomerulonephritis (PIGN, lambda positivity was more as compared to kappa. Light chain deposits in LCDD and membranous nephropathy were more kappa type. The IF pattern in amyloidosis was not consistent. Conclusion: The pathogenesis of light chain predominance in glomerulopathies is not clear and it depends on isoelectric point and size of the immune complex. Light chain IF should be performed routinely in all the renal biopsies.

  15. Membranous nephropathy

    Science.gov (United States)

    ... page: //medlineplus.gov/ency/article/000472.htm Membranous nephropathy To use the sharing features on this page, please enable JavaScript. Membranous nephropathy is a kidney disorder that leads to changes ...

  16. 免疫抑制治疗IgA肾病远期疗效的Meta分析%Meta analysis for the long-term efficacy and safety of immunosuppression on the progression of IgA nephropathy

    Institute of Scientific and Technical Information of China (English)

    田磊; 邵兴华; 谢园园; 王琴; 王玲; 倪兆慧; 牟姗

    2014-01-01

    Objective To access the long-term efficacy and safety of immunosuppression on the progression of IgA nephropathy (IgAN) by Meta analysis.Methods Databases EMBASE,Pubmed,Elsevier Science Direct,Scopus,Web of Science,Google Scholar,Cochrane Library,China National Knowledge Infrastructure,WanFang and VIP Data were retrieved to collect the randomized controlled trials (RCTs) at least 3 years follow-up on immunosuppression for IgAN published before May 2014.The literatures were screened independently by two reviewers according to the inclusion and exclusion criteria,and the methodological quality was assessed.Statistic software Stata 12.0 was used to conduct analysis.Results Nine articles were included in this study with a total of 568 patients.Immurnosuppression could lowered the risk for the progression to ESRD (RR=0.32,95%CI:0.20-0.49,P < 0.01).As far as the efficacy of immunosuppression,subgroup analysis indicated that three studies with more than 7 year follow-up (RR=0.28,95%CI:0.13-0.59,P < 0.01) were similar with 7 studies followed by for less than 7 years (RR=0.34,95% CI:0.19-0.59,P<0.01); six adopted immunosuppressor monotherapy (RR=0.29,95% CI:0.15-0.58,P< 0.01) were similar to two used corticosteroids plus other immunosuppression (RR=0.33,95%CI:0.18-0.59,P < 0.01); There were no significant differences between four studies from Europe (RR=0.27,95%CI:0.14-0.53,P < 0.01) and five from Asia (RR=0.35,95% CI:0.19-0.65,P<0.01).Immunosuppression was associated with an increased risk for adverse events (RR=2.33,95% CI:1.33-4.09,P<0.01).Conclusion Immunosuppression for IgAN may reduce long-term risk of progression to ESRD,but increase the risk of adverse events to some extent.%目 的应用Meta分析的方法评价IgA肾病免疫抑制治疗的远期疗效.方法 采用计算机检索EMBASE、Pubmed、Elsevier Science Direct、Scopus、Web of Science、Google Scholar、Cochrane图书馆、中国知网期刊、万方、维普等数据

  17. Clinicopathological analysis of IgA nephropathy patients with mild proteinuria and/or hematuria%少量蛋白尿和(或)血尿IgA肾病临床病理分析

    Institute of Scientific and Technical Information of China (English)

    萨茹拉; 傅辰生; 陈利明; 袁敏; 丁小强; 刘红; 於佳炜; 章晓燕; 蒋素华; 邹建洲; 滕杰; 吉俊; 钟一红

    2010-01-01

    Objective To clarify the relationship between clinical manifestation and pathological features of IgA nephropathy (IgAN) patients with mild proteinuria and/or hematuria.Methods Clinicopathological data from 316 biopsy-proven IgAN cases (proteinuria<1 g/24 h and/or hematuria, and Scr<133 μmol/L) from our hospital between January 1993 and October 2009 were studied retrospectively. The renal histopathology was quantified according to Lee's grading and Katafuchi's semi-quantitative standard, and the risk factors for renal pathological lesions were evaluated using multifactor logistic regression analysis. Results Among these 316 patients, 123 were male and 193 patients were female. The mean age at the time of renal biopsy was (33.10±10.69) years old. Clinical features were found as follows: hematuria with proteinuria was found in 267 patients (84.5%), isolated hematuria in 24 patients (7.6%), and isolated proteinuria in 25 patients (7.9%). 16.5% of patients had hypertension. The percentages of CKD stage Ⅰ, Ⅱ, Ⅲ were 76.9%, 20.9% and 2.2%, respectively. 31.3% of patients presented Lee's grade Ⅲ or more severe.52.8% of patients had various degrees of glomerulosclerosis. Crescent formation was observed in 20.3% of patients. 22.5% of patients showed tubular atrophy;16.8% showed interstitial fibrosis and 24.7% also had renal vascular lesions. The extent of glomerulosclerosis was negatively correlated with eGFR levels, but positively correlated with the amount of proteinuria and mean arterial pressure (MAP) level (P<0.05). The score of tubulointerstitial lesion was positively correlated with the amount of proteinuria and negatively correlated with eGFR and hemoglobin (Hb)level (P<0.05). The degree of renal vascular lesion was also correlated to MAP level positively and eGFR level negatively (P<0.05). Multifactor logistic regression analysis revealed that proteinuria, Scr and Hb at the time of renal biopsy were independent risk factors for severe renal

  18. 尿蛋白标志物对儿童IgA肾病和IgM肾病肾脏病理损伤的预测价值%Urinary protein markers predict the severity of renal histological lesions in children with IgA nephropathy and IgM nephropathy

    Institute of Scientific and Technical Information of China (English)

    王雪芹; 李梦霞; 李晓忠; 朱雪明; 封其华; 李艳红

    2015-01-01

    目的 比较儿童IgM肾病(IgMN)和IgA肾病(IgAN)的临床表现、病理损伤及尿蛋白标志物水平变化,探讨尿蛋白标志物对IgAN和IgMN患儿肾脏病理损伤严重程度的预测价值.方法 选择2002年1月至2014年10月行肾组织活检的74例患儿为研究对象.检测活检当日晨尿中IgG、清蛋白(Alb)、转铁蛋白(TRF)、α1-微球蛋白(α1-MG)、β2-微球蛋白(β2-MG)和N-乙酰-β-葡萄糖苷酶(NAG)水平.采用系膜细胞增生(MC)、肾小球硬化(GS)、肾小管间质损伤(TID)的评分半定量评估肾脏病理损伤严重程度.以多因素Logistic回归分析评估在校正混杂因素后尿液蛋白与肾脏病理损伤的关系,用受试者工作特征曲线下面积(AUC)评价尿液蛋白对肾脏病理损伤严重程度的预测价值.结果 74例患儿中,IgAN 44例,IgMN 30例.IgAN患儿尿oα1-MG和Alb水平高于IgMN患儿;然而,校正年龄后,差异无统计学意义.多因素Logistic回归分析显示,尿液蛋白中TRF与IgAN和IgMN患儿严重MC(>5个系膜细胞/系膜区)显著相关(B=0.010);Alb与严重GS(≥10%肾小球节段性黏连或硬化)显著相关(B=0.001);NAG与严重TID(局灶或弥散性肾小管间质病变)显著相关(B =0.038).尿β2-MG与严重MC、GS、TID无显著相关.尿TRF预测严重MC的AUC值为0.85(P <0.001).尿Alb预测严重GS的AUC值为0.78 (P =0.002).尿NAG预测严重TID的AUC值为0.78(P =0.003).结论 尿蛋白标志物是预测儿童IgAN和IgMN肾脏病理损伤严重程度的指标.其中尿TRF、Alb和NAG具有更好的预测价值.%Objective To compare the clinical manifestations,renal histological lesions,and the levels of urinary protein markers between the children with IgA nephropathy (IgAN) and those with IgM nephropathy (IgMN), and to determine whether urinary protein markers could predict the severity of renal histological lesions in children with IgAN and IgMN.Methods Seventy-four children with renal biopsy-proven IgAN and IgMN from

  19. 伴有牙周炎的IgA肾病患者牙周治疗前后血清TNF-α与IL-8变化分析%Changes in levels of serum TNF-α and IL-8 before and after periodontal therapy for patients of IgA nephropathy with periodontitis

    Institute of Scientific and Technical Information of China (English)

    许志鹏; 宋勇; 孙燕

    2015-01-01

    Objective To examine the levels of serum tumor necrosis factor-α(TNF-α) and interleukin-8 (IL-8) for IgA nephropathy patients with periodontitis before and after periodontal therapy. Methods Fifty pa-tients of IgA nephropathy with chronic periodontitis (study group) and 30 healthy individuals (control group) were in-cluded in this study. The study group was treated by ultrasonic and VECTOR periodontal therapy apparatus, whereas the control group received no special treatment. We measured the periodontal indexes, and serum TNF-αand IL-8 lev-els by enzyme-linked immunosorbent assay (ELISA) at the visit of doctor in the control group as well as before treat-ment and 4 weeks after treatment in the study group. Results After periodontal therapy, the periodontal status was significantly improved, with the levels of periodontal indexes and serum TNF-α and IL-8 significantly decreased. Compared with the control group, the periodontal indexes and the serum TNF-αand IL-8 were still significantly higher. Conclusion Periodontal treatment can not only effectively improve the periodontal lesions and promote the recovery of periodontitis, but also reduce the levels of serum TNF-αand IL-8 and benefit the treatment for IgA nephropathy.%目的:观察伴有慢性牙周炎的IgA肾病患者牙周治疗前后血清肿瘤坏死因子-α(TNF-α)和白细胞介素-8(IL-8)变化。方法选取伴有慢性牙周炎的IgA肾病患者50例(治疗组)和健康者30例(对照组)。对照组不予特殊处理,治疗组采用超声洁牙和VECTOR牙周治疗仪进行牙周治疗,对照组于初诊时,治疗组于治疗前及治疗后4周,分别检测两组受试者的牙周指数和血清TNF-α与IL-8的浓度。血清中TNF-α与IL-8的浓度采用酶联免疫吸附法测定。结果治疗组患者经过牙周治疗后,牙周状况明显好转,血清TNF-α与IL-8浓度与治疗前比较均明显降低,差异均有统计学意义(P<0.05),但是牙周指数、TNF-α和IL-8

  20. Risk factor analysis of blood pressure circadian rhythm abnormality in IgA nephropathy patients with hypertension%126例IgA肾病伴高血压患者血压昼夜节律异常的危险因素分析

    Institute of Scientific and Technical Information of China (English)

    林利容; 张炜炜; 黄坤; 杨聚荣; 张建国; 李开龙; 何娅妮

    2013-01-01

    Objective To investigate the risk factors for blood pressure circadian rhythm abnormality in the IgA nephropathy ( IgAN) patients with hypertension. Methods The study enrolled 126 adult IgA nephropathy patients with hypertension,all of them underwent renal biopsy and ambulatory blood pressure monitoring. Patients were excluded if they were treated by antihypertensive drugs or glucocorti-coids. Baseline patient characteristics and clinical data were collected. Risk factors of blood pressure rhythm abnormality were determined by Spearman correlation analysis and Logistic regression analysis. Results The prevalence of non-dipper blood pressure was 73. 8% for IgAN patients with hypertension. Uric acid,serum creatinine,urine protein/creatinine,nocturnal urinary sodium,urinary chlorine excretion levels were higher in patients with non-dipper hypertension than those with dipper hypertension. Patients with non-dipper hypertension had significantly lower eGFR than those with non-dipper hypertension. Of logistic regression analysis showed that high uric acid,urine protein/creatinine≥ 1.84 mg/mg,24 h urinary sodium > 62. 5 mmol/L,eGFR < 60 ml/min/1. 73 m2 were risk factors for blood pressure circadian rhythm abnormality in the IgA nephropathy patients with hypertension. Conclusion Kidney lesion not only leads to hypertension,also plays a crucial role in the regulation of blood pressure rhythm abnormalities.%目的 分析IrA肾病伴高血压患者血压昼夜节律异常的危险因素.方法 收集成人IrA肾病伴高血压患者126例,均行肾活检及动态血压监测,排除服用降压药物、糖皮质激素者.采用单因素及多因素Logistic回归分析血压昼夜节律异常的危险因素.结果 (1)成人IgA肾病伴高血压患者非勺型血压患病率为73.8%;(2)与勺型血压组比较,非勺型血压患者总胆固醇、血尿酸、血清肌酐、尿蛋白/肌酐、夜间尿钠、尿氯排泄水平均显著增高,肾小球滤过率(eGFR)

  1. Effect of tripterygium glycosides on serum ion and β2-GPI/ox-LDL in patients with IgA nephropathy%雷公藤多苷对IgAN患者血清离子及β2-GPI/ox-LDL影响

    Institute of Scientific and Technical Information of China (English)

    彭健韫; 苏震

    2015-01-01

    目的:探讨雷公藤多苷对IgA肾病(IgA nephropathy,IgAN)患者血清离子及β2糖蛋白I(β2-glycoprotein,β2-GPI)/氧化低密度脂蛋白( oxidized low density lipoprotein ,ox-LDL)的影响。方法收集丽水市人民医院肾内科2014年1月~2015年4月收治的原发性IgAN患者54例,随机分为对照组和实验组,每组27例,对照组患者常规给予盐酸贝那普利片10 mg,1次/天口服;给予双嘧达莫片50 mg,3次/天口服,实验组在对照组基础上给予雷公藤多苷片20 mg,3次/天口服。2组患者均治疗6个月。治疗结束后,对所有患者的血清Ca、P、β2-GPI/ox-LDL水平及肾功能相关指标进行检测。结果与对照组治疗后比较,实验组患者血清Ca水平较高,血清P水平较低(P<0.05);实验组患者的血清β2-GPI/ox-LDL水平较低(P<0.05);实验组患者的血清Scr、BUN水平较低(P<0.05)。结论雷公藤多苷能够显著降低IgAN患者血清P、β2-GPI/ox-LDL、Scr及BUN水平,提高血清Ca水平,改善肾功能。%Objective To analyse effect of tripterygium glycosides on serum ion and β2-GPI/ox-LDL in patients with IgA nephropathy. Methods 54 patients who were diagnosed with primary IgA nephropathy in our hospital were collected.All patients were randomly divided into experimental group and control group, 27 cases in each group.Control group was given conventional Benazepri1 Hydrochloride Tablets 10 mg, 1 times per day orally;given Dipyridamole Tablets 50 mg, 3 times per day orally, patients in experimental group were given tripterygium glycosides 20 mg on the basis of control group treatment orally,3 times per day.All the patients were treated for 6 month.After the treatment, the serum levels of Ca,P,β2-GPI/ox-LDL and renal function related index were detected in all patients.Results Compared with control group post-treatment, the serum level of Ca was higher(P<0.05); and the serum level of P

  2. Acute Cresentric IgA Nephritis in a Patient with Hodgkin's Lymphoma

    Directory of Open Access Journals (Sweden)

    Ebru GÖK OĞUZ

    2014-09-01

    Full Text Available In glomerular diseases, the occurence of lymphoma is mostly observed in the form of both minimal change disease and Hodgkin’s lymphoma. The coocurrence of Membranous nephropathy and membranoproliferative glomerulonephritis are generally associated with non-Hodgkin’s lymphoma. While Ig A nephropathy-lymphoma association is rare, it is generally observed in the form of non- Hodgkin’s lymphoma, and there are also cases proposed the cooccurence of Ig A nephropathy and cutaneous T-cell lymphoma. In this case, it is emphasized that IgA nephropathy presented with cresentric glomerulonephritis should be considered in patients with hodgkin’s lymphoma who have sudden renal disorder.

  3. Study on the Regularity of Prescription for IgA Nephropathy Basing on Text Mining Technique with Chinese Patent Medicine and West Medicine%基于文本挖掘技术的中成药及西药治疗IgA肾病用药规律研究

    Institute of Scientific and Technical Information of China (English)

    蔡峰; 郑光; 郭洪涛; 姜淼; 谭勇; 杨静; 张弛; 张志华; 吕爱平

    2011-01-01

    Objective:To explore the data to reveal meaningful regularity of Chinese patent medicine and west medicine. Methods: Papers from large data sets of IgA nephropathy were used to set the Acess database. And then,these articles were used to data processing by the SQL. After article denoise,the regulation of IgA nephropathy was analyzed about the TCM medicine and the west medicine. Results:Glucoside Tripterygium Total, Huangkui Capsule ,hemostasis tablets,Bailing Capsule were the commonly used Chinese patent medicine. Im-munosuppressive agents, ACEI, glucocorticoids, ARB were of the core west medicine. Glucoside Tripterygium Total, Huangkui Capsule and ACEI,ARB,glucocorticoids were often used in conjunction with Chinese patent medicine and western medicine. Conclusion:The result,which provides an objective basis with text mining technique, not only reflects the actual clinical use,but also expands the scope of clinical medicine for the scientific and rational use of Chinese proprietary medicine and west medicine.%目的 利用文本挖掘技术探索中成药及西药治疗IgA肾病的用药规律.方法在中国生物医学文献数据库(CBM)中采集治疗IgA肾病的相关文献,建立Access数据库,运用SQL对数据进行处理,结合人工降噪后,分析中成药及西药治疗IgA肾病的用药规律.结果雷公藤多苷片、黄葵胶囊、止血片、百令胶囊等依次为治疗IgA肾病的高频中成药;免疫抑制剂、ACEI、糖皮质激素、ARB等依次为治疗IgA肾病的高频西药;雷公藤多苷片、黄葵胶囊和ACEI、ARB、糖皮质激素分别是联用时常用的中成药和西药.结论文本挖掘能够分析中成药及西药治疗IgA肾病的用药规律,获得的结果不仅反映了临床用药实际,而且拓展了临床用药的范畴与思路,为科学合理地使用中成药及西药提供了客观依据.

  4. Radiogenic nephropathy; Radiogene Nephropathie

    Energy Technology Data Exchange (ETDEWEB)

    Gotthardt, M. [Univ. Medisch Centrum St Radboud, Nijmegen (Netherlands). Nucleaire Geneeskunde

    2010-07-01

    Patient-individual dosimetric analyses are a useful tool in external beam radiotherapy (EBR) to protect patients from side effects such as radiogenic nephropathy. At this point in time, individual dosimetry is not used as a standard in patient treated with radiolabelled antibody fragments or polypeptides. The reasons are a number of problems, which make patient dosimetry more challenging than in EBR. While in EBR, the dose is distributed evenly in the organ and the organ volume can exactly be determined, in internal radiotherapy the tracer is not evenly distributed within the organ leading to a non-uniform dose distribution. In addition, the dose rate of the most commonly used radionuclides is lower than in EBR and the range of their radiation differ, so that the radiobiological effects are differing considerably in comparison to EBR. Conclusion: More complex models have to be used for clinical kidney dosimetry in internal radiotherapy. In this paper, we give a concise overview of the reasons for accumulation of radiotracers in the kidney, the most recent developments in kidney dosimetry, and approaches to reduce the kidney uptake of radiotracers in order to avoid radiogenic nephropathy. (orig.)

  5. Effects of C1 GALT1 and Cosmc mRNA Expression of Liuwei Dihuang Decoction on IgA Nephropathy Rat Peripheral Blood%六味地黄汤对IgA肾病大鼠外周血C1 GALT1及Cosmc mRNA表达影响研究∗

    Institute of Scientific and Technical Information of China (English)

    彭亚军; 李旭华; 何泽云; 廖春来; 何雅琴; 胡淑娟

    2016-01-01

    目的:观察六味地黄汤对IgA肾病大鼠外周血C1GALT1及Cosmc mRNA表达,探讨其对IgA糖基化的影响。方法:SD大鼠40只随机分为正常对照组,模型组,雷公藤多苷片组,六味地黄汤组。除正常对照组外均采用运用牛血清白蛋白+脂多糖+四氯化碳方法建立实验性IgA肾病模型。造模成功后连续灌胃4周。将各组大鼠外周血分离的PBMC悬液,采用实时荧光定量PCR检测C1GALT1及Cosmc mRNA的表达,ELISA法测定外周血中IgA含量,凝集素亲和 ELISA 法检测IgA1异常糖基化程度,并进行定量分析。结果:各组C1GALT1mRNA表达量并差异无统计学意义(P>0.05);在各组Cosmc mRNA表达中,六味地黄汤组及雷公藤多苷片组表达均较模型组升高(P0. 05); In each group Cosmc mRNA expression, Liuwei Dihuang decoction group and tripterygium glycosides tablets more expression were higher in the model group (P<0. 05), but express Liuwei Dihuang decoction group lower than tripterygium glycosides tablets group (P<0. 05). The IgA content and the degree of abnormal glycosylation of the rats in each group were compared. The Liuwei Dihuang decoction group were significantly lower than the model group, the difference was statistically significant (P<0. 05). Conclusion:Liuwei Dihuang decoction can regulate the expression of Cosmc and mRNA, in-crease the activity of C1GALT1, improve the IgA1 abnorant glycosylation, which down regulate theIgA1 content, to avoid further dep-osition in kidney, and delay the progression of IgA nephropathy.

  6. 血清IgA及IgA/C3 比值在IgA肾病诊断中的价值%Value of serum IgA,IgA/C3 ratio in diagnosis of IgA nephropathy

    Institute of Scientific and Technical Information of China (English)

    张郁苒; 于青; 姚建

    2009-01-01

    @@ IgA肾病(IgA nephropathy,IgAN)是我国最常见的原发性肾小球肾炎,但至今其确切发病机制仍不明确.免疫反应和补体系统可能在IgAN的发生、发展中起重要作用,9%~70%的IgAN患者血清IgA升高[1],其血清C3水平是否下降仍有争议.

  7. Minimal Change Disease and IgA Deposition: Separate Entities or Common Pathophysiology?

    Directory of Open Access Journals (Sweden)

    Brandon S. Oberweis

    2013-01-01

    Full Text Available Introduction. Minimal Change Disease (MCD is the most common cause of nephrotic syndrome in children, while IgA nephropathy is the most common cause of glomerulonephritis worldwide. MCD is responsive to glucocorticoids, while the role of steroids in IgA nephropathy remains unclear. We describe a case of two distinct clinical and pathological findings, raising the question of whether MCD and IgA nephropathy are separate entities or if there is a common pathophysiology. Case Report. A 19-year old man with no medical history presented to the Emergency Department with a 20-day history of anasarca and frothy urine, BUN 68 mg/dL, Cr 2.3 mg/dL, urinalysis 3+ RBCs, 3+ protein, and urine protein : creatinine ratio 6.4. Renal biopsy revealed hypertrophic podocytes on light microscopy, podocyte foot process effacement on electron microscopy, and immunofluorescent mesangial staining for IgA. The patient was started on prednisone and exhibited dramatic improvement. Discussion. MCD typically has an overwhelming improvement with glucocorticoids, while the resolution of IgA nephropathy is rare. Our patient presented with MCD with the uncharacteristic finding of hematuria. Given the improvement with glucocorticoids, we raise the question of whether there is a shared pathophysiologic component of these two distinct clinical diseases that represents a clinical variant.

  8. Minimal change disease and IgA deposition: separate entities or common pathophysiology?

    Science.gov (United States)

    Oberweis, Brandon S; Mattoo, Aditya; Wu, Ming; Goldfarb, David S

    2013-01-01

    Introduction. Minimal Change Disease (MCD) is the most common cause of nephrotic syndrome in children, while IgA nephropathy is the most common cause of glomerulonephritis worldwide. MCD is responsive to glucocorticoids, while the role of steroids in IgA nephropathy remains unclear. We describe a case of two distinct clinical and pathological findings, raising the question of whether MCD and IgA nephropathy are separate entities or if there is a common pathophysiology. Case Report. A 19-year old man with no medical history presented to the Emergency Department with a 20-day history of anasarca and frothy urine, BUN 68 mg/dL, Cr 2.3 mg/dL, urinalysis 3+ RBCs, 3+ protein, and urine protein : creatinine ratio 6.4. Renal biopsy revealed hypertrophic podocytes on light microscopy, podocyte foot process effacement on electron microscopy, and immunofluorescent mesangial staining for IgA. The patient was started on prednisone and exhibited dramatic improvement. Discussion. MCD typically has an overwhelming improvement with glucocorticoids, while the resolution of IgA nephropathy is rare. Our patient presented with MCD with the uncharacteristic finding of hematuria. Given the improvement with glucocorticoids, we raise the question of whether there is a shared pathophysiologic component of these two distinct clinical diseases that represents a clinical variant.

  9. 替米沙坦对大鼠IgA肾病模型肾小管间质损伤及PPARγ表达的影响%Effect of telmisartan on tubulointerstital injury and expression of PPARγin rat renal tissue of IgA nephropathy model

    Institute of Scientific and Technical Information of China (English)

    邢丽; 柏林; 禹程远; 解汝娟

    2010-01-01

    Objective To observe the effect of telmisartan on the expression of PPARγin rat renal tissue of IgA nephropathy model and clarify the possible mechanism of telmisartan in tubulointerstitial injury.Methods The experimental rat model with IgA nephropathy was induced by bovine serum albumin ( BSA),lipopolysaccharide(LPS)and carbon tetrachloride(CCl4). Forty male SD rats were randomly divided into control group, IgA model group, rosiglitazone group, telmisartan group and losartan group. At preadministration, Weeks 4, 8 and 10, the quantity of 24-hour proteinuria was measured. The morphologic changes of renal tissues were evaluated by electron microscope. Immunohistochemistry was used to observe the expressions of PPARγ, TGF-β1 and α-smooth muscle actin (α-SMA) in different groups and RT-PCR to detect the expressions of PPARγ, TGF-β1 and monocyte chemoattractant protein-1 ( MCP-1 ) in different groups. Results Compared with control group, 24-hour proteinuria(mg) increased markedly in IgA model group( 14. 14 ± 1.99 vs 1.59 ±0. 18), but rosiglitazone group(2. 35 ±0. 33), telmisartan group( 1.88 ±0. 09)and losartan group( 2. 82 ± 0. 34 ) was much lower and telmisartan had the most significant effect (all P <0. 05). Compared with control group, there were varying degrees of mesangial proliferation and infiltration of inflammatory cell in IgA model group(3. 10 ±0. 18). The tubulointerstitial injury was notably alleviated in rosiglitazone group( 1.97 ±0. 23), telmisartan group( 1.57 ±0. 14) and losartan group (2. 15 ±0. 22) while telmisartan had the most significant effect (all P < 0.01 =. With immunohistochemistry and reverse transcription-polymerase chain reaction (RT-PCR), PPARγ, TGF-β1, α-SMA and MCP-1 had minimal expression on tubule and interstitium in normal group. But there was a high expression in model group. There was no difference between losartan and model groups. There was a lowered expression in rosiglitazone and telmisartan groups

  10. Transferrin receptor and Fc α/μ receptor may not be the major IgA1 receptor on human mesangial cells

    Institute of Scientific and Technical Information of China (English)

    HU Rui-hai; ZHANG Ying; ZHAO Ming-hui

    2005-01-01

    @@ IgA nephropathy (IgAN) is the most common form of primary glomerulonephritis.1 The histopathology of IgAN is characterized by abundance of mesangial matrix and proliferation of mesangial cells. IgA1 deposition in the mesangium plays an important role in the inflammatory process in this disease.

  11. Diabetic nephropathy

    Directory of Open Access Journals (Sweden)

    Zelmanovitz Themis

    2009-09-01

    Full Text Available Abstract Diabetic nephropathy is the leading cause of chronic renal disease and a major cause of cardiovascular mortality. Diabetic nephropathy has been categorized into stages: microalbuminuria and macroalbuminuria. The cut-off values of micro- and macroalbuminuria are arbitrary and their values have been questioned. Subjects in the upper-normal range of albuminuria seem to be at high risk of progression to micro- or macroalbuminuria and they also had a higher blood pressure than normoalbuminuric subjects in the lower normoalbuminuria range. Diabetic nephropathy screening is made by measuring albumin in spot urine. If abnormal, it should be confirmed in two out three samples collected in a three to six-months interval. Additionally, it is recommended that glomerular filtration rate be routinely estimated for appropriate screening of nephropathy, because some patients present a decreased glomerular filtration rate when urine albumin values are in the normal range. The two main risk factors for diabetic nephropathy are hyperglycemia and arterial hypertension, but the genetic susceptibility in both type 1 and type 2 diabetes is of great importance. Other risk factors are smoking, dyslipidemia, proteinuria, glomerular hyperfiltration and dietary factors. Nephropathy is pathologically characterized in individuals with type 1 diabetes by thickening of glomerular and tubular basal membranes, with progressive mesangial expansion (diffuse or nodular leading to progressive reduction of glomerular filtration surface. Concurrent interstitial morphological alterations and hyalinization of afferent and efferent glomerular arterioles also occur. Podocytes abnormalities also appear to be involved in the glomerulosclerosis process. In patients with type 2 diabetes, renal lesions are heterogeneous and more complex than in individuals with type 1 diabetes. Treatment of diabetic nephropathy is based on a multiple risk factor approach, and the goal is retarding the

  12. Suppression subtractive hybridization to obtain differential genes associated kidney yang defficiency of IgA nephropathy%运用消减杂交技术构建IgA肾病肾阳虚证的相关基因文库

    Institute of Scientific and Technical Information of China (English)

    李玉萍; 罗仁; 孙晓敏; 聂晓莉; 赵晓山; 杨海文

    2011-01-01

    Objective To construct cDNA substractive library of kidney yang defficiency of IgA nephropathy ( IgAN)by suppression subtractive hybridization ( SSH).Methods The research related genes of kidney yang defficiency syndrome of IgAN.Microamount RNA was amplified.SSH and gene cloning were used to obtain differential genes associated kidney yang defficiency of IgAN.Result A Total of 537 clones were obtained.276 from the forward subtractive library and 261 from the reverse subtractive library.Conclusion The cDNA subtractive library is successfully constructed and it may provide a solid foundation for screening and cloning the genes of kidney yang defficiency.%目的 采用抑制性消减杂交技术构建汉族人IgA肾病肾阳虚证cDNA消减文库.方法 以辨病与辨证相结合,以IgA肾病肾阳虚证入手,应用RNA微量扩增、抑制性消减杂交、基因克隆等技术和方法,构建IgA肾病肾阳虚证消减文库.结果 该研究成功地构建了IgA肾病肾阳虚证的cDNA消减文库,其中正向消减文库获得276个阳性克隆,反向消减文库获得261个阳性克隆.结论 该研究初步构建了IgA肾病肾阳虚证的cDNA消减文库,为进一步筛选和克隆肾阳虚证的相关基因奠定了基础.

  13. Effects of leflunomide on renal pathology and expression of MCP-1 in renal tissue in rat experimental IgA nephropathy model%Leflunomide对实验性IgA肾病大鼠肾脏病理及MCP-1表达的影响

    Institute of Scientific and Technical Information of China (English)

    汤颖; 娄探奇; 成彩联; 陈珠江; 张俊

    2006-01-01

    目的观察leflunomide对实验性IgA肾病(IgA nephropathy IgA N)大鼠肾脏病理及肾组织单核细胞趋化因子(monocyte chemoattractive peptide1,MCP-1)表达的影响,了解其作用机制.方法建立IgAN大鼠模型,随机分成leflunomide组,强的松组,模型对照组,并同时设立正常对照组.行免疫荧光、光镜检查,并用免疫组化和RT-PCR方法分别检测肾组织MCP-1蛋白和基因水平的表达.结果与模型对照组相比,leflunomide组免疫复合物在肾脏的沉积明显减少,系膜区基质增生程度显著减轻(P<0.01);Leflunomide在基因和蛋白水平均能够有效抑制MCP-1在肾组织的表达(P<0.05).结论Leflunomide能够减少免疫复合物在肾脏的沉积,减轻系膜区基质增生,并且下调MCP-1在肾脏的表达,减少局部炎症反应,减轻肾脏损害,保护肾脏.

  14. 激素联合血管紧张素转换酶抑制剂治疗中度蛋白尿IgA肾病的随机对照研究%Corticosteroids Plus Angiotensin Converting Enzyme Inhibitor in Treating IgA Nephropathy: A Randomized Control Trial

    Institute of Scientific and Technical Information of China (English)

    熊子波; 梁伟; 王勇强; 侯霜; 张帆; 贺丽娟; 罗琼

    2012-01-01

    目的 探讨激素与血管紧张素转换酶抑制剂(ACEI)联合治疗中度蛋白尿IgA肾病患者的疗效及其影响因素.方法 选择2003年5月-2009年1月在北京大学深圳医院住院的102例IgA肾病患者,均满足血肌酐<133 μmol/L,尿蛋白为1.0 ~3.5 g/24 h.按照随机数字表分为试验组和对照组.对照组(50例)给予ACEI类药物治疗(洛汀新10 mg/d),试验组(52例)在此基础上口服泼尼松0.5 mg/kg,隔日给药,治疗12 个月,并在治疗的第1、3、5个月初分别给予甲基泼尼松龙0.5 g/d,冲击3 d.对肾脏病理改变进行分级并对各种病变进行半定量分析.结果 在0、2、4、6、8、10、12个月时两组患者平均24 h尿蛋白水平分别为:试验组(2.07±0.88)g/24 h、(0.82±0.66)g/24 h、(0.63±0.53)g/24 h、(0.56±0.51)g/24 h、(0.58±0.47)g/24 h、(0.57±0.48)g/24 h及(0.64±0.54) g/24 h;对照组(1.88±0.67)g/24 h、(1.67±0.75)g/24 h、(1.55±0.81)g/24 h、(1.24±0.77)g/24 h、(1.44±0.92)g/24 h、(1.31±0.79)g/24 h及(1.28±0.85) g/24 h,两组在治疗2个月后尿蛋白水平间差异有统计学意义(P<0.01);而试验组和对照组的血肌酐水平在治疗期间均稳定,且两组间差异无统计学意义(P>0.05).多因素分析显示激素联合ACEI治疗的疗效与肾小球硬化积分及肾小管间质积分呈负相关.结论 激素联合ACEI治疗中度蛋白尿IgA肾病,能更有效降低尿蛋白,稳定肾功能.其中影响疗效的主要因素为肾小球硬化率及肾小管间质病变程度.%Objective To evaluate the efficacy of the corticosteroids plus angiotensin converting enzyme inhibitor ( ACEI ) in treating IgA nephropathy and its influencing factors. Methods Totally 102 inpatients with IgA nephropathy hospitalized in Peking University Shenzhen Hospital were randomly enrolled. All of them met the inclusion criteria: serum creatinine a-bove 133 |j,mol/L and urine proteins 1.0 - 3. 5 g/24 h. These patients were divided into two groups: control group

  15. [Analgesic nephropathy].

    Science.gov (United States)

    Pintér, I; Nagy, J

    1998-11-22

    Analgesic nephropathy is a slowly progressive disease caused by the chronic abuse of analgesic mixtures containing two analgesic components combined with potentially addictive substances (coffeine and/or codeine). Pathologically, the nephropathy is characterized by renal papillary necrosis with calcification and chronic interstitial nephritis sometimes in association with transitional-cell carcinoma of the uroepithelium. In the early stage, the clinical characteristics are polyuria, sterile pyuria, sometimes renal colic and haematuria. With further progression of the disease, there are the nonspecific symptoms of advanced renal failure. The incidence of classic analgesic nephropathy among Hungarian patients on chronic renal replacement therapy has proven. There is an urgent need for the estimation of analgesic nephropathy among patients with chronic renal disease and among patients with chronic pain presumably regularly taking analgesics in Hungary. As long as analgesic mixtures containing phenacetin or paracetamol and/or nonsteroidal antiinflammatory drugs and addictive substances are available "over-the-counter", analgesic nephropathy will continue to be a problem also in our country.

  16. 15.4.Interstitial nephritis and interstitial nephropathy

    Institute of Scientific and Technical Information of China (English)

    1992-01-01

    920149 The relationship between renal tis-sue subsets of lymphocytes and clinicalfeatures in IgA nephropathy.LIU Zhihong (刘志红),et al.Dept Nephrol,Jinling Hosp,Nanjing(210002).Chin J Nephrol 1991; 7 (4): 230.Amount of CD4+,CD8+ and B cells in the

  17. Risk factors analysis of prognosis of microalbuminuria IgA nephropathy patients with decreased ;serum C3 level%表现为微量白蛋白尿IgA肾病伴低C3血症患者的预后危险因素分析

    Institute of Scientific and Technical Information of China (English)

    郭宗运; 李霞; 周世菊; 赵东; 刘金彦; 吴玉梅

    2016-01-01

    proliferation (RR = 0.872, 95% CI: 0.491- 1.275, P =0.042), crescentic (RR = 1.528, 95% CI: 1.073- 2.385, P = 0.009) affected prognosis of microalbuminuria IgA nephropathy as the independent risk factors. Conclusions The clinical and pathological features in patients of microalbuminuria IgA nephropathy with decreased serum C3 level is more severe, and the prognosis is poor. The patients should be followed up closely and early intervention treatment and early active control of microalbuminuria should be done at the same time.%目的:对表现为微量白蛋白尿IgA肾病伴低C3血症患者的临床资料以及预后进行分析,探讨低C3血症在微量白蛋白尿IgA肾病中的意义。方法分析肾活检确诊且随访时间大于6个月的135例微量白蛋白尿IgA肾病患者的临床资料,依据血清C3水平,分为低C3组(34例)和正常C3组(101例)。以24 h尿蛋白定量大于1 g或肾穿刺时血清肌酐(SCr)值正常者出现SCr异常或肾穿刺时SCr异常者出现SCr升高1倍以上为随访终点,用Kaplan-Meier法分析肾脏生存率,应用Cox回归模型分析影响预后的相关危险因素。结果随访时间(53.4±21.9)个月,低C3组有27例(79.41%)、正常C3组有32例(31.68%)进入随访终点。通过Kaplan-Meier生存分析显示低C3组肾脏中位生存时间显著短于正常C3组[(46.7±9.1)个月比(68.4±9.9)个月,P=0.014]。应用Cox回归方法分析显示血清Scr异常(RR=1.147,95%CI:1.129~1.395,P=0.008)、低C3血症(RR=1.028,95%CI:0.672~1.495,P=0.039)、尿蛋白定量>1 g/24 h(RR=2.066,95%CI:1.242~3.838,P=0.006)以及肾穿刺时的病理指标Lee分级Ⅲ~Ⅴ级(RR=2.820,95%CI:1.249~5.638,P=0.041)、节段性肾小球硬化或粘连(RR=1.232,95%CI:1.065~1.520,P=0.040)、肾小管萎缩或间质纤维化(RR=2.604,95%CI:1.748~4.104,P=0.037)、毛细血管内细胞增生(RR=0

  18. Causes of reduced expression of Cosmc gene in IgA nephropathy patients%IgA肾病患者Cosmc基因表达低下原因探讨

    Institute of Scientific and Technical Information of China (English)

    秦伟; 钟翔; 张颖娟; 谭淳予; 杨立川; 樊均明

    2008-01-01

    目的 探讨IgA肾病(IgAN)患者核心1β1,3半乳糖基转移酶伴侣蛋白(Cosmc)表达低下的原因,到底是基因突变还是外源性抑制.方法 40例IgAN患者、16例非IgAN肾小球肾炎患者、21例健康对照被纳入本研究.直接测序方法测定Cosmc基因外显子区域的核酸序列.免疫磁珠分离IgAN患者、非IgAN肾小球.肾炎患者和健康对照者外周血B淋巴细胞,并分别在RPMI-1640及RPMI-1640+脂多糖(LPS)中培养72 h.实时荧光RT-PCR方法测定Cosmc基因的mRNA表达水平.结果 (1)IgAN患者Cosmc基因外显子区域没有共同突变位点,仅在2例患者中发现2处错义突变和2处无意义突变,而且这些突变并不影响患者cosmc基因的表达.(2)IgAN患者外周血B淋巴细胞的基础Cosmc基因表达显著降低,为健康对照组的31%.(3)经RPMI-1640培养后,IgAN患者Cosmc基因的表达水平升高至基础值的219%.RPMI-1640+LPS组Cosmc基因的h调受到明显抑制.(4)健康对照Cosmc基因的表达不受RPMI-1640和LPS的影响.结论 IgAN患者外周血B淋巴细胞Cosmc基因表达低下可能与基因突变无关,而是受外源性抑制囚素影响所致.%Objective To clarify whether the Cosmc gene down-regnhtion in lgA nephropathy (IgAN) patients is resulted from genetic disorders or external suppressions. Methods Forty IgAN patients, 16 non-IgAN glomerulonephritis patients and 21 healthy controls were enrolled in the study. Genomie DNA was extracted and then Cosmc gene was amplified and sequenced. Peripheral B lymphoeytes were isolated and cultured with RPMI-1640 alone or plus lipopolysaecharide (LPS). The Cosmc mRNA expression levels at baseline, after RPMI-1640 culture or RPMI-1640+LPS treatment were measured respectively by real-time RT-PCR. Results (1) Only 2 missense mutations and 2 silent mutations were detected in coding frame region of Cosine gene in 2 IgAN patients. (2) The baseline Cosmc gene expression level was significantly lower in IgAN patients (31% of

  19. 内蒙地区 IgA 肾病患者血清IgA1 分子N-乙酰半乳糖胺异常与其病理表现关系探讨%Discussion of the Relationship between Serum IgA1 Molecular N-Acetlygalactosamine Abnormality and its Pathological Manifestations in IgA Nephropathy in Inner Mongolia Region

    Institute of Scientific and Technical Information of China (English)

    徐秀芝; 王新; 肖秀清; 王巧莲

    2009-01-01

    目的 探讨内蒙地区蒙古族与汉族人群IgA肾病患者血清IgA1分子N-乙酰半乳糖胺异常程度与肾脏病理表现的关系.方法将入选的60 例IgA肾病患者,用ELISA法测定蚕豆凝集素(VVL)与IgA1分子铰链区上N-乙酰半乳糖胺(GalNAc)的总结合力,即VVL总结合力,同时计算VVL与IgA1的结合力.将20例原发性肾病综合征微小病变与膜性肾病患者设为对照组,根据IgA1与VVL结合力的均值加减2个标准差为界,将IgA肾病组分为正常糖基化与低糖基化,同时进行常规肾活检.结果 IgA1肾病组VVL总结合力、VVL与IgA1结合力情况与对照组比较有显著性差异(P<0.05);蒙古族与汉族IgA1糖基化程度、肾脏病理情况、年龄及血肌酐比较,无显著性差异(P>0.05).结论内蒙地区蒙古族与汉族人群中IgA1低糖基化程度与肾脏病理轻重无明显的相关关系,IgA肾病患者血清IgA1糖基化异常,血清低糖基化程度重者发病年龄较轻但肾小球滤过功能损伤较重.IgA1低糖基化程度尚不能推测肾脏病理轻重,并且在蒙古族与汉族人群中,低糖基化IgA1对IgA肾病的致病作用亦无差别.

  20. 肾小管肝型脂肪酸结合蛋白对小鼠IgA肾病的肾脏保护作用%Protective effects of tubular liver-type fatty acid binding protein on murine IgA nephropathy

    Institute of Scientific and Technical Information of China (English)

    佐楠; 李艳秋; 王力宁; 李子龙; 王均; 冯江敏; 马健飞; 范秋灵; 姚丽

    2011-01-01

    目的 探讨近曲小管肝型脂肪酸结合蛋白(L-FABP)对骨髓移植诱导的小鼠IgAN的肾脏保护作用.方法 通过骨髓移植在肾小管高表达人类L-FABP(hL-FABP)基因的转基因鼠(Tg)和相同背景的野生鼠(WT)上重建IgAN.受体鼠分别在骨髓移植后第6和12周处死,留取肾脏标本.用实时荧光定量PCR方法检测肾脏hL-FABP、纤连蛋白(FN)和单核细胞趋化蛋白1(MCP-1)的mRNA表达;免疫组化法检测hL-FABP和FN的蛋白表达分布;Western印迹法检测hL-FABP、4-羟壬烯醛(4-HNE)、血红素加氧酶1(HO-1)、FN、Ⅳ型胶原的蛋白表达水平;ELISA法检测血清IgA、尿白蛋白和尿hL-FABP水平.结果 骨髓移植在受体转基因鼠(Tg-ddY)和野生鼠(WT-ddY)上均重建了IgAN,血清IgA水平升高伴有系膜区IgA沉积,组间差异无统计学意义.正常Tg鼠的肾小管表达hL-FABP.与正常Tg鼠相比,骨髓移植后第6周,Tg-ddY鼠肾脏hL-FABP的mRNA(1.62±0.32比0.46±0.09,P<0.01)和蛋白(1.74±0.76比1.14±0.31,P<0.01)表达水平显著上调,伴有尿hL-FABP水平(μg/g肌酐)显著升高(59.87±26.75比31.01±14.86,P<0.05).与Tg-ddY鼠相比,WT-ddY鼠在第12周出现明显的白蛋白尿(mg/L)(828±656比82±22,P<0.01)、明显的系膜基质扩张(P<0.01),并在肾小球出现更多的FN及Ⅳ型胶原沉积.Tg-ddY鼠的肾脏HO-1和4-HNE修饰蛋白(均P<0.05)以及MCP-1 mRNA的表达(P<0.01)被显著抑制.结论 肾小管L-FABP可能通过抑制氧化应激和炎性介质的产生减轻肾小球损伤,在IgAN早期发挥了肾脏保护作用.%Objective To investigate the renoprotection of tubular L-FABP in murine IgA nephropathy (IgAN) induced by bone marrow transplantation(BMT). Methods IgAN models were reconstituted by BMT from IgAN-prone mice into mice (Tg) transgenically tubular overexpressing human L-FABP (hL-FABP) and wild type (WT) mice. These recipients were sacrificed at 6 and 12 weeks after BMT and their kidneys were collected. The expressions

  1. Clinical application value of urinary transforming growth factor-beta 1 in predicting IgA nephropathy progress%尿转化生长因子-β1在预测IgA肾病进展中的临床应用价值

    Institute of Scientific and Technical Information of China (English)

    周金金; 徐家云; 彭霞; 邵叶青

    2016-01-01

    Objective Through analyzing relevance of urinary transforming growth factor-beta 1(TGF-β1)and clinicopathological characteristics of IgA nephropathy, study clinical significance of urinary TGF- beta 1 in IgAN pathogenesis and progress.Methods Choose 30 cases primary IgAN patients hospitalized in nephrology department of The First Affiliated Hospital of Henan University of Science and Technology from March 2013 to May 2015; and 5 cases healthy control subjects. Test urine TGF - 1 level by ELISA method, and test other clinical indicators including serum creatinine, urine creatinine, 24h urinary protein quantitative. Result 1 urinary TGF- beta 1/ urinary Cr ratio was higher than normal controls of IgAN patients (P<0.05); 2 urinary TGF- beta 1/urinary Cr level of IgAN patients were positively related with pathological grading, 24h urinary protein, CKD classification.Conclusion Urinary TGF- beta 1 level can be used as early molecular biological indicator of IgAN activity and progress.%目的:通过分析IgA肾病(IgAN)尿转化生长因子β1(TGF-β1)与临床病理的相关性,探讨尿TGF-β1在IgAN发病和进展中的临床意义。方法选择2013年3月至2015年5月间在河南科技大学第一附属医院肾内科住院的原发性IgAN患者30例,健康对照者5例,采用ELISA法检测尿TGF-β1水平,同时测定血、尿肌酐,24h尿蛋白定量等临床指标。结果1、IgAN患者尿TGF-β1/尿Cr比值高于正常对照者(P<0.05);2、IgAN患者尿 TGF-β1/尿Cr水平分别与病理分级、24h尿蛋白定量、CKD分期呈正相关。结论尿TGF-β1水平可作为反映IgAN活动和进展的早期分子生物学指标。

  2. The Expression of Soluble and Active Recombinant Haemophilus influenzae IgA1 Protease in E. coli

    Directory of Open Access Journals (Sweden)

    Shinong Long

    2010-01-01

    Full Text Available Immunoglobulin A1 (IgA1 proteases from Haemophilus influenzae are extracellular proteases that specifically cleave the hinge region of human IgA1, the predominant class of immunoglobulin present on mucosal membranes. The IgA1 proteases may have the potential to cleave IgA1 complexes in the kidney and be a therapeutic agent for IgA1 nephropathy (IgAN, a disease characterized by deposition of the IgA1 antibody in the glomerulus. We have screened for the expression of recombinant H. influenzae IgA1 protease by combining various expression plasmids, IgA1 protease constructs, and E. coli strains under multiple conditions. Using the method we have developed, approximately 20–40 mg/L of soluble and active H. influenzae IgA1 protease can be produced from E. coli strain C41(DE3, a significant increase in yield compared to the yield upon expression in H. influenzae or other related bacteria.

  3. Occurrence of vascular IgA deposits in clinically normal skin of patients with renal disease.

    Science.gov (United States)

    Faille-Kuyper, E H; Kater, L; Kuijten, R H; Kooiker, C J; Wagenaar, S S; van der Zouwen, P; Mees, E J

    1976-05-01

    Skin and kidney biopsies were performed on 262 patients with various nephropathies. In 45 skin biopsy specimens finely granular deposits of predominantly IgA and late-acting complement factors were detected in the walls of superficial capillaries, sometimes concomitantly with IgM, IgG, C4 or a combination of these proteins. Twelve of the 45 patients presented with anaphylactoid purpura and the majority of the other 33 patients had either recurrent macroscopic or microscopic hematuria. The renal lesions in 32 of these 45 patients consisted of focal segmental intracapillary proliferation. In 35 the kidney biopsy specimen showed mesangial deposits of IgA; in one case IgA was deposited along the glomerular basement membrane. In only three of the remaining 217 patients without cutaneous IgA deposits were typical mesangial IgA deposits found. The close correlation between IgA deposits in cutaneous vessels and focal segmental intracapillary proliferation with mesangial IgA deposits suggests that immunofluorescence examination of skin biopsy specimens could prove of diagnostic value. The results provide additional evidence for a close pathogenic relationship between IgA-associated glomerulonephritis and anaphylactoid purpura.

  4. Influence of Personalized Nursing Mode on the Emotions and Quality of Life in Different Classification IgA Nephropathy Patients%个性化护理模式对不同分级IgA肾病患者情绪及生活质量的影响

    Institute of Scientific and Technical Information of China (English)

    尹晓丽; 王丽; 韩梦雨; 金娟; 朱俊

    2013-01-01

    目的:探讨个性化护理模式对不同分级IgA肾病(IgAN)患者负性情绪及生活质量的影响。方法将80例住院诊断IgAN患者,随机分为观察组和对照组,各40例。对照组采用常规护理。观察组采用个性化护理模式。比较两组干预前后负性情绪及生活质量情况。结果观察组干预后焦虑及抑郁评分明显下降(P<0.01),观察组较对照组干预后的焦虑及抑郁评分明显下降(P<0.01);观察组生活质量评分(除疼痛和肾病负担外)优于对照组(P<0.001)。结论针对不同分级IgAN患者实行个性化护理模式,可以明显改善患者的负性情绪,提高其生活质量。%Objective To evaluate the effect of personalized nursing mode on negative emotions and quality of life in different classification IgA nephropathy (IgAN) patients and observe its clinical value .Methods 80 cases of IgAN patients were randomly di-vided into observation group and control group .Used conventional care for the control group and the patients in observation group were given the model of personalized care .And then compared the negative emotions and quality of life before and after intervention in two groups .Results The negative emotions ,such as anxiety and depression scores in observation group before and after interven-tion improved significantly (P<0 .01) ,the observation group after the intervention of anxiety and depression scores improved signif-icantly compared with the control group (P<0 .01) .Overall health ,society and physical function ,functions of physical and emo-tional ,energy and emotional state ,social cognitive function ,symptoms and discomfort ,kidney affect ,sexual function ,sleep quali-ty ,comprehensive health evaluation and patient satisfaction in the observation group were better than that in control group signifi -cantly(P<0 .001) .Conclusions The personalized nursing mode in different classification of IgAN patients can

  5. [Lithium nephropathy].

    Science.gov (United States)

    Kaczmarczyk, Ireneusz; Sułowicz, Władysław

    2013-01-01

    Lithium salts are the first-line drug therapy in the treatment of uni- and bipolar disorder since the sixties of the twentieth century. In the mid-70s, the first information about their nephrotoxicity appeared. Lithium salts have a narrow therapeutic index. Side effects during treatment are polyuria, polydipsia and nephrogenic diabetes insipidus. Accidental intoxication can cause acute renal failure requiring renal replacement therapy while receiving long-term lithium salt can lead to the development of chronic kidney disease. The renal biopsy changes revealed a type of chronic tubulointerstitial nephropathy. The imaging studies revealed the presence of numerous symmetric microcysts. Care of the patient receiving lithium should include regular determination of serum creatinine, creatinine clearance and monitoring of urine volume. In case of deterioration of renal function reducing the dose should be considered.

  6. Silica Nephropathy

    Directory of Open Access Journals (Sweden)

    N Ghahramani

    2010-06-01

    Full Text Available Occupational exposure to heavy metals, organic solvents and silica is associated with a variety of renal manifestations. Improved understanding of occupational renal disease provides insight into environmental renal disease, improving knowledge of disease pathogenesis. Silica (SiO2 is an abundant mineral found in sand, rock, and soil. Workers exposed to silica include sandblasters, miners, quarry workers, masons, ceramic workers and glass manufacturers. New cases of silicosis per year have been estimated in the US to be 3600–7300. Exposure to silica has been associated with tubulointerstitial disease, immune-mediated multisystem disease, chronic kidney disease and end-stage renal disease. A rare syndrome of painful, nodular skin lesions has been described in dialysis patients with excessive levels of silicon. Balkan endemic nephropathy is postulated to be due to chronic intoxication with drinking water polluted by silicates released during soil erosion. The mechanism of silica nephrotoxicity is thought to be through direct nephrotoxicity, as well as silica-induced autoimmune diseases such as scleroderma and systemic lupus erythematosus. The renal histopathology varies from focal to crescentic and necrotizing glomerulonephritis with aneurysm formation suggestive of polyarteritis nodosa. The treatment for silica nephrotoxicity is non-specific and depends on the mechanism and stage of the disease. It is quite clear that further research is needed, particularly to elucidate the pathogenesis of silica nephropathy. Considering the importance of diagnosing exposure-related renal disease at early stages, it is imperative to obtain a thorough occupational history in all patients with renal disease, with particular emphasis on exposure to silica, heavy metals, and solvents.

  7. Crystalglobulin-induced nephropathy.

    Science.gov (United States)

    Gupta, Vinay; El Ters, Mireille; Kashani, Kianoush; Leung, Nelson; Nasr, Samih H

    2015-03-01

    Crystalline nephropathy refers to renal parenchymal deposition of crystals leading to kidney damage. The most common forms of crystalline nephropathy encountered in renal pathology are nephrocalcinosis and oxalate nephropathy. Less frequent types include urate nephropathy, cystinosis, dihydroxyadeninuria, and drug-induced crystalline nephropathy (e.g., caused by indinavir or triamterene). Monoclonal proteins can also deposit in the kidney as crystals and cause tissue damage. This occurs in conditions such as light chain proximal tubulopathy, crystal-storing histiocytosis, and crystalglobulinemia. The latter is a rare complication of multiple myeloma that results from crystallization of monoclonal proteins in the systemic vasculature, leading to vascular injury, thrombosis, and occlusion. In this report, we describe a case of crystalglobulin-induced nephropathy and discuss its pathophysiology and the differential diagnosis of paraprotein-induced crystalline nephropathy.

  8. Recognition of galactose-deficient O-glycans in the hinge region of IgA1 by N-acetylgalactosamine-specific snail lectins: a comparative binding study.

    Science.gov (United States)

    Gomes, Michelle M; Suzuki, Hitoshi; Brooks, Monica T; Tomana, Milan; Moldoveanu, Zina; Mestecky, Jiri; Julian, Bruce A; Novak, Jan; Herr, Andrew B

    2010-07-13

    Aberrancies in IgA1 glycosylation have been linked to the pathogenesis of IgA nephropathy (IgAN), a kidney disease characterized by deposits of IgA1-containing immune complexes in the glomerular mesangium. IgA1 from IgAN patients is characterized by the presence of galactose (Gal)-deficient O-glycans in the hinge region that can act as epitopes for anti-glycan IgG or IgA1 antibodies. The resulting circulating immune complexes are trapped in the glomerular mesangium of the kidney where they trigger localized inflammatory responses by activating mesangial cells. Certain lectins recognize the terminal N-acetylgalactosamine (GalNAc)-containing O-glycans on Gal-deficient IgA1 and can be potentially used as diagnostic tools. To improve our understanding of GalNAc recognition by these lectins, we have conducted binding studies to assess the interaction of Helix aspersa agglutinin (HAA) and Helix pomatia agglutinin (HPA) with Gal-deficient IgA1. Surface plasmon resonance spectroscopy revealed that both HAA and HPA bind to a Gal-deficient synthetic hinge region glycopeptide (HR-GalNAc) as well as various aberrantly glycosylated IgA1 myeloma proteins. Despite having six binding sites, both HAA and HPA bind IgA1 in a functionally bivalent manner, with the apparent affinity for IgA1 related to the number of exposed GalNAc groups in the IgA1 hinge. Finally, HAA and HPA were shown to discriminate very effectively between the IgA1 secreted by cell lines derived from peripheral blood cells of patients with IgAN and that from cells of healthy controls. These studies provide insight into lectin recognition of the Gal-deficient IgA1 hinge region and lay the groundwork for the development of reliable diagnostic tools for IgAN.

  9. Urinary C3dg and C5b-9 indicate active immune disease in human membranous nephropathy.

    Science.gov (United States)

    Brenchley, P E; Coupes, B; Short, C D; O'Donoghue, D J; Ballardie, F W; Mallick, N P

    1992-04-01

    We have measured complement activation markers, C3dg and C5b-9 in plasma and urine from patients with idiopathic membranous nephropathy and IgA nephropathy. There was no significant difference in levels of plasma C5b-9 between the patient groups. However, high plasma concentrations of C3dg were associated significantly with IgA nephropathy with 45% of patients having levels over 25 U/ml (P less than 0.001). High concentrations of urinary C3dg and C5b-9 were associated significantly with membranous nephropathy (43% and 43% of the patient group, respectively) compared to patients with IgA nephropathy (10% and 0%, respectively, P less than 0.001). In a retrospective analysis of 31 patients with membranous nephropathy, 66% of patients with high initial urinary C5b-9 showed an unstable clinical course compared to 18% of patients with initially absent or low C5b-9 (P less than 0.001). We suggest that high urinary C5b-9 identifies those patients with a membranous lesion which retains an active immunological component contributing to the pathology of progressive glomerular damage.

  10. 呈慢性肾炎的IgA肾病96例临床病理和预后分析%Analysis of clinico-pathological characteristics and prognosis in 96 patients of IgA nephropathy with chronic glomerulonephritis

    Institute of Scientific and Technical Information of China (English)

    徐晓燕; 黄朝兴

    2011-01-01

    Objective: To discuss the early diagnostic criterion of chronic glomerulonephritis in primary IgA Nephropathy (IgAN). Methods: From march 1993 to august 2009, 96 biopsy-proven IgAN patients from The First Affiliated Hospital of Wenzhou Medical College with serum creatinine level above the upper limit (Scr≥ 105 μ mol/L by picric kinetic method) were enrolled in this study. The patients were divided into two groups according to creatinine clearance rate (Ccr): Group A (Ccr≥ 60 mL/min, n=43), and group B (Ccr<60 mL/min, n=53). Two groups of patients with clinico-pathological characteristics and prognosis were compared and analyzed. Endpoint was defined as double of baseline serum creatinine or Ccr<15 mL/min.Results:Among 96 cases with IgAN, incidence of proteinuria>1.O g/day, severe glomerular sclerosis, moderate to severe tubelointerstitial lesions was 73.0%,82.3%and 76.0%respectively,and the average Ccr at renal biopsy was (59.15± 20.73) mL/min. All cases with a median followup of 18.5 months, 21 patients developed to ESRD. The incidence of intermediate to severe tubulointerstitial lesions and nephrotic-range proteinuria in group B was significantly higher than that in group A (84.9% vs 65.1%, P<O.05 and 41.5% vs 18.6%, P<O.05, respectively).Patients in B and A groups were followed-up for a median duration of 33 and 21 months,respectively, 28,0% and 14.0% patients reaching the endpoint. Conclusion: For patients with IgAN, the fundamental clinico-pathological features of chronic glomerulonephritis are as follows:①durative proteinuria>1.O g/day; ②lasting Ccr decrease; ③moderate to severe tubulointerstitial lesions accompanied with severe glomerular sclerosis. The three items mentioned above may be used as the early diagnostic criterion for chronic glomerulonephritis.%目的:探讨在原发性IgA肾病(IgAN)中如何早期作出慢性肾小球肾炎(慢性肾炎)的临床诊断.方法:于1993年3月-2009年8月我院肾活检

  11. Clinical study on treatment of IgA nephropathy with renal insufficiency by corticosteroid, corticosteroid combined with cyclophosphamide and corticosteroid combined with mycophenolate mofetil%单纯激素、激素联合环磷酰胺和激素联合麦考酚酯治疗肾功能不全IgA肾病的临床研究

    Institute of Scientific and Technical Information of China (English)

    王伟铭; 贾晓媛; 潘晓霞; 沈平雁; 刘剑; 徐丽梨; 李娅; 王朝晖; 李晓

    2013-01-01

    Objective To investigate the clinical efficacy and safety of corticosteroid, corticosteroid combined with cyclophosphamide ( CTX) and corticosteroid combined with mycophenolate mofetil ( MMF) in IgA nephropathy (IgAN) with renal insufficiency. Methods Patients confirmed as primary IgAN by renal biopsy were selected, with chronic renal disease (CKD) of 3-4 stage and moderate renal lesions. Sixty patients were enrolled, and randomly received corticosteroid therapy (corticosteroid group, re =20), corticosteroid combined with CTX therapy (corticosteroid + CTX group, n=20) and corticosteroid combined with MMF therapy ( corticosteroid + MMF group, n = 20) . The 24 h urine protein, renal function parameters and adverse effect were observed during treatment. Results With the time of treatment, 24 h urine protein was gradually reduced in each group, and 24 h urine protein in corticosteroid group and corticosteroid + CTX group 3, 6 and 12 months after treatment and in corticosteroid + MMF group 12 months after treatment was significantly lower than the baseline (P 0. 05). Eight patients (8/20) in corticosteroid + MMF group suffered from serious pulmonary infection during treatment for 3 to 4 months, the baseline eGFR of whom was significantly lower than that of patients without serious pulmonary infection in corticosteroid + MMF group (P <0. 05). Conclusion Twenty-four hour urine protein can be significantly decreased with corticostsroid therapy, corticosteroid combined with CTX therapy and corticosteroid combined with MMF therapy in patients with IgAN and impaired renal function, and stable renal function can be maintained during treatment. Intensive follow-up should be carried out in the treatment with MMF due to the possibility of occurrence of serious pulmonary infection.%目的 观察单纯激素、激素联合环磷酰胺(CTX)、激素联合霉酚酸酯(MMF)治疗伴慢性肾功能不全原发性IgA肾病(IgAN)患者的临床效果和安全性.方法 选取经肾穿

  12. Immunoglobulin A nephropathy: Basic characteristics

    Directory of Open Access Journals (Sweden)

    Petrović Lada

    2003-01-01

    Full Text Available Introduction Immunoglobulin A nephropathy (IgAN is one of the most common forms of primary glomerulonephritis in many countries. Most clinical features of IgAN point to a renal problem, such as recurrent macroscopic hematuria or asymptomatic microscopic hematuria and proteinuria. Pathologic features of IgAN present with different types and different degrees of glomerular tubulointerstitial and vascular lesions. The aim of this study was detailed analysis of clinical and laboratory findings, as well as findings of immunofluorescence and light microscopy. We also investigated associations between these factors. Material and methods We investigated 60 patients who underwent renal biopsy. The study was partly retrospective and partly prospective. Results The average age of patients was 34.19 years. Male female ratio was 2.33:1. IgAN was most frequently asymptomatic (83.33% as microhematuria and proteinuria, while gross hematuria was found in 16.667%. Renal biopsy material was analyzed by light microscopy revealing changes in all glomerular structures. Immunofluorescence microscopy demonstrated dominant IgA deposits. This study established association of glomerulosclerosis with clinical features of disease. Discussion and conclusions IgAN frequently develops in the 4th decade of life, mostly in males and presents as asymptomatic (83.33%. Patohistological changes include all glomerular structures. There is no specific serological test for IgAN, but pathological changes affect clinical features of the disease, as proteinuria and increase of creatinine concentration.

  13. Blood Test: Immunoglobulin A (IgA)

    Science.gov (United States)

    ... Your 1- to 2-Year-Old Blood Test: Immunoglobulin A (IgA) KidsHealth > For Parents > Blood Test: Immunoglobulin A (IgA) Print A A A What's in ... An IgA test measures the blood level of immunoglobulin A, one of the most common antibodies in ...

  14. Iga viies SVH on ennetatav

    Index Scriptorium Estoniae

    2009-01-01

    ONTARGET uuring tõestab, et telmisartaan kaitseb kõrge kardiovaskulaarse riskiga patsiente sama tõhusalt kui ramipriil, kuid on paremini talutav. Uuringust võib järeldada, et telmisartaaniga saab ennetada iga viiendat tõsist kardiovaskulaarset juhtumit. Eesti arstidele tutvustati uuringut 6. mail toimunud sümpoosiumil

  15. Kidney Disease (Nephropathy)

    Science.gov (United States)

    ... Text Size: A A A Listen En Español Kidney Disease (Nephropathy) Kidneys are remarkable organs. Inside them ... resulting in kidney disease. How Does Diabetes Cause Kidney Disease? When our bodies digest the protein we ...

  16. Proliferative retinopathy predicts nephropathy

    DEFF Research Database (Denmark)

    Karlberg, Charlotte; Falk, Christine; Green, Anders;

    2012-01-01

    We wanted to examine proliferative retinopathy as a marker of incident nephropathy in a 25-year follow-up study of a population-based cohort of Danish type 1 diabetic patients and to examine cross-sectional associations between nephropathy and retinopathy in long-term surviving patients of the same...... photographs at follow-up. Single spot urine was used to evaluate nephropathy at both examinations. Proliferative retinopathy was present in 29 patients (15.8%) at baseline. At follow-up, these patients were more likely to macroalbuminuria (20.7% vs. 6.5%) than patients without proliferative retinopathy...... at baseline. In a multivariate logistic regression adjusted for baseline age, sex, duration of diabetes, smoking, HbA(1,) systolic and diastolic blood pressure, odds ratio of nephropathy (micro- and macroalbuminuria combined) was 2.98 (95% confidence interval 1.18-7.51, p = 0.02) for patients...

  17. CagA, a major virulence factor of Helicobacter pylori, promotes the production and underglycosylation of IgA1 in DAKIKI cells

    Energy Technology Data Exchange (ETDEWEB)

    Yang, Man [Department of Nephrology, The First Affiliated Hospital of Chengdu Medical College, Chengdu City 610500 (China); Li, Fu-gang [Department of Nephrology, Affiliated Hospital of Luzhou Medical College, Luzhou City 646000 (China); Xie, Xi-sheng [Department of Nephrology, Second Clinical Medical Institution of North Sichuan Medical College (Nanchong Central Hospital), Nanchong City 637400 (China); Wang, Shao-qing [Department of Nephrology, The First Affiliated Hospital of Chengdu Medical College, Chengdu City 610500 (China); Fan, Jun-ming, E-mail: junmingfan@163.com [Department of Nephrology, The First Affiliated Hospital of Chengdu Medical College, Chengdu City 610500 (China); Department of Nephrology, Affiliated Hospital of Luzhou Medical College, Luzhou City 646000 (China)

    2014-02-07

    Highlights: • CagA stimulated cell proliferation and the production of IgA1 in DAKIKI cells. • CagA promoted the underglycosylation of IgA1 in DAKIKI cells. • CagA decreased the expression of C1GALT1 and its chaperone Cosmc in DAKIKI cells. • Helicobacter pylori infection may participate in the pathogenesis of IgAN via CagA. - Abstract: While Helicobacter pylori (Hp) infection is closely associated with IgA nephropathy (IgAN), the underlying molecular mechanisms remain to be elucidated. This study was to investigate the effect of cytotoxin associated gene A protein (CagA), a major virulence factor of Hp, on the production and underglycosylation of IgA1 in the B cell line DAKIKI cells. Cells were cultured and treated with recombinant CagA protein. We found that CagA stimulated cell proliferation and the production of IgA1 in a dose-dependent and time-dependent manner. Moreover, CagA promoted the underglycosylation of IgA1, which at least partly attributed to the downregulation of β1,3-galactosyltransferase (C1GALT1) and its chaperone Cosmc. In conclusion, we demonstrated that Hp infection, at least via CagA, may participate in the pathogenesis of IgAN by influencing the production and glycosylation of IgA1 in B cells.

  18. Glyco-engineering for the production of recombinant IgA1 with distinct mucin-type O-glycans in plants.

    Science.gov (United States)

    Dicker, Martina; Maresch, Daniel; Strasser, Richard

    2016-11-01

    IgA nephropathy (IgAN) is a common autoimmune disease that is characterized by formation and deposition of IgA1-containing immune complexes frequently leading to end-stage kidney disease. The IgA1 in these immune complexes carries aberrantly glycosylated O-glycans. In circulating IgA1 these galactose-deficient mucin-type O-glycans are bound by autoantibodies and thus, contribute to immune complex formation and pathogenesis. Even though the disease is associated with the overproduction of aberrant O-glycans on IgA1, specific structure-function-studies of mucin-type O-glycans are limited. Compared to other expression hosts, plants offer the opportunity for de novo synthesis of O-glycans on recombinant glycoproteins as they are lacking the mammalian O-glycosylation pathway. Recently, we demonstrated that Nicotiana benthamiana are suitable for the generation of distinct O-glycans on recombinant IgA1. Here, we expand our engineering repertoire by in planta generation of galactose-deficient and α2,6-sialylated O-glycans which are the prevailing glycans detected on IgA1 from patients with IgAN.

  19. M-Type Phospholipase A2 Receptor as Target Antigen in Idiopathic Membranous Nephropathy

    Science.gov (United States)

    Beck, Laurence H.; Bonegio, Ramon G.B.; Lambeau, Gérard; Beck, David M.; Powell, David W.; Cummins, Timothy D.; Klein, Jon B.; Salant, David J.

    2009-01-01

    BACKGROUND Idiopathic membranous nephropathy, a common form of the nephrotic syndrome, is an antibody-mediated autoimmune glomerular disease. Serologic diagnosis has been elusive because the target antigen is unknown. METHODS We performed Western blotting of protein extracts from normal human glomeruli with serum samples from patients with idiopathic or secondary membranous nephropathy or other proteinuric or autoimmune diseases and from normal controls. We used mass spectrometry to analyze the reactive protein bands and confirmed the identity and location of the target antigen with a monospecific antibody. RESULTS Serum samples from 26 of 37 patients (70%) with idiopathic but not secondary membranous nephropathy specifically identified a 185-kD glycoprotein in non-reduced glomerular extract. Mass spectrometry of the reactive protein band detected the M-type phospholipase A2 receptor (PLA2R). Reactive serum specimens recognized recombinant PLA2R and bound the same 185-kD glomerular protein as did the monospecific anti-PLA2R antibody. Anti-PLA2R autoantibodies in serum samples from patients with membranous nephropathy were mainly IgG4, the predominant immunoglobulin subclass in glomerular deposits. PLA2R was expressed in podocytes in normal human glomeruli and colocalized with IgG4 in immune deposits in glomeruli of patients with membranous nephropathy. IgG eluted from such deposits in patients with idiopathic membranous nephropathy, but not in those with lupus membranous or IgA nephropathy, recognized PLA2R. CONCLUSIONS A majority of patients with idiopathic membranous nephropathy have antibodies against a conformation-dependent epitope in PLA2R. PLA2R is present in normal podocytes and in immune deposits in patients with idiopathic membranous nephropathy, indicating that PLA2R is a major antigen in this disease. PMID:19571279

  20. Linear IgA dermatosis: report of an infantile case and analysis of 213 cases in Japan.

    Science.gov (United States)

    Horiguchi, Yuji; Ikoma, Akihiko; Sakai, Rie; Masatsugu, Asako; Ohta, Miyuki; Hashimoto, Takashi

    2008-11-01

    A 3-year-old boy presented with multiple vesicles, showing a rosette-like arrangement around the crusts. Histopathological and immunohistochemical examinations demonstrated subepidermal blistering with neutrophilic infiltration associated with deposition of IgA, but not IgG, linearly distributed along the basement membrane zone (BMZ) of the epidermis. Indirect immunofluorescence revealed circulating antibodies (IgA class, x160) against the BMZ of guinea pig lip skin. Based on the diagnosis of linear IgA dermatosis (LAD) of childhood, administration of dexamethasone (2 mg/day) was started, and the eruptions diminished immediately. Western blot analysis using extract of the HaCaT cell as a substrate, demonstrated the corresponding antigen at 120-kDa molecular weight. There have been 213 cases of LAD reported in Japan including conference abstracts and these were studied to determine whether infantile cases differed from adult ones, and whether cases associated with IgG as well as IgA (IgA/G type), differed from the cases associated with IgA only (IgA type). IgG contributed less frequently to the infantile type (age of onset, or =16 years). Clinical appearance did not show any obvious difference between the IgA/G type and IgA type. However, three-quarters of cases showing localization of antigen to the dermal side were the IgA/G type.

  1. Mesangial cell-derived tumor necrosis factor α up-regulates the expression of tubular liver type fatty acid binding-protein and its renoprotective role in IgA nephropathy%系膜细胞源性肿瘤坏死因子α上调IgA肾病肾小管肝型脂肪酸结合蛋白的表达及其肾脏保护作用

    Institute of Scientific and Technical Information of China (English)

    佐楠; 栗霄立; 王力宁; 李子龙; 王均; 冯江敏; 马健飞; 范秋灵; 姚丽

    2011-01-01

    Objective To explore the mechanism of up-regulation of tubular liver-type fatty acid binding-protein (L-FABP) in IgA nephropathy (IgAN) and its renoprotective role.Methods Murine mesangial cells (MCs) from primary cell culture were cultured with aggregated IgA (AIgA) (10 to 250 mg/L) for 48 hours. The supernatant after culture was collected as AIgA-MC medium. Murine proximal tubular cell line (mProx) stably expressing human L-FABP (hL-FABP) by transfection (mProx-L) were cultured with AIgA, AIgA-MC medium and /or neutralizing anti-TNF-α antibody and recombinant murine TNF-α, respectively. AIgA-MC medium (AIgA final concentration was 25 mg/L) was cultured with mProx and mProx-L cells. The mRNA expressions of hL-FABP and MCP-1 of the cells were detected by real-time PCR. The protein expressions of hL-FABP and 4-HNE of the cells were detected by Western blotting. Results (1) The hL-FABP mRNA and protein expression stimulated by AIgA-MC medium was significantly higher as compared to AIgA (P<0.01). (2) Pre-incubation of neutralizing anti-TNF-α antibody (final concentration was 1 and 5 mg/L) with mProx-L cells could significantly suppress the up-regulation of hL-FABP protein expression induced by AlgA-MC medium (P<0.05 and P<0.01).(3) Recombinant murine TNF-α (final concentration was 50 and 250 ng/L) also induced a significant up-regulation of hL-FABP expression (P<0.01). (4) After the stimulation of AIgA-MC medium, both 4-HNE protein expression and MCP-1 mRNA expression were significantly suppressed in mProx-L cells compared to those of mProx cells (P <0.05 and P<0.01). Conclusion Mesangial cell-derived TNF-α can induce up-regulation of tubular L-FABP expression. Overexpression of tubular L-FABP may lessen the progression of IgAN by reducing oxidative stress and inflammatory mediators.%目的 探讨体外近曲小管肝型脂肪酸结合蛋白(L-FABP)在IgA肾病(IgAN)中的上调机制及其对肾脏的保护作用.方法 原代培养的小鼠系膜

  2. [Clinical symptoms in IgA deficiency].

    Science.gov (United States)

    De Oliveira-Serra, Flavio Augusto; Mosca, Tainá; Santos de Menezes, Maria da Conceição; Carvalho-Neves Forte, Wilma

    2017-01-01

    Antecedentes: La deficiencia de inmunoglobulina A (IgA) es la inmunodeficiencia primaria más frecuente. El diagnóstico oportuno y el seguimiento clínico pueden mejorar la calidad de vida de los portadores. Para ello, deben estudiarse y entenderse las manifestaciones clínicas de este trastorno. Objetivo: Determinar las manifestaciones clínicas de la deficiencia de IgA. Métodos: Estudio transversal, retrospectivo, exploratorio, realizado mediante análisis de expedientes de 39 pacientes con deficiencia de IgA. Resultados: De los pacientes analizados, 10 fueron diagnosticados con deficiencia total de IgA y 29 con deficiencia parcial. Las principales manifestaciones clínicas fueron rinoconjuntivitis y asma alérgicas. En los pacientes con deficiencia total de IgA, además de las enfermedades alérgicas se observó un mayor número de cuadros infecciosos de rinosinusitis, amigdalitis y conjuntivitis (p estudio, las principales manifestaciones clínicas de la deficiencia de IgA fueron los cuadros alérgicos de rinoconjuntivitis y el asma; además, los pacientes portadores de deficiencia total de IgA presentaron aumento significativo de cuadros infecciosos de rinosinusitis, amigdalitis y conjuntivitis, comparados con los pacientes con deficiencia parcial de IgA.

  3. IgA as therapeutic antibody

    NARCIS (Netherlands)

    Leusen, Jeanette H W

    2015-01-01

    This review is focused on the promises of IgA as a new therapeutic antibody. For more than 30 years IgG molecules have been used in the clinic in the fields of oncology, hematology, auto immune diseases and infections. However, IgA might be a good alternative, since it recruits different effector ce

  4. OBSTRUCTIVE NEPHROPATHY: ITS PHYSIOPATHOLOGY

    Directory of Open Access Journals (Sweden)

    Musso C

    2011-01-01

    Full Text Available Obstructive nephropathy is the functional and /or parenchymal renal damage secondary to the urinary tract occlusion at any part of it. The inducing urinary obstruction diseases can vary depending on the patient´s age and gender. There are many renal dysfunction inducing mechanisms involved in this entity: increase in the intra-luminal pressure, ureteral dilatation with ineffective ureteral peristalsis, glomerular ultrafiltration net pressure reduction, intra-renal glomerular blood flux reduction due to vasoconstriction, and local disease of chemotactic substances. Obstructive nephropathy can also lead to hypertension (vasoconstriction-hypervolemia, hyperkalemia, metabolic acidosis (aldosterone resistance, diabetes insipidus (vasopressine resistance. In conclusion, since obstructive nephropathy is a potentially reversible cause of renal dysfunction, it should always be taken into account among the differential diagnosis of renal failure inducing mechanisms.

  5. Separation of Two Distinct O-Glycoforms of Human IgA1 by Serial Lectin Chromatography Followed by Mass Spectrometry O-Glycan Analysis.

    Science.gov (United States)

    Lehoux, S; Ju, T

    2017-01-01

    Human immunoglobulin A1 (IgA1), which carries four to six mucin-type O-glycans (O-glycans) on its hinge region (HR), is the most abundant O-glycoprotein in plasma or serum. While normal O-glycans from hematopoietic-originated cells are core 1-based complex structures, many reports showed that the IgA1 from patients with IgA nephropathy (IgAN) carries undergalactosylated or truncated O-glycans such as the Tn antigen and its sialylated version the SialylTn (STn) antigen on the HR. Yet, there is still a debate whether Tn/STn on the HR of IgA1 is specific to the IgA1 from patients with IgAN since these antigens have also been seen in serum IgA1 of healthy individuals. An additional question is whether the O-glycans at all sites on the two HRs of one IgA1 molecule are homogeneous (either all normal or all Tn/STn) or heterogeneous (both normal and Tn/STn O-glycans). To address these questions, we conducted a systematic study on the O-glycans of plasma IgA1 from both IgAN patients and healthy controls using serial HPA and PNA lectin chromatography followed by western blotting and further analysis of O-glycans from HPA-bound and PNA-bound IgA1 fractions by mass spectrometry. Unexpectedly, we found that a variable minor fraction of IgA1 from both IgAN patients and healthy controls had Tn/STn antigens, and that the O-glycoprotein IgA1 molecules from most samples had only two distinct O-glycoforms: one major glycoform with homogeneous normal core 1-based O-glycans and one minor glycoform with homogeneous Tn/STn antigens. These results raised a serious question about the role of Tn/STn antigens on IgA1 in pathogenesis of IgAN, and there is a demand for a practical methodology that any laboratory can utilize to analyze the O-glycans of IgA1. Herein, we describe the methodology we developed in more detail. The method could also be applied to the analysis of any other O-glycosylated proteins.

  6. Ichthyosiform mycosis fungoides with alopecia and atypical membranous nephropathy

    Directory of Open Access Journals (Sweden)

    Qiang Zhou

    2011-01-01

    Full Text Available We describe here a rare case of variant of mycosis fungoides (MF: ichthyosiform MF with alopecia and atypical membranous nephropathy. The diagnosis was made based on the following findings: generalized ichthyosis-like eruption, alopecia, enlarged superficial lymph nodes, proteinuria, and hematuria, the histological features of the skin biopsy from both ichthyotic and alopecic lesions with immunohistochemical staining, and the renal biopsy examination with immunofluorescence. The histological examination of ichthyotic and alopecic lesions displayed a predominant infiltration of atypical lymphocytes in the upper dermis with the characteristics of epidermotropism and folliculotropism. Immunohistochemical studies demonstrated that most infiltrated atypical lymphocytes were CD3, CD4, and CD45RO positive, whereas negative for CD5, CD7, CD20, CD30, and CD56. A renal biopsy examination revealed atypical membranous nephropathy with deposition of immunoglobulin G (IgG, IgM, IgA, C1q, and C3. In this case atypical membranous nephropathy was involved, which is very uncommon and has never been presented in the literature to date. Although ichthyosiform MF usually features a relatively favorable course, diffuse alopecia and the renal involvement in this case might indicate aggressive disease and poor prognosis.

  7. Haridus - iga lapse õigus / Gerd Tarand

    Index Scriptorium Estoniae

    Tarand, Gerd, 1985-

    2009-01-01

    MTÜ Mondo alustas veebruaris maailmaharidusprojektiga „Haridus - iga lapse õigus“, mille üldine eesmärk on tõsta Eesti elanikkonna, eriti noorte huvi arengumaade vastu ning teadmisi ja arusaamist arengumaade haridusega seonduvatest probleemidest

  8. Mouse Models of Diabetic Nephropathy

    OpenAIRE

    Brosius, Frank C.; Alpers, Charles E.; Bottinger, Erwin P.; Breyer, Matthew D.; Coffman, Thomas M.; Gurley, Susan B.; Harris, Raymond C.; Kakoki, Masao; Kretzler, Matthias; Leiter, Edward H.; Levi, Moshe; McIndoe, Richard A.; Sharma, Kumar; Smithies, Oliver; Susztak, Katalin

    2009-01-01

    Diabetic nephropathy is the major cause of end-stage renal disease worldwide. Despite its prevalence, identification of specific factors that cause or predict diabetic nephropathy has been delayed in part by lack of reliable animal models that mimic the disease in humans. The Animal Models of Diabetic Complications Consortium (AMDCC) was created 8 years ago by the National Institutes of Health to develop and characterize models of diabetic nephropathy and other complications. This interim rep...

  9. Differential expression of laminin isoforms in diabetic nephropathy and other renal diseases.

    Science.gov (United States)

    Setty, Suman; Michael, Alfred A; Fish, Alfred J; Michael Mauer, S; Butkowski, Ralph J; Virtanen, Ismo; Kim, Youngki

    2012-06-01

    Laminin a non-collagenous glycoprotein is a major component of the renal glomerular basement membrane and mesangium. Thus far eleven distinct chains have been described, permutations of which make up 15 laminin isoforms. Laminin molecules interact with cells and other matrix molecules during organ development and differentiation. We studied the distribution of laminin isoforms in patients with type 1 diabetic nephropathy, membranous nephropathy, membranoproliferative glomerulonephritis and IgA nephropathy/ Henoch-Schönlein purpura. Immunofluorescence microscopic studies with laminin-chain-specific antibodies to the α1, α2, α5, β1, β2 and γ1 chains detected α2, β1 and γ1 chain expression in the normal mesangium and α5, β2 and γ1 in normal glomerular basement membrane. Significantly, constituents of the glomerular basement membrane, α5, β2 and γ1 chains were overexpressed in kidneys with diabetic nephropathy. Initially the constituents of the mesangium increased commensurate with the degree of mesangial expansion and degree of diabetic nephropathy. Reduction in α2 chain intensity was observed with severe mesangial expansion and in the areas of nodular glomerulosclerosis. In addition, with late disease aberrant expression of α2 and β2 chains was observed in the mesangium. Glomerular basement membrane in renal disease overexpressed molecules normally present in that location. In summary, the alterations in basement membrane composition in various renal diseases seem to not only reflect the balance between synthesis and degradation of normal basement membrane constituents, but also their aberrant expression.

  10. Light chain nephropathy

    Directory of Open Access Journals (Sweden)

    Sihem Darouich

    2015-01-01

    Full Text Available Light chain deposition disease (LCDD is characterized by the tissue deposition of monotypic immunoglobulin light chains of either kappa or lambda isotype. It is the archetypal systemic disease that is most frequently diagnosed on a kidney biopsy, although the deposits may involve several other organs. This brief review focuses on the clinicopathological features of LCDD-associated nephropathy with an emphasis on the diagnostic and therapeutic difficulties related to this elusive condition.

  11. Scorpion sting nephropathy

    OpenAIRE

    2011-01-01

    Scorpion envenomations are ubiquitous, but nephropathy is a rare manifestation, reported mainly from the Middle East and North Africa. Rapid venom redistribution from blood, delayed excretion from the kidneys, direct toxicity of venom enzymes, cytokine release and afferent arteriolar constriction have been seen in experimental animals. Haemoglobinuria, acute tubular necrosis, interstitial nephritis and haemolytic–uraemic syndrome have been documented in human victims of scorpion envenomation....

  12. Contrast-induced nephropathy

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    Ricardo A. García Hernández

    2016-06-01

    Full Text Available Contrast-induced nephropathy is an important complication associated with the use of contrast media. Favoring factors for the development of contrast-induced nephronpathy have been widely described, being diabetes mellitus and previous renal disease the greatest risk. The pathophysiology is a complex process where the medullary hypoxia represents the trigger element. Previous hydration and the use of low osmolality contrast are the most recommended measures to prevent its development.

  13. IgA autoantibodies in the pemphigoids and linear IgA bullous dermatosis

    NARCIS (Netherlands)

    Horvath, Barbara; Niedermeier, Andrea; Podstawa, Eva; Mueller, Ralf; Hunzelmann, Nicolas; Karpati, Sarolta; Hertl, Michael

    2010-01-01

    Background: Patients with bullous pemphigoid (BP), mucous membrane pemphigoid (MMP) and pemphigoid gestationis (PG) have IgG antibodies against BP180 and BP230, components of the hemidesmosomes. Patients with linear IgA bullous dermatosis (LABD) have IgA autoantibodies against a 97/120-kDa protein w

  14. Diabetic nephropathy : pathology, genetics and carnosine metabolism

    NARCIS (Netherlands)

    Mooyaart, Antien Leonora

    2011-01-01

    My thesis concerns different aspects of diabetic nephropathy. A pathologic classification of diabetic nephropathy is developed, a meta-analyis of genes in diabetic nephropathy is developed and the other chapters are about the CNDP1 gene in relation to kidney disease, mainly diabetic nephropathy.

  15. [Classification of Diabetic Nephropathy 2014].

    Science.gov (United States)

    Haneda, Masakazu; Utsunomiya, Kazunori; Koya, Daisuke; Babazono, Tetsuya; Moriya, Tatsumi; Makino, Hirofumi; Kimura, Kenjiro; Suzuki, Yoshiki; Wada, Takashi; Ogawa, Susumu; Inaba, Masaaki; Kanno, Yoshihiko; Shigematsu, Takashi; Masakane, Ikuto; Tsuchiya, Ken; Honda, Keiko; Ichikawa, Kazuko; Shide, Kenichiro

    2014-01-01

    The Committee on Diabetic Nephropathy revised the classification of diabetic nephropathy in view of the current status of eGFR and CKD in Japan. To make revisions for the classification of diabetic nephropathy 2014, the Committee carefully evaluated the report of the Research Group on Diabetic Nephropathy, Ministry of Health, Labour, and Welfare of Japan. The major revisions made were as follows: 1. eGFR can be used for the evaluation of GFR; 2. In stage 3 (overt nephropathy), A and B were combined; 3. Stage 4 (renal failure) was defined as GFR less than 30 mL/min/1.73 m2, regardless of albuminuria; and 4. The importance of differential diagnosis was stressed in all stages.

  16. Contrast induced nephropathy

    DEFF Research Database (Denmark)

    Stacul, Fulvio; van der Molen, Aart J; Reimer, Peter

    2011-01-01

    measures used to reduce the incidence of CIN, and the management of patients receiving metformin. Key Points • Definition, risk factors and prevention of contrast medium induced nephropathy are reviewed. • CIN risk is lower with intravenous than intra-arterial iodinated contrast medium. • eGFR of 45 ml....../min/1.73 m (2) is CIN risk threshold for intravenous contrast medium. • Hydration with either saline or sodium bicarbonate reduces CIN incidence. • Patients with eGFR ≥ 60 ml/min/1.73 m (2) receiving contrast medium can continue metformin normally....

  17. Contrast induced nephropathy

    DEFF Research Database (Denmark)

    Stacul, Fulvio; van der Molen, Aart J; Reimer, Peter

    2011-01-01

    measures used to reduce the incidence of CIN, and the management of patients receiving metformin. Key Points • Definition, risk factors and prevention of contrast medium induced nephropathy are reviewed. • CIN risk is lower with intravenous than intra-arterial iodinated contrast medium. • eGFR of 45 ml....../min/1.73 m (2) is CIN risk threshold for intravenous contrast medium. • Hydration with either saline or sodium bicarbonate reduces CIN incidence. • Patients with eGFR = 60 ml/min/1.73 m (2) receiving contrast medium can continue metformin normally....

  18. Heroin crystal nephropathy.

    Science.gov (United States)

    Bautista, Josef Edrik Keith; Merhi, Basma; Gregory, Oliver; Hu, Susie; Henriksen, Kammi; Gohh, Reginald

    2015-06-01

    In this paper we present an interesting case of acute kidney injury and severe metabolic alkalosis in a patient with a history of heavy heroin abuse. Urine microscopy showed numerous broomstick-like crystals. These crystals are also identified in light and electron microscopy. We hypothesize that heroin crystalizes in an alkaline pH, resulting in tubular obstruction and acute kidney injury. Management is mainly supportive as there is no known specific therapy for this condition. This paper highlights the utility of urine microscopy in diagnosing the etiology of acute kidney injury and proposes a novel disease called heroin crystal nephropathy.

  19. [Linear IgA bullous dermatosis].

    Science.gov (United States)

    Lorette, Gérard; Georgesco, Gabriella

    2010-10-01

    The linear IgA bullous dermatosis can have various aspects involving erythema and bullous lesions. It is a rare disease. Two peaks of frequency are noticed in children before puberty and in adults around 60 years of age. The histological and immunological characterisation is infraepidermal bullous lesions and linear deposits of IgA along the dermoepidermal basement membrane. There are some targets antigens. There is often a medical condition that seems to trigger. The link with drugs in particular with vancomycin was established. The mainstay of treatment is dapsone generally associated with steroids.

  20. Outcomes of renal transplantation in patients with immunoglobulin A nephropathy in India

    Directory of Open Access Journals (Sweden)

    Chacko B

    2007-01-01

    Full Text Available Background: There is a paucity of data on the course of renal transplant in patients with immunoglobulin A (IgA nephropathy (IgAN from India. While the natural history of IgAN in the Indian context is rapidly progressive, the post-transplant course remains speculative. Aim: To study the graft survival in renal transplant recipients whose native kidney disease was IgAN and the incidence and correlates of recurrent disease. Settings and Designs: Retrospective case control study from a Nephrology unit of a large tertiary care center. Materials and Methods: The outcomes of 56 transplant patients (58 grafts with biopsy-proven IgAN and of 116 patients without IgAN or diabetic nephropathy, transplanted during the same period were analyzed. Correlates of biopsy-confirmed recurrent disease were determined. Statistical Analysis: Means were analyzed by Student′s t test and Mann-Whitney test; proportions were determined by Chi-square analysis and graft survival curves were generated using the Kaplan-Meier. Results: Five-year graft survival for IgA patients was not significantly different from that in the reference group (90% and 79%, P = 0.6. During a mean follow-up of 42 months (range, 1-144, 28 event graft biopsies were required in 20 grafts of IgAN. Histological recurrence was diagnosed in five of the 20 available biopsies (25% after a mean duration of 28 months. Recurrence did not correlate with donor status, HLA B35 and A2, recipient age, gender or immunosuppression. Conclusions: Renal transplantation is an appropriate treatment modality for IgA nephropathy patients with end-stage renal disease in India, despite the potential for recurrent disease. The posttransplant course is an indolent one when compared to the malignant pretransplant phase.

  1. IgA Antibodies in Rett Syndrome

    Science.gov (United States)

    Reichelt, K. L.; Skjeldal, O.

    2006-01-01

    The level of IgA antibodies to gluten and gliadin proteins found in grains and to casein found in milk, as well as the level of IgG to gluten and gliadin, have been examined in 23 girls with Rett syndrome and 53 controls. Highly statistically significant increases were found for the Rett population compared to the controls. The reason for this…

  2. Do we need to measure total serum IgA to exclude IgA deficiency in coeliac disease?

    Science.gov (United States)

    Sinclair, D; Saas, M; Turk, A; Goble, M; Kerr, D

    2006-01-01

    Background Screening for IgA deficiency in patients with coeliac disease is essential because of the increased incidence of IgA deficiency associated with the disease, which usually relies on the estimation of IgA levels in each case. Aim To devise a method of excluding IgA deficiency without measuring total serum IgA in each case. Materials and methods The optical density readings on enzyme‐linked immunosorbent assay (ELISA) of 608 routine samples received for tissue transglutaminase (TTG) antibody testing for coeliac disease were compared with their total IgA concentrations. Dilution experiments were also carried out to ensure linear relationships between optical density on ELISA and IgA concentrations and to compare the sensitivities for TTG and endomysium antibodies in TTG‐positive samples. Results and discussion A clear relationship was shown between total IgA concentration and TTG optical density readings by ELISA. To ensure a positive TTG result if antibodies are present, it was possible to recommend an optical density level above which all samples have sufficient IgA. Samples with optical density <0.05 should be investigated further by estimating total IgA and, if low, samples should be subjected to immunofluorescence microscopy testing for IgA and IgG endomysium antibodies. Conclusions An easier, more cost‐effective and practical way of excluding IgA deficiency in the investigation on coeliac disease is reported. PMID:16489174

  3. ANTIOXIDANT STATUS IN DIABETIC NEPHROPATHY

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    Giriraja

    2015-12-01

    Full Text Available BACKGROUND Hyperglycemia and dislipidemia in DM induce increased lipid peroxdation and free radical formation. This is an important mechanism of microangiopathy. AIM To measure the antioxidant status in type 2 DM with nephropathy and compared with nondiabetic control group. MATERIALS AND METHODS 50 type 2 DM patients aged between 50 to 70 years according to national diabetes data group criteria with nephropathy diagnosed on the basis of history, physical examination and biochemical parameters were included. 50 age and sex matched apparently healthy individuals with normal plasma glucose, normal renal parameters and with no symptoms suggestive of DM were taken as controls. RESULTS Antioxidant status was significantly less in patients with diabetic nephropathy. CONCLUSION Data suggests that alteration in antioxidant status may help predict the risk of diabetic nephropathy.

  4. GENETICS ASPECTS OF DIABETIC NEPHROPATHY

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    Oana-Elena Sauca

    2010-09-01

    Full Text Available Diabetic nephropathy is a clinical syndrome characterized by persistent albuminuria, a relentless decline in GFR, raised arterial blood pressure, and increased relative mortality for cardiovascular diseases. The pathogenesis of diabetic nephropathy is multifactorial, with contributions from metabolic abnormalities, hemodynamic alteration, and various growth and genetic factors. The identification of the main genes would allow the detection of those individuals at high risk for diabetic nephropathy and better understanding of its pathophysiologyas well.The present review discusses the main information available in literature regarding some genetic variants (involved in the renin-angiotensin system, glucose and lipid metabolism and some cytoskeleton proteins that reaffirms the importance of genetic factors in diabetic nephropathy.

  5. Diabetic Nephropathy without Diabetes

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    Katia López-Revuelta

    2015-07-01

    Full Text Available Diabetic nephropathy without diabetes (DNND, previously known as idiopathic nodular glomerulosclerosis, is an uncommon entity and thus rarely suspected; diagnosis is histological once diabetes is discarded. In this study we describe two new cases of DNND and review the literature. We analyzed all the individualized data of previous publications except one series of attached data. DNND appears to be favored by recognized cardiovascular risk factors. However, in contrast with diabetes, apparently no factor alone has been demonstrated to be sufficient to develop DNND. Other factors not considered as genetic and environmental factors could play a role or interact. The most plausible hypothesis for the occurrence of DNND would be a special form of atherosclerotic or metabolic glomerulopathy than can occur with or without diabetes. The clinical spectrum of cardiovascular risk factors and histological findings support this theory, with hypertension as one of the characteristic clinical features.

  6. Saliva and sera IgA and IgG in Egyptian Giardia-infected children.

    Science.gov (United States)

    El-Gebaly, Naglaa Saad M; Halawa, Eman Fawzy; Moussa, Hanaa M Ezzat; Rabia, Ibrahim; Abu-Zekry, Maha

    2012-08-01

    Giardiasis is a gastrointestinal infection of wide distribution that is more prevalent in childhood. Easy and rapid diagnosis of giardiasis is essential for reduction of this infection. This cross-sectional study included 62 children in which collection of saliva, stool and serum samples was performed. An enzyme-linked immunosorbent assay (ELISA) technique was evaluated to detect IgA and IgG responses in both saliva and serum samples. Twenty-two children were positive for Giardia duodenalis infection by direct examination of faecal specimens, 20 non-infected and 20 infected with other parasites. Salivary and serum IgA and IgG responses against G. duodenalis infection were significantly higher in Giardia parasitized than non-Giardia parasitized children (p < 0.001). This concludes that specific salivary IgA may serve as a diagnostic tool and specific salivary IgG as a screening tool in monitoring the exposure of various populations to Giardia duodenalis. The advantage of salivary assays over serum immunoglobulin assay is being easy and non-invasive in sampling technique which is important especially for young children.

  7. Refractory linear IgA bullous dermatosis successfully treated with mycophenolate sodium.

    Science.gov (United States)

    Marzano, Angelo V; Ramoni, Stefano; Spinelli, Diana; Alessi, Elvio; Berti, Emilio

    2008-01-01

    Linear IgA bullous dermatosis (LABD) is a rare, blistering autoimmune disease characterized by linear deposits of IgA at the basement membrane zone (BMZ), with the possible presence of circulating IgA anti-BMZ antibodies. LABD of childhood is usually self-healing, while in adults it follows a more prolonged course and refractory cases may rarely occur. The first-line treatment for LABD is dapsone in monotherapy or in combination with systemic corticosteroids, but various therapeutic approaches have been used in non-responder patients. We report two adult patients with refractory LABD successfully treated with enteric-coated mycophenolate sodium (EC-MPS), a recently introduced formulation of mycophenolic acid (MPA). MPA is an immunosuppressive agent that acts by inhibiting monophosphate dehydrogenase, a key enzyme in the novo synthesis of purines. Based on the present cases, we indicate EC-MPS as being a safe and effective adjuvant therapy in the treatment of LABD when dapsone or the other steroid-sparing drugs fail. It seems to offer an improved gastric side effect profile in comparison with the classic formulation of MPA, namely its ester mycophenolate mofetil (MMF).

  8. Evaluation of Children with IgA Nephropathy According to the Oxford Classification

    Directory of Open Access Journals (Sweden)

    Yel, Sibel

    2013-01-01

    Full Text Available OBJECTIVE: In this study, we aim to evaluate the clinical findings and short term results of our patients with IgAN according to Oxford classification.MATERIAL and METHODS: The medical records and the renal biopsy findings of 25 patients with IgAN were retrospectively reviewed. According to the pathological findings in Oxford classification, those without obvious disorder were accepted as Group 1 (n=16, and those with obvious disorders were accepted as Group 2 (n=9.These groups were compared for gender, age of onset, age of diagnosis, mean blood pressure and estimated glomerular filtration rate (GFR at diagnosis and follow-up, proteinuria, clinical presentation and prognosis.RESULTS: The most common complaint of the patients at presentation was hematuria. The most common histopathological renal finding was the proliferation of mesengial cells in varying degrees. Statistically no significant differences of mean blood pressure, GRF changes, protenuria, clinical findings, and prognosis were detected at presentation and follow-up.CONCLUSION: We could not show short-term effectiveness to predict clinical and laboratory findings, and prognosis of the patients of Oxford classification in children with IgAN.

  9. Clonal antibody dominance after influenza vaccination in IgA nephropathy patients and controls

    NARCIS (Netherlands)

    Radl, J.; Hoogeveen, C.M.; Wall Bake, A.W.L. van den; Mestecky, J.

    1995-01-01

    Chemicals/CAS: hemagglutinin, 37333-12-3; sialidase, 9001-67-6; Antibodies, Monoclonal; Antibodies, Viral; Hemagglutinins, Viral; Immunoglobulin A; Immunoglobulin G; Influenza Vaccine; Neuraminidase, EC 3.2.1.18

  10. [Novel biomarkers for diabetic nephropathy].

    Science.gov (United States)

    Araki, Shin-ichi

    2014-02-01

    Diabetic nephropathy is a leading cause of end-stage renal disease worldwide. An early clinical sign of this complication is an increase of urinary albumin excretion, called microalbuminuria, which is not only a predictor of the progression of nephropathy, but also an independent risk factor for cardiovascular disease. Although microalbuminuria is clinically important to assess the prognosis of diabetic patients, it may be insufficient as an early and specific biomarker of diabetic nephropathy because of a large day-to-day variation and lack of a good correlation of microalbuminuria with renal dysfunction and pathohistological changes. Thus, more sensitive and specific biomarkers are needed to improve the diagnostic capability of identifying patients at high risk. The factors involved in renal tubulo-interstitial damage, the production and degradation of extracellular matrix, microinflammation, etc., are investigated as candidate molecules. Despite numerous efforts so far, the assessment of these biomarkers is still a subject of ongoing investigations. Recently, a variety of omics and quantitative techniques in systems biology are rapidly emerging in the field of biomarker discovery, including proteomics, transcriptomics, and metabolomics, and they have been applied to search for novel putative biomarkers of diabetic nephropathy. Novel biomarkers or their combination with microalbuminuria provide a better diagnostic accuracy than microalbuminuria alone, and may be useful for establishing personal medicine. Furthermore, the identification of novel biomarkers may provide insight into the mechanisms underlying diabetic nephropathy.

  11. Release from Th1-type immune tolerance in spleen and enhanced production of IL-5 in Peyer's patch by cholera toxin B induce the glomerular deposition of IgA.

    Science.gov (United States)

    Yamanaka, Takahiro; Tamauchi, Hidekazu; Suzuki, Yusuke; Suzuki, Hitoshi; Horikoshi, Satoshi; Terashima, Masazumi; Iwabuchi, Kazuya; Habu, Sonoko; Okumura, Ko; Tomino, Yasuhiko

    2016-04-01

    We examined the pathogenesis of glomerular damage in Th2 type-dependent GATA-3 transgenic (GATA-3 Tg) mice with IgA nephropathy (IgAN). GATA-3 Tg mice were immunized orally using OVA plus cholera toxin B (CTB), and measurement of the serum IgA antibody level and histopathological examination were performed. Marked increases in the serum levels of OVA-specific IgA antibody, IgA and IgG, C3 deposits analogous to those seen in IgAN, and expansion of the matrix in association with mesangial cell proliferation were observed. Furthermore, glomerular IgA deposits were co-localized with mannan-binding lectin (MBL) deposits, which might actually have been abnormal IgA deposits. In GATA-3/TCR-Tg mice that had been orally sensitized with CTB plus OVA and were re-stimulated with OVA in vitro, cultured Peyer's patch cells showed the enhanced production of IL-5 and supernatants from cultures of spleen cells showed a reduction of TGF-β production with a simultaneous increase in IL-2 production and the recovery of IFN-γ formation. The amount of TGF-β produced by the spleen cells was found to be correlated with the amount of IFN-γ and IL-IL-2 produced by the cells. Also, the percentage of regulatory T cells (Treg) in the spleens of mice sensitized with OVA plus CTB was lower than that in mice orally sensitized with OVA alone. These results suggest that the increased production of IL-5 from Peyer's patch cells (PPc) and the restored Th1-type immune response might cause the production of abnormal IgA and might induce the deposition of IgA in glomeruli.

  12. Cleavage of a recombinant human immunoglobulin A2 (IgA2)-IgA1 hybrid antibody by certain bacterial IgA1 proteases

    DEFF Research Database (Denmark)

    Senior, B; Dunlop, JI; Batten, MR;

    2000-01-01

    , Streptococcus pneumoniae, S. sanguis, Neisseria meningitidis types 1 and 2, N. gonorrhoeae types 1 and 2, and Haemophilus influenzae type 2. Thus, for these enzymes the recognition site for IgA1 cleavage is contained within half of the IgA1 hinge region; additional distal elements, if required, are provided...

  13. Non-Proteinuric Diabetic Nephropathy

    Science.gov (United States)

    Robles, Nicolas Roberto; Villa, Juan; Hernandez Gallego, Roman

    2015-01-01

    Diabetic nephropathy patients traditionally show significant macroalbuminuria prior to the development of renal impairment. However, this clinical paradigm has recently been questioned. Epidemiological surveys confirm that chronic kidney disease (CKD) diagnosed by a low glomerular filtration rate (GFR) is more common in diabetic patients than in the non-diabetic population but a low number of patients had levels of proteinuria above that which traditionally defines overt diabetic nephropathy (>500 mg/g). The large number of patients with low levels of proteinuria suggests that the traditional clinical paradigm of overt diabetic nephropathy is changing since it does not seem to be the underlying renal lesion in most of diabetic subjects with CKD. PMID:26371050

  14. TCM Differential Treatment of IgA Nephrosis

    Institute of Scientific and Technical Information of China (English)

    聂莉芳

    2005-01-01

    @@ IgA nephrosis, with an incidence of 38%-49% in the primary glomerulopathy in China, is one of the main causes for the late renal failure. In view of the fact that there are no specific Western remedies for IgA nephrosis characterized by hematuria, the researches on TCM differential treatment of IgA nephrosis have been listed in the major projects of "the Scientific Technologies in thel0th 5-year plan of China".

  15. Interstitial nephritis and interstital nephropathy

    Institute of Scientific and Technical Information of China (English)

    1993-01-01

    930154 Plasma thromboxane B2 and 6-keto-prostaglandin F1 alpha in diabetic nephropathyWANG Yao(王尧),et al,Dept Endocrinol,1stAffil Hosp,Nanjihng Railway Med Coll.210009.Chin J Nephrol 1992;8(5):275-277.The plasma levels of TXB2 and 6-keto-PGF1were measured by RIA in 14 diabetics with and49 diabetics without nephropathy and 35 normalsubjects.The results showed that the levels ofplasma TXB2 and the ratio of TXB2/6-keto-PGF.were increased both in diabetics with or

  16. Oral manifestations caused by the linear IgA disease.

    Science.gov (United States)

    Eguia del Valle, Asier; Aguirre Urízar, José Manuel; Martínez Sahuquillo, Angel

    2004-01-01

    The Linear IgA deposit related disease or Linear IgA disease (LAD) is a chronic, uncommon and autoimmunological mucocutaneous disease, characterised by linear IgA deposits along the basement membrane zone. In mainly cases, moreover cutaneous lesions, there are oral mucosal and other mucosal lesions. There are also, some cases published of Linear IgA disease limited to oral mucosa. The known of this disease is important for the establishment of a correct differential diagnosis in cases of blistering mucocutaneous diseases. In this paper, we analyze the most important features of this disease, attending specially to the oral manifestations.

  17. Podocyte Pathology and Nephropathy

    Directory of Open Access Journals (Sweden)

    Sandra eMerscher

    2014-07-01

    Full Text Available Sphingolipids are components of the lipid rafts in plasma membranes, which are important for proper function of podocytes, a key element of the glomerular filtration barrier. Research revealed an essential role of sphingolipids and sphingolipid metabolites in glomerular disorders of genetic and non-genetic origin. The discovery that glucocerebrosides accumulate in Gaucher disease in glomerular cells and are associated with clinical proteinuria initiated intensive research into the function of other sphingolipids in glomerular disorders. The accumulation of sphingolipids in other genetic diseases including Tay-Sachs, Sandhoff, Fabry, hereditary inclusion body myopathy 2, Niemann-Pick and nephrotic syndrome of the Finnish type and its implications with respect to glomerular pathology will be discussed. Similarily, sphingolipid accumulation occurs in glomerular diseases of non-genetic origin including diabetic kidney disease (DKD, HIV-associated nephropathy, focal segmental glomerulosclerosis (FSGS and lupus nephritis. Sphingomyelin metabolites, such as ceramide, sphingosine and sphingosine-1-phosphate have also gained tremendous interest. We recently described that sphingomyelin phosphodiesterase acid-like 3b (SMPDL3b is expressed in podocytes where it modulates acid sphingomyelinase (ASMase activity and acts as a master modulator of danger signaling. Decreased SMPDL3b expression in post-reperfusion kidney biopsies from transplant recipients with idiopathic FSGS correlates with the recurrence of proteinuria in patients and in experimental models of xenotransplantation. Increased SMPDL3b expression is associated with DKD. The consequences of differential SMPDL3b expression in podocytes in these diseases with respect to their pathogenesis will be discussed. Finally, the role of sphingolipids in the formation of lipid rafts in podocytes and their contribution to the maintenance of a functional slit diaphragm in the glomerulus will be discussed.

  18. IMMUNOGLOBULIN A (IgA AND ITS RECEPTORS

    Directory of Open Access Journals (Sweden)

    V. B. Klimovich

    2006-01-01

    Full Text Available Abstract. Daily IgA production in human organism comprises 3 to 5 g, thus exceeding total synthesis of other Ig classes. IgA in human body is presented by 9 structural variants. Its molecules belong to two subclasses, IgA1 and IgA2, the latter represented by two allotypes. In human serum, IgA1 monomers predominate, that are produced by the bone marrow cells. Mucosa-associated lymphoid tissues produce dimeric IgA1 and IgA2 molecules containing an accessory polypeptide J-chain. When transported across epithelial layer to the mucosal surface, an extracellular segment of polymeric IgA receptor (pIgAR is joining the dimeric IgA1, which becomes a ‘secretory’ component being a part cesretory IgA (sIgA molecule. The main function of sIgA is to bind bacteria and viruses at the mucosal surfaces, thus preventing pathogens to invade the internal spaces of the organism (immune exclusion. If transferred across epithelium, IgA may neutralize the viruses penetrating the cells, like as bind and deliver proteins and other antigens to the mucosal surface. The leukocyte IgA receptor (FcαRI, CD89 is expressed on the neutrophils, eosinophiles, monocytes/macrophages, as well as dendritic and Kupffer cells. The cytoplasmic domain FcαRI is devoid of an activation ITAM motif. To transduce signal, an FcαRI-associated chain of Fcγ receptor is used. Due to this mechanism, IgA binding leads to activation of phagocytosis, endocytosis, antigen presentation, synthesis of proinflammatory mediator and other immune functions. Fcα/μR receptor is a structural homologue of pIgR, and it is able to bind IgA and IgM, being, however, expressed only at the surface of mature B lymphocytes and macrophages. Interaction of IgA with asialoglycoprotein and transferrin (CD71 receptors, like as with some other molecules, that have yet undetermined role in immune defense and development of pathological events.

  19. Lithium nephropathy: a case report

    OpenAIRE

    Raphael Reis Pereira-Silva; Debora Esperancini-Tebar

    2014-01-01

    Although widely used in the management of bipolar disorder, lithium may cause adverse kidney effects. The importance of the present study is to report the case of a 59-year-old woman who was under regular treatment with lithium for bipolar disorder and whose imaging studies demonstrated the presence of multiple renal microcysts, suggesting lithium nephropathy as main diagnostic hypothesis.

  20. Lithium nephropathy: a case report

    Directory of Open Access Journals (Sweden)

    Raphael Reis Pereira-Silva

    2014-01-01

    Full Text Available Although widely used in the management of bipolar disorder, lithium may cause adverse kidney effects. The importance of the present study is to report the case of a 59-year-old woman who was under regular treatment with lithium for bipolar disorder and whose imaging studies demonstrated the presence of multiple renal microcysts, suggesting lithium nephropathy as main diagnostic hypothesis.

  1. Linear IgA bullous dermatosis following influenza vaccination.

    Science.gov (United States)

    Alberta-Wszolek, Lauren; Mousette, Alyse M; Mahalingam, Meera; Levin, Nikki A

    2009-11-15

    Linear IgA Bullous Dermatosis (LABD) is an immune-mediated subepidermal vesiculobullous eruption characterized by linear deposits of IgA at the basement membrane zone. Most cases are idiopathic but medications, infections, and malignancies have also been reported to induce LABD. We report the case of a 54-year-old woman who developed LABD shortly after receiving an influenza vaccination.

  2. Diabetic nephropathy: a national dialogue.

    Science.gov (United States)

    Breyer, Matthew D; Coffman, Thomas M; Flessner, Michael F; Fried, Linda F; Harris, Raymond C; Ketchum, Christian J; Kretzler, Matthias; Nelson, Robert G; Sedor, John R; Susztak, Katalin

    2013-09-01

    The National Institute of Diabetes and Digestive and Kidney Diseases-supported Kidney Research National Dialogue (KRND) asked the scientific community to formulate and prioritize research objectives that would improve our understanding of kidney function and disease. Several high-priority objectives for diabetic nephropathy were identified in data and sample collection, hypothesis generation, hypothesis testing, and translation promotion. The lack of readily available human samples linked to comprehensive phenotypic, clinical, and demographic data remains a significant obstacle. With data and biological samples in place, several possibilities exist for using new technologies to develop hypotheses. Testing novel disease mechanisms with state-of-the-art tools should continue to be the foundation of the investigative community. Research must be translated to improve diagnosis and treatment of people. The objectives identified by the KRND provide the research community with future opportunities for improving the prevention, diagnosis, and treatment of diabetic nephropathy.

  3. Lithium clearance in chronic nephropathy

    DEFF Research Database (Denmark)

    Kamper, A L; Holstein-Rathlou, N H; Leyssac, P P

    1989-01-01

    1. Lithium clearance measurements were made in 72 patients with chronic nephropathy of different aetiology and moderate to severely reduced renal function. 2. Lithium clearance was strictly correlated with glomerular filtration rate, and there was no suggestion of distal tubular reabsorption...... clearance data were independent of whether renal disease was of primarily glomerular or tubular origin and, further, were not influenced by long-term conventional antihypertensive treatment. 6. It is concluded that, even with a reduced kidney function, the data are compatible with the suggestion...... that lithium clearance may be a measure of the delivery of sodium and water from the renal proximal tubule. With this assumption it was found that adjustment of the sodium excretion in chronic nephropathy initially takes place in the distal parts of the nephron (loop of Henle, distal tubule and collecting duct...

  4. Lithium clearance in chronic nephropathy

    DEFF Research Database (Denmark)

    Kamper, A L; Holstein-Rathlou, N H; Leyssac, P P

    1989-01-01

    1. Lithium clearance measurements were made in 72 patients with chronic nephropathy of different aetiology and moderate to severely reduced renal function. 2. Lithium clearance was strictly correlated with glomerular filtration rate, and there was no suggestion of distal tubular reabsorption...... of lithium or influence of osmotic diuresis. 3. Fractional reabsorption of lithium was reduced in most patients with glomerular filtration rates below 25 ml/min. 4. Calculated fractional distal reabsorption of sodium was reduced in most patients with glomerular filtration rates below 50 ml/min. 5. Lithium...... that lithium clearance may be a measure of the delivery of sodium and water from the renal proximal tubule. With this assumption it was found that adjustment of the sodium excretion in chronic nephropathy initially takes place in the distal parts of the nephron (loop of Henle, distal tubule and collecting duct...

  5. HIV Associated Lupus Like Nephropathy

    OpenAIRE

    2014-01-01

    Background Human immunodeficiency virus type 1 (HIV-1)-seropositive patients are at a high risk for the development of a variety of acute and chronic renal diseases. Most patients with HIVAN are of African descent, presenting late in the course of their HIV-1 infection. The only reliable test to establish or rule out the presence of HIVAN (HIV associated nephropathy) is renal biopsy. The most common lesion associated with HIV is a focal segmental glomeruloscelerosis, but several times, other ...

  6. Phase Field Modeling Using PetIGA

    KAUST Repository

    Vignal, Philippe A.

    2013-06-01

    Phase field modeling has become a widely used framework in the computational material science community. Its ability to model different problems by defining appropriate phase field parameters and relating it to a free energy functional makes it highly versatile. Thermodynamically consistent partial differential equations can then be generated by assuming dissipative dynamics, and setting up the problem as one of minimizing this free energy. The equations are nonetheless challenging to solve, and having a highly efficient and parallel framework to solve them is necessary. In this work, a brief review on phase field models is given, followed by a short analysis of the Phase Field Crystal Model solved with Isogeometric Analysis us- ing PetIGA. We end with an introduction to a new modeling concept, where free energy functions are built with a periodic equilibrium structure in mind.

  7. Exposures influencing total IgA level in colostrum.

    Science.gov (United States)

    Munblit, D; Sheth, S; Abrol, P; Treneva, M; Peroni, D G; Chow, L-Y; Boner, A L; Pampura, A; Warner, J O; Boyle, R J

    2016-02-01

    Immunoglobulin A (IgA) is a predominant immunoglobulin present in human breast milk and is known to play an important role in infant gut immunity maturation. Breast milk composition varies between populations, but the environmental and maternal factors responsible for these variations are still unclear. We examined the relationship between different exposures and levels of IgA in colostrum. The objective of this study was to examine whether exposures analysed influence levels of IgA in colostrum. The present study used 294 colostrum samples from the MecMilk International cohort, collected from women residing in London, Moscow and Verona. Samples were analysed in automated Abbott Architect Analyser. We found an inverse correlation between time postpartum and colostrum total IgA level (r=-0.49, Pcolostrum collection time (Pcolostrum drops rapidly after birth and future studies should always consider this factor in analysis. IgA concentration varied significantly between countries, with the highest level detected in Moscow and lowest in Verona. Mode of delivery effect should be confirmed on larger cohorts. Further work is needed to determine ways to correct for IgA decline over time in colostrum, and to find the cause of variations in IgA levels between the countries.

  8. Application of Oxford classification, and overexpression of transforming growth factor-β1 and immunoglobulins in immunoglobulin A nephropathy: correlation with World Health Organization classification of immunoglobulin A nephropathy in a Chinese patient cohort.

    Science.gov (United States)

    Meng, Hongxue; Zhang, Lei; E, Xiaoqiang; Ye, Fei; Li, Huining; Han, Changsong; Yamakawa, Mitsunori; Jin, Xiaoming

    2014-01-01

    Immunoglobulin A nephropathy (IgAN) is characterized by the qualitative abnormality of immunoglobulin A (IgA) in circulation and deposits of IgA in the renal mesangium. Transforming growth factor β1 (TGF-β1) plays a key role in fibrogenesis and the progression of renal damage. This study aimed to investigate the clinicopathologic data on IgAN in northeastern China and the presence of TGF-β1, total IgA, and secretory IgA in the glomeruli and sera, as well as changes in galactose-deficient IgA1 in the serum. We investigated the clinicopathologic data of 1050 cases of IgAN diagnosed in a single center over 13 years. We then assessed the concentrations of TGF-β1 and immunoglobulins in the serum of 100 patients with IgAN and 56 healthy control subjects by enzyme-linked immunosorbent assay, and investigated their presence in the glomeruli by immunofluorescence and reverse transcriptase-polymerase chain reaction. From our data, 76.17% of the IgAN cases belonged to classes I and II according to the World Health Organization classification, representing the early stage. Compared with other studies, we found significantly lower frequencies of segmental glomerulosclerosis (27.71%) but higher frequencies of endocapillary proliferation (50.67%), and a similar proportion of mesangial hypercellularity (68.48%) and tubular atrophy/interstitial fibrosis (moderate, 17.81%; severe, 1.52%) in the northeastern Chinese cohort. There was an increased presence of TGF-β1 and immunoglobulins in the serum and glomeruli of IgAN, which correlates with the progression of pathologic classification. The pathologic variables of the Oxford classification correlated significantly with the WHO classifications. TGF-β1 and immunoglobulins could be used as biomarkers of IgAN pathogenic mechanisms, acting as important adjuncts to the original Oxford Classification.

  9. Do immunoglobulin A vasculitis and immunoglobulin A nephropathy belong to one desease%IgA血管炎和IgA肾病是同一种疾病吗?

    Institute of Scientific and Technical Information of China (English)

    吴小川; 廖娟

    2014-01-01

    IgA血管炎(IgA vasculitis,IgAV)与IgA肾病(IgA nephropathy,IgAN)免疫病理均显示为IgA沉积,且IgA1分子糖基化异常是其共同主要的发病机制.因此,究竟两者是不是同一种疾病,临床上一直存在争议.现就病因、流行病学、临床、病理、发病机制及预后等方面来分析二者异同.%The immunopathogenesis of IgA vasculitis and IgA nephropathy both demonstrate IgA deposition,and abnormal glycosylation of IgAl molecule is their major pathogenesis.Therefore,it is clinically controversial that they are actually one disease.The present text will delineate their similarities and differences from aspects of epidemiology,clinic,pathology,mechanism and prognosis.

  10. Association of genetic variants with diabetic nephropathy.

    Science.gov (United States)

    Rizvi, Saliha; Raza, Syed Tasleem; Mahdi, Farzana

    2014-12-15

    Diabetic nephropathy accounts for the most serious microvascular complication of diabetes mellitus. It is suggested that the prevalence of diabetic nephropathy will continue to increase in future posing a major challenge to the healthcare system resulting in increased morbidity and mortality. It occurs as a result of interaction between both genetic and environmental factors in individuals with both type 1 and type 2 diabetes. Genetic susceptibility has been proposed as an important factor for the development and progression of diabetic nephropathy, and various research efforts are being executed worldwide to identify the susceptibility gene for diabetic nephropathy. Numerous single nucleotide polymorphisms have been found in various genes giving rise to various gene variants which have been found to play a major role in genetic susceptibility to diabetic nephropathy. The risk of developing diabetic nephropathy is increased several times by inheriting risk alleles at susceptibility loci of various genes like ACE, IL, TNF-α, COL4A1, eNOS, SOD2, APOE, GLUT, etc. The identification of these genetic variants at a biomarker level could thus, allow the detection of those individuals at high risk for diabetic nephropathy which could thus help in the treatment, diagnosis and early prevention of the disease. The present review discusses about the various gene variants found till date to be associated with diabetic nephropathy.

  11. Association of genetic variants with diabetic nephropathy

    Institute of Scientific and Technical Information of China (English)

    Saliha; Rizvi; Syed; Tasleem; Raza; Farzana; Mahdi

    2014-01-01

    Diabetic nephropathy accounts for the most serious microvascular complication of diabetes mellitus. It is suggested that the prevalence of diabetic nephropathy will continue to increase in future posing a major challenge to the healthcare system resulting in increased morbidity and mortality. It occurs as a result of interaction between both genetic and environmental factors in individuals with both type 1 and type 2 diabetes. Genetic susceptibility has been proposed as an important factor for the development and progression of diabetic nephropathy, and various research efforts are being executed worldwide to identify the susceptibility gene for diabetic nephropathy. Numerous single nucleotide polymorphisms have been found in various genes giving rise to various gene variants which have been found to play a major role in genetic susceptibility to diabetic nephropathy. The risk of developing diabetic nephropathy is increased several times by inheriting risk alleles at susceptibility loci of various genes like ACE, IL, TNF-α, COL4A1, e NOS, SOD2, APOE, GLUT, etc. The identification of these genetic variants at a biomarker level could thus, allow the detection of those individuals at high risk for diabetic nephropathy which could thus help in the treatment, diagnosis and early prevention of the disease. The present review discusses about the various gene variants found till date to be associated with diabetic nephropathy.

  12. Nodular lesions and mesangiolysis in diabetic nephropathy.

    Science.gov (United States)

    Wada, Takashi; Shimizu, Miho; Yokoyama, Hitoshi; Iwata, Yasunori; Sakai, Yoshio; Kaneko, Shuichi; Furuichi, Kengo

    2013-02-01

    Diabetic nephropathy is a leading cause of end-stage renal failure all over the world. Advanced human diabetic nephropathy is characterized by the presence of specific lesions including nodular lesions, doughnut lesions, and exudative lesions. Thus far, animal models precisely mimicking advanced human diabetic nephropathy, especially nodular lesions, remain to be fully established. Animal models with spontaneous diabetic kidney diseases or with inducible kidney lesions may be useful for investigating the pathogenesis of diabetic nephropathy. Based on pathological features, we previously reported that diabetic glomerular nodular-like lesions were formed during the reconstruction process of mesangiolysis. Recently, we established nodular-like lesions resembling those seen in advanced human diabetic nephropathy through vascular endothelial injury and mesangiolysis by administration of monocrotaline. Here, in this review, we discuss diabetic nodular lesions and its animal models resembling human diabetic kidney lesions, with our hypothesis that endothelial cell injury and mesangiolysis might be required for nodular lesions.

  13. Linear IgA dermatosis induced by pregnancy

    OpenAIRE

    Telma Kanagusuko; Valéria Aoki; Antonio José Tebcherani; Ana Paula Galli Sanchez

    2008-01-01

    A dermatose por IgA linear é doença bolhosa auto-imune subepidérmica rara, caracterizada pelo depósito linear de IgA na zona da membrana basal da epiderme. Nos relatos de gestação em pacientes com essa dermatose, nota-se sempre melhora do quadro clínico. Contrariando essas observações,é apresentado caso de dermatose por IgA linear induzida pela gestação, que demonstrou boa resposta terapêutica à dapsona e prednisona , sem complicações materno-fetais.Linear IgA disease is a rare autoimmune sub...

  14. Generalized linear IgA dermatosis with palmar involvement

    OpenAIRE

    Norris, Ivy N; Haeberle, M Tye; Callen, Jeffrey P.; Malone, Janine C

    2015-01-01

    Linear IgA bullous dermatosis (LABD) is a sub-epidermal blistering disorder characterized by deposition of IgA along the basement membrane zone (BMZ) as detected by immunofluorescence microscopy. The diagnosis is made by clinicopathologic correlation with immunofluorescence confirmation. Differentiation from other bullous dermatoses is important because therapeutic measures differ. Prompt initiation of the appropriate therapies can have a major impact on outcomes. We present three cases with ...

  15. Extrinsic allergic alveolitis with IgA deficiency.

    Science.gov (United States)

    Sennekamp, J; Morr, H; Behr, J

    2004-12-22

    Up to now only 3 cases of extrinsic allergic alveolitis (hypersensitivity pneumonitis) with IgA deficiency have been published worldwide. We had the opportunity to detect two additional cases which will be presented here. Summarizing all cases IgA deficiency is a risk factor for a severe course of the disease and an increased susceptibility to acquire allergic alveolitis by low dose antigen exposure.

  16. Childhood Schizophrenia

    Science.gov (United States)

    Childhood schizophrenia Overview By Mayo Clinic Staff Childhood schizophrenia is an uncommon but severe mental disorder in which children interpret reality abnormally. Schizophrenia involves a range of problems with thinking (cognitive), ...

  17. Lithium nephropathy: unique sonographic findings.

    Science.gov (United States)

    Di Salvo, Donald N; Park, Joseph; Laing, Faye C

    2012-04-01

    This case series describes a unique sonographic appearance consisting of numerous microcysts and punctate echogenic foci seen on renal sonograms of 10 adult patients receiving chronic lithium therapy. Clinically, chronic renal insufficiency was present in 6 and nephrogenic diabetes insipidus in 2. Sonography showed numerous microcysts and punctate echogenic foci. Computed tomography in 5 patients confirmed microcysts and microcalcifications, which were fewer in number than on sonography. Magnetic resonance imaging in 2 patients confirmed microcysts in each case. Renal biopsy in 1 patient showed chronic interstitial nephritis, microcysts, and tubular dilatation. The diagnosis of lithium nephropathy should be considered when sonography shows these findings.

  18. Childhood obesity

    OpenAIRE

    Wilkinson, Justine; Howard, Simon

    2006-01-01

    Childhood obesity has important consequences for health and wellbeing both during childhood and also in later adult life. The rising prevalence of childhood obesity poses a major public health challenge in both developed and developing countries by increasing the burden of chronic non-communicable diseases. Despite the urgent need for effective preventative strategies, there remains disagreement over its definition due to a lack of evidence on the optimal cut-offs linking childhood BMI to dis...

  19. Amino acid sequence requirements in the human IgA1 hinge for cleavage by streptococcal IgA1 proteases

    DEFF Research Database (Denmark)

    Senior, BW; Batten, MR; Kilian, Mogens;

    2002-01-01

    All the IgA1 proteases of the different pathogenic species of Streptococcus cleave the hinge of the alpha chain of human IgA1 only at one proline-threonine peptide bond. In order to study the importance of these amino acids for cleavage, several hinge mutant recombinant IgA1 antibodies were...

  20. Recombinant human immunoglobulin (Ig)A1 and IgA2 anti-D used for detection of IgA deficiency and anti-IgA

    DEFF Research Database (Denmark)

    Nielsen, Leif K; Dziegiel, Morten Hanefeld

    2008-01-01

    To avoid anaphylactic reactions, immunoglobulin (Ig)A-deficient patients with anti-IgA should be transfused with IgA-deficient blood components. There is a need for fast and robust assays for demonstration of IgA deficiency and for detection of anti-IgA.......To avoid anaphylactic reactions, immunoglobulin (Ig)A-deficient patients with anti-IgA should be transfused with IgA-deficient blood components. There is a need for fast and robust assays for demonstration of IgA deficiency and for detection of anti-IgA....

  1. Sonographic findings in Gouty Nephropathy

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Mi Young; Jeon, Woo Ki; Kim, Ho Kyun; Kim, Yong Soo; Han, Chang Yul; Kim, Young Tong; Han, Sung Tag; Lee, Yoon Woo [Inje University College of Medicine, Seoul (Korea, Republic of)

    1994-09-15

    Ultrasound(US) findings of hyperechoic renal medulla in gouty nephropathy were compared with clinical features such as serum uric acid level to evaluate its usefulness in determination of the treatment and prognosis. A retrospective review of US of 36 cases of qouty arthritis was classified into four groups according to the medullary echogenicity (O :normal, grade I: renal medulla as isoechoic as renal cortex, grade II; heterogeneous increased echogenicity of renal medulla than that of renal cortex, grade III: the echogenicity of all renal medulla higher than that of renal cortex with renal contour deformity) which were compared with the serum urate level and associated conditions. Nephrocalcinosis and nephrolithiasis were analyzed through the KUB and the RGB. The degree of hyperechoic renal medulla was related to the level of serum uric acid, and in group IV, six cases of obstructive uropathy (nephrocalcinosis and nephrolithiasis) showed deformed renal contour. Associated conditions such as hypertension, alcoholism, diabetes mellitus and drug abuse were distributed in relation to the degree of hyperechoic renal medullas. US findings of hyperechoic renal mebulla was related with uric acid level in gouty nephropathy and thus could be valuable for treatment decision and prediction of prognosis.

  2. Comprehensive approach to diabetic nephropathy

    Directory of Open Access Journals (Sweden)

    Bancha Satirapoj

    2014-09-01

    Full Text Available Diabetic nephropathy (DN is a leading cause of mortality and morbidity in patients with diabetes. This complication reflects a complex pathophysiology, whereby various genetic and environmental factors determine susceptibility and progression to end-stage renal disease. DN should be considered in patients with type 1 diabetes for at least 10 years who have microalbuminuria and diabetic retinopathy, as well as in patients with type 1 or type 2 diabetes with macroalbuminuria in whom other causes for proteinuria are absent. DN may also present as a falling estimated glomerular filtration rate with albuminuria as a minor presenting feature, especially in patients taking renin–angiotensin–aldosterone system inhibitors (RAASi. The pathological characteristic features of disease are three major lesions: diffuse mesangial expansion, diffuse thickened glomerular basement membrane, and hyalinosis of arterioles. Functionally, however, the pathophysiology is reflected in dysfunction of the mesangium, the glomerular capillary wall, the tubulointerstitium, and the vasculature. For all diabetic patients, a comprehensive approach to management including glycemic and hypertensive control with RAASi combined with lipid control, dietary salt restriction, lowering of protein intake, increased physical activity, weight reduction, and smoking cessation can reduce the rate of progression of nephropathy and minimize the risk for cardiovascular events. This review focuses on the latest published data dealing with the mechanisms, diagnosis, and current treatment of DN.

  3. Clinical and pathological aspects of analgesic nephropathy

    OpenAIRE

    Nanra, R. S.

    1980-01-01

    1 Analgesic nephropathy is part of the analgesic syndrome which has gastrointestinal, haematological, cardiovascular, psychological and psychiatric, and pregnancy and gonadal manifestations; premature ageing may also be a feature.

  4. The course of incipient diabetic nephropathy

    DEFF Research Database (Denmark)

    Christensen, Cramer; Mogensen, C E

    1985-01-01

    With the aim of defining the transitional phase from normal or near normal albumin excretion to overt diabetic nephropathy, 23 male diabetics of more than 7 years' duration, below 40 years of age and a baseline urinary albumin excretion above 15 micrograms/min but without clinical proteinuria...... (incipient diabetic nephropathy) were studied. For comparison 18 normals, 23 diabetics with normal albumin excretion and 10 patients with overt nephropathy were also examined. Diastolic blood pressure (DBP) was elevated to 88 +/- 9 mmHg (mean +/- S.D.) compared to patients with normal urinary albumin...... excretion: 80 +/- 7 (S.D.) (2p = 0.13%) but was below pressures in patients with overt diabetic nephropathy 109 +/- 15 (2p = 0.002%). Glomerular filtration rate (GFR) was elevated to 142 +/- 21 ml/min (mean +/- S.D.) compared to 132 +/- 9 in patients with normal urinary albumin excretion (2p = 4.3%). Renal...

  5. Mechanism of hypertension in diabetic nephropathy

    OpenAIRE

    Nazar, Chaudhary Muhammad Junaid

    2014-01-01

    High prevalence of hypertension is observed in diabetic patients of both the types. Diabetic nephropathy is one of the major reason for high morbidity, mortality and financial burden in such hypertensive diabetic patients. For this review, electronic databases including PubMed/Medline, Embase, Cochrane and Google scholar were searched from 1990-2013. Multiple inter-related factors are responsible for the development of hypertension and therefore nephropathy in the chronic diabetic patients. M...

  6. Clinical validation of immunoglobulin A nephropathy diagnosis in Swedish biopsy registers

    Directory of Open Access Journals (Sweden)

    Jarrick S

    2017-01-01

    Full Text Available Simon Jarrick,1,2 Sigrid Lundberg,3,4 Adina Welander,5,6 C Michael Fored,6 Jonas F Ludvigsson2,7,8 1Department of Pediatrics, Faculty of Health and Medicine, Örebro University, 2Department of Pediatrics, Örebro University Hospital, Örebro, 3Department of Nephrology, Karolinska University Hospital, 4Department of Clinical Science, Intervention and Technology, Karolinska Institutet, 5Boston Consulting Group, 6Clinical Epidemiology Unit, Department of Medicine, 7Department of Medical Epidemiology and Biostatistics, Karolinska Institutet, Stockholm, Sweden; 8Division of Epidemiology and Public Health, School of Medicine, University of Nottingham, Nottingham, UK Aims: The aims of this study were to validate the diagnosis of IgA nephropathy (IgAN in Swedish biopsy registers against patient charts and to describe the clinical characteristics of patients with a biopsy indicating IgAN. Methods: This is a population-based cohort study. Out of 4,069 individuals with a renal biopsy consistent with IgAN (biopsies performed in 1974–2011, this study reviewed patient charts of a random subset of 127 individuals. Clinical and biopsy characteristics at the time of biopsy were evaluated, and positive predictive values (PPV were calculated with 95% confidence intervals (CI. Results: Out of 127 individuals with a renal biopsy consistent with IgAN, 121 had a likely or confirmed clinical diagnosis of IgAN, primary or secondary to Henoch–Schönlein purpura, yielding a PPV of 95% (95% CI =92%–99%. The median age at biopsy was 39 years (range: 4–79 years; seven patients (6% were <16 years. The male to female ratio was 2.8:1. The most common causes for consultation were macroscopic hematuria (n=37; 29%, screening (n=33; 26%, and purpura (n=14, 11%. In patients with available data, the median creatinine level was 104 µmol/L (range 26–986 µmol/L, n=110 and glomerular filtration rate 75 mL/min/1.73m² (range 5–173 mL/min/1.73m², n=114. Hypertension was

  7. BIOCHEMICAL SCREENING OF DIABETIC NEPHROPATHY

    Directory of Open Access Journals (Sweden)

    Vivek

    2016-01-01

    Full Text Available Diabetic nephropathy is a clinical syndrome characterized by the following- Persistent albuminuria (>300mg/d or >200μg/min, that is confirmed on at least 2 occasions 3-6 months apart diabetic, progressive decline in the Glomerular Filtration Rate (GFR, elevated arterial blood pressure. The earliest biochemical criteria for the diagnosis of diabetic nephropathy is the presence of micro-albumin in the urine, which if left untreated will eventually lead to End-Stage Renal Disease (ESRD. Micro-albuminuria refers to the excretion of albumin in the urine at a rate that exceeds normal limits. The current study was conducted to establish the prevalence of micro-albuminuria in a sequential sample of diabetic patients attending hospital and OPD Clinic to determine its relationship with known and putative risk factors to identify micro- and normo-albuminuric patients in their sample for subsequent comparison in different age, sex, weight and creatinine clearance of the micro- and normo-albuminuric patients. This cross-sectional analytical study was conducted in one hundred patients at Saraswathi Institute of Medical Sciences, Anwarpur, Hapur, U. P. Patients having diabetes mellitus in different age group ranging from 30 to 70 years were selected. Data was analysed by SPSS software. Micro-albuminuria was observed in 35% in patients with type 2 diabetes mellitus. It was observed that 65% patients were free from any type of albuminuria. Also micro-albuminuria was present in 10% of the patients less than 50 yrs. of age, while 15% of the patients more than 50 yrs. of age were having micro-albuminuria. There was a statistically significant correlation of micro-albuminuria with duration of diabetes. Incidence of micro-albuminuria increases with age as well as increased duration of diabetes mellitus. Our study shows that only 5% patients developed macro-albuminuria. Glycosylated haemoglobin and fasting plasma glucose was significantly raised among all these

  8. Amadori albumin in diabetic nephropathy

    Directory of Open Access Journals (Sweden)

    Km. Neelofar

    2015-01-01

    Full Text Available Nonenzymatic glycation of macromolecules in diabetes mellitus (DM is accelerated due to persistent hyperglycemia. Reducing sugar such as glucose reacts non enzymatically with free €- amino groups of proteins through series of reactions forming Schiff bases. These bases are converted into Amadori product and further into AGEs. Non enzymatic glycation has the potential to alter the biological, structural and functional properties of macromolecules both in vitro and in vivo. Studies have suggested that amadori as well as AGEs are involved in the micro-macro vascular complications in DM, but most studies have focused on the role of AGEs in vascular complications of diabetes. Recently putative AGE-induced patho-physiology has shifted attention from the possible role of amadori-modified proteins, the predominant form of the glycated proteins in the development of the diabetic complications. Human serum albumin (HSA, the most abundant protein in circulation contains 59 lysine and 23 arginine residues that could, in theory be involved in glycation. Albumin has dual nature, first as a marker of intermediate glycation and second as a causative agent of the damage of tissues. Among the blood proteins, hemoglobin and albumin are the most common proteins that are glycated. HSA with a shorter half life than RBC, appears to be an alternative marker of glycemic control as it can indicate blood glucose status over a short period (2-3 weeks and being unaffected by RBCs life span and variant haemoglobin, anemia etc which however, affect HbA1c. On the other hand, Amadori albumin may accumulate in the body tissues of the diabetic patients and participate in secondary complications. Amadori-albumin has potential role in diabetic glomerulosclerosis due to long term hyperglycaemia and plays an important role in the pathogenesis of diabetic nephropathy. This review is an approach to compile both the nature of glycated albumin as a damaging agent of tissues and as an

  9. Tubulointerstitial disease in diabetic nephropathy

    Directory of Open Access Journals (Sweden)

    Tonolo G

    2014-03-01

    Full Text Available Giancarlo Tonolo, Sara Cherchi SC Diabetologia Aziendale ASL 2 Olbia, Hospital San Giovanni di Dio, Olbia, Italy Abstract: Diabetes mellitus is the major cause of end stage renal disease (ESRD. We cannot predict which patient will be affected. ESRD patients suffer an extremely high mortality rate, due to a very high incidence of cardiovascular disease. Several randomized, prospective studies have been conducted to quantify the impact of strict glycemic control on morbidity and mortality, and have consistently demonstrated an association between strict glycemic control and a reduction in ESRD. Within the past 20 years, despite the implementation of treatments that were presumed to be renoprotective, diabetes mellitus has continued to rank as the leading cause of ESRD, which clearly indicates that we are still far from understanding the mechanisms involved in the initiation of ESRD. Progressive albuminuria has been considered as the sine qua non of diabetic nephropathy, but we know now that progression to diabetic nephropathy may well happen in the absence of initial microalbuminuria. The search for new biomarkers of early kidney damage has received increasing interest, since early identification of the pathways leading to kidney damage may allow us to adopt measures to prevent the development of ESRD. Most of these biomarkers are deeply influenced by environment, genetics, sex differences, and so on, making it extremely difficult to identify the ideal biomarker to target. At present, there are no new drugs that come close to providing the solutions we desire for our patients (ie, reducing complications. Even when used in combination with standard care, renal complications are, at best, only modestly reduced, at the considerable expense of additional pill burden and exposure to serious off-target effects. In this review, some of the hypothesized mechanisms of this heterogeneous disease will be considered, with particular attention to the tubule

  10. Characterisation of kidney lesions in early schistosomal-specific nephropathy.

    Science.gov (United States)

    Sobh, M A; Moustafa, F E; Sally, S M; Deelder, A M; Ghoniem, M A

    1988-01-01

    In this work 42 patients with active Schistosoma mansoni infection and renal involvement were examined. Of these, 16 had asymptomatic proteinuria (group I) and 26 had the nephrotic syndrome (group II). Fifteen nonschistosomal patients with idiopathic nephrotic syndrome were included as control cases (group III). Renal biopsy specimens were obtained from all patients and controls. These were examined by light microscopy (LM), by direct immunofluorescence microscopy using antisera against human IgG, IgM, IgA, C3, C4, C1q, and fibrinogen, and by indirect immunofluorescence microscopy using monoclonal antibodies directed against the circulating schistosome antigens, circulating anodic antigen (CCA) and circulating cathodic antigen (CCA). Schistosomal-specific deposits were seen in the renal glomeruli in 24 of the 42 schistosomal patients but in none of the 15 control patients. Although schistosomal-specific deposits were seen in seven of the 16 patients presenting with asymptomatic proteinuria, no morphological changes could be seen by LM. On the other hand, schistosomal-specific deposits could be seen in the kidneys of 17 of the 26 patients presenting with the nephrotic syndrome. All but one specimen showed morphological changes when examined by LM. These were consistent with mesangioproliferative glomerulonephritis in seven, focal segmental glomerulosclerosis in five, mesangiocapillary glomerulonephritis in two, membranous glomerulonephritis in one, and focal segmental hyalinosis in one patient. The present study clearly suggests that (a) schistosomal-specific nephropathy does exist in human settings, (b) it is an immune complex disease, and (c) CAA and CCA are major responsible antigens.

  11. Working mechanism of immunoglobulin A1 (IgA1) protease: cleavage of IgA1 antibody to Neisseria meningitidis PorA requires de novo synthesis of IgA1 Protease

    DEFF Research Database (Denmark)

    Vidarsson, G; Overbeeke, N; Stemerding, AM

    2005-01-01

    Neisseria meningitidis secretes a protease that specifically cleaves the hinge region of immunoglobulin A1 (IgA1), releasing the effector (Fc) domain of IgA1 from the antigen binding (Fab) determinants. Theoretically, the remaining Fab fragments can block pathogen receptors or toxins and still....... Experiments with protein synthesis inhibitors showed that de novo production of IgA1 protease was responsible for cleavage of PorA-bound IgA1 on encapsulated bacteria. Finally, our data suggest that cleavage of IgA1 by IgA1 protease releases a significant proportion of Fab fragments from the bacterium......, probably as a result of their reduced avidity compared to that of whole antibodies....

  12. Generalized linear IgA dermatosis with palmar involvement.

    Science.gov (United States)

    Norris, Ivy N; Haeberle, M Tye; Callen, Jeffrey P; Malone, Janine C

    2015-09-17

    Linear IgA bullous dermatosis (LABD) is a sub-epidermal blistering disorder characterized by deposition of IgA along the basement membrane zone (BMZ) as detected by immunofluorescence microscopy. The diagnosis is made by clinicopathologic correlation with immunofluorescence confirmation. Differentiation from other bullous dermatoses is important because therapeutic measures differ. Prompt initiation of the appropriate therapies can have a major impact on outcomes. We present three cases with prominent palmar involvement to alert the clinician of this potential physical exam finding and to consider LABD in the right context.

  13. [Linear IgA bullous dermatosis of children].

    Science.gov (United States)

    Pierchalla, A; Bruch-Gerharz, D; Homey, B; Reifenberger, J

    2011-04-01

    Linear IgA bullous dermatosis is an acquired autoimmune subepidermal blistering disease, characterized by linear IgA deposits at the basement membrane zone. Described in both children and adults, it occurs as tense pruritic vesicles and bullae in a "cluster of jewels" configuration with central crusting on an inflammatory elevated base. It is typically located on the face, anogenital region and trunk. Whilst the adult manifestations can be chronic, in children a spontaneous remission has often been reported. Our patient showed a spontaneous remission after 8 weeks of symptomatic topic treatment with methylprednisolone and oral cetirizine dihydrochloride.

  14. Isogeometric Analysis of Hyperelastic Materials Using PetIGA

    KAUST Repository

    Bernal, L.M.

    2013-06-01

    In this work different nonlinear hyperelastic models for slightly compressible materials are implemented in an isogeometric finite element model. This is done within the recently developed computational framework called PetIGA, which uses isoge- ometric analysis and modern computational tools to solve systems of equations directly and iteratively. A flexible theoretical background is described to implement other hyperelastic models and possibly transient problems in future work. Results show quadratic convergence of the nonlinear solution consistent with the Newton-Raphson method that was used. Finally, PetIGA proves to be a powerful and versatile tool to solve these types of problems efficiently.

  15. Linear IgA dermatosis, coeliac disease, and extraintestinal B cell lymphoma.

    OpenAIRE

    Kapur, A.; Isaacs, P E; Kelsey, P R

    1995-01-01

    Linear IgA dermatosis is a malignancy associated rare bullous disorder similar to dermatitis herpetiformis. Linear IgA dermatosis differs from dermatitis herpetiformis in that the IgA deposits in the epidermal basement membrane are linear rather than granular. A patient is presented with coeliac disease who presented with linear IgA dermatosis and anaemia caused by chronic low grade B cell lymphoma.

  16. A Case of BK Nephropathy without Detectable Viremia or Viruria

    OpenAIRE

    Kamel, Mahmoud; Kadian, Manish; Salazar, Maria Nieva; Mohan, Prince; Self, Sally; Srinivas, Titte; Salas, Maria Aurora Posadas

    2015-01-01

    Patient: Male, 49 Final Diagnosis: BK nephropathy without detectable viremia or viruria Symptoms: — Medication: — Clinical Procedure: Kidney biopsy Specialty: Nephrology Objective: Unusual clinical course Background: BK nephropathy is an evolving challenge among kidney transplant recipients. Diagnosis of BK nephropathy depends on the presence of BK viral inclusions on renal biopsy. Most cases of BK nephropathy are preceded by BK viremia or viruria. Case Report: We report a case of BK nephropa...

  17. Drug-induced linear IgA bullous dermatosis.

    Science.gov (United States)

    Navi, Daniel; Michael, Daniel J; Fazel, Nasim

    2006-09-08

    A 73-year-old man was admitted to the University of California Davis Medical Center for treatment of a pleural effusion and congestive heart failure. His hospital course was complicated by asymptomatic sustained ventricular tachycardia requiring placement of an implantable cardiac defibrillator. The patient was treated with vancomycin and cefazolin during the procedure. After 3 days he developed tense vesicles over the dorsal aspect of the hands. Perilesional skin biopsy showed subepidermal cleavage with a neutrophilic infiltrate. Direct immunofluorescence revealed granular IgA and C3 deposition along the dermal epidermal junction. A diagnosis of drug-induced linear IgA bullous dermatosis secondary to vancomycin was established. Linear IgA bullous dermatosis is a rare autoimmune blistering disorder with clinical features that can overlap with bullous pemphigoid and dermatitis herpetiformis. Drug-induced linear IgA bullous dermatosis is a less common variant that is correspondingly less well characterized. Although a variety of medications have been implicated, vancomycin is the most common associated drug.

  18. Streikijate armee paisub iga päevaga / Aivar Reinap

    Index Scriptorium Estoniae

    Reinap, Aivar, 1968-

    2004-01-01

    23. septembril alanud raudteetöötajate streigist osavõtjate arv kasvab iga päevaga, kaubarongid veel liiguvad, kuid kui vajalikke samme ei astuta, võib kogu rongiliiklus seiskuda. Lisa: Ronge asendavad bussid. Vt. ka: Edelaraudtee rendib streigi ajaks bussid

  19. Abortide arv kahaneb Eestis iga aastaga / Rebekka Lotman

    Index Scriptorium Estoniae

    Lotman, Rebekka, 1978-

    2005-01-01

    Vt. ka Zdorovje dlja Vsehh 2005 märts, lk. 6. Kuigi abortide arv väheneb Eestis iga aastaga, tuleb naistearstide hinnangul oodata veel kümmekond aastat, et see langeks sama madalale tasemele nagu Põhjamaades. Lisaks diagramm abortide arvu kohta 1991-2004

  20. Selective IgA Deficiency and Common Variable Immunodeficiency

    Directory of Open Access Journals (Sweden)

    Kadri Kamber

    2009-09-01

    Full Text Available Selective IgA deficiency (sIgAD, using 5 mg/dl of serum IgA as the upper limit for diagnosis and concomitant lack of secretory IgA, is the most common form of primary immunodeficiency. The pathogenesis of IgA deficiency is not known, although abnormalities in Ig class switching and the cytokines involved in isotype switching have been implicated. Common Variable Immunodeficiency (CVID is a heterogenous group of B cell deficiency syndromes characterized by hypogammaglobulinemia, impaired antibody production and recurrent bacterial infections. Defective T-cell activation may lead to an impairment in cognate T-B-cell interaction due to impaired expression of CD40 ligand and/or abnormalities in the production of T-cell-derived cytokines required for fully functional B-cell activation, proliferation and/or differentiation which could indeed explain the impairment in antibody production present in CVID patients. It has been found that cytokines are produced in low levels due to the decreased T cell function which occurs as a result of the defect in CD40L expression in CVID patients. (Journal of Current Pediatrics 2009; 7: 90-5

  1. A new Classification of Diabetic Nephropathy 2014: a report from Joint Committee on Diabetic Nephropathy.

    Science.gov (United States)

    Haneda, Masakazu; Utsunomiya, Kazunori; Koya, Daisuke; Babazono, Tetsuya; Moriya, Tatsumi; Makino, Hirofumi; Kimura, Kenjiro; Suzuki, Yoshiki; Wada, Takashi; Ogawa, Susumu; Inaba, Masaaki; Kanno, Yoshihiko; Shigematsu, Takashi; Masakane, Ikuto; Tsuchiya, Ken; Honda, Keiko; Ichikawa, Kazuko; Shide, Kenichiro

    2015-03-01

    The Joint Committee on Diabetic Nephropathy has revised its Classification of Diabetic Nephropathy (Classification of Diabetic Nephropathy 2014) in line with the widespread use of key concepts, such as the estimated glomerular filtration rate (eGFR) and chronic kidney disease (CKD). In revising the Classification, the Committee carefully evaluated, as relevant to current revision, the report of a study conducted by the Research Group of Diabetic Nephropathy, Ministry of Health, Labor and Welfare of Japan. Major revisions to the Classification are summarized as follows: (i) eGFR is substituted for GFR in the Classification; (ii) the subdivisions A and B in stage 3 (overt nephropathy) have been reintegrated; (iii) stage 4 (kidney failure) has been redefined as a GFR <30 mL/min/1.73 m(2), regardless of the extent of albuminuria; and (iv) stress has been placed on the differential diagnosis of diabetic nephropathy versus non-diabetic kidney disease as being crucial in all stages of diabetic nephropathy.

  2. A new classification of Diabetic Nephropathy 2014: a report from Joint Committee on Diabetic Nephropathy.

    Science.gov (United States)

    Haneda, Masakazu; Utsunomiya, Kazunori; Koya, Daisuke; Babazono, Tetsuya; Moriya, Tatsumi; Makino, Hirofumi; Kimura, Kenjiro; Suzuki, Yoshiki; Wada, Takashi; Ogawa, Susumu; Inaba, Masaaki; Kanno, Yoshihiko; Shigematsu, Takashi; Masakane, Ikuto; Tsuchiya, Ken; Honda, Keiko; Ichikawa, Kazuko; Shide, Kenichiro

    2015-02-01

    The Joint Committee on Diabetic Nephropathy has revised its Classification of Diabetic Nephropathy (Classification of Diabetic Nephropathy 2014) in line with the widespread use of key concepts such as the estimated glomerular filtration rate (eGFR) and chronic kidney disease. In revising the Classification, the Committee carefully evaluated, as relevant to current revision, the report of a study conducted by the Research Group of Diabetic Nephropathy, Ministry of Health, Labour and Welfare of Japan. Major revisions to the Classification are summarized as follows: (1) eGFR is substituted for GFR in the Classification; (2) the subdivisions A and B in stage 3 (overt nephropathy) have been reintegrated; (3) stage 4 (kidney failure) has been redefined as a GFR less than 30 mL/min/1.73 m(2), regardless of the extent of albuminuria; and (4) stress has been placed on the differential diagnosis of diabetic nephropathy versus non-diabetic kidney disease as being crucial in all stages of diabetic nephropathy.

  3. IgM nephropathy revisited.

    Science.gov (United States)

    Mubarak, Muhammed; Kazi, Javed I

    2012-01-01

    IgM nephropathy (IgMN) is an idiopathic immune complex-mediated glomerulopathy that was first described as a distinct disease in a nephropathology literature in 1978. Here, a historical review and the current status of IgMN in the light of world literature and the current experience will be presented. The Pubmed (www.pubmed.gov) search was made for articles on IgMN as the sole subject of the study or where it constituted a significant number of cases in a biopsy series in the world literature written in English. A total of 41 articles were found. A critical review of the literature was made. Soon after 1978, a series of reports were published mostly from the western world, but the interest in the entity did not withstand the test of time. No substantial basic medical research was carried out and the disease was largely ignored by the western researchers. More recently, a flurry of articles have appeared in the literature on the topic, mostly from tropical countries, and have renewed the interest in the entity. However, most of the current literature on IgMN is based on clinical observations, and experimental models and mechanistic studies of IgMN are lacking. There is an urgent need to develop consensus based criteria for the diagnosis of the condition, as well as, to focus the research on mechanistic studies to understand the pathogenesis of the disease better.

  4. Associated with Balkan Endemic Nephropathy

    Directory of Open Access Journals (Sweden)

    Ljubinka Jankovic-Velickovic

    2011-01-01

    Full Text Available The role of aristolochic acid in the etiology of Balkan endemic nephropathy (BEN and associated upper-tract urothelial carcinoma (UTUC has been recently confirmed. The aim of this study was to determine apoptosis-related marker(s specific for BEN-associated UTUC. Present investigation included 105 patients with UTUC, 44 from BEN region and 61 control tumors. Altered expression of Survivin was more often present in BEN UTUC with high grade and solid growth (P<0.005; P<0.05 than in control tumors. Significantly lower expression of proapoptotic marker Bax was found in BEN tumors with high grade, high stage, necrosis, and without metaplastic change (P<0.05; 0.05; 0.05; 0.05 compared to control tumors with the same features. Group (BEN-related/control, stage, growth pattern, and caspase 3 activity were significantly associated with the expression of Bax (P=0.002, 0.034, 0.047, 0.028, resp.,. This investigation identifies Bax as specific marker of BEN-associated UTUC. Decrease of pro-apoptotic protein Bax together with alteration of Survivin may be indicative for specific disturbances of intrinsic apoptotic pathway in UTUC arising in endemic areas.

  5. Role of Mindin in Diabetic Nephropathy

    Directory of Open Access Journals (Sweden)

    Maki Murakoshi

    2011-01-01

    Full Text Available A number of studies have shown that proinflammatory cytokines have important roles in determining the development of microvascular diabetic complications, including nephropathy. Inflammatory biomarkers should be useful for diagnosis or monitoring of diabetic nephropathy. Mindin (spondin 2 is a member of the mindin-/F-spondin family of secreted extracellular matrix (ECM proteins. Recent studies showed that mindin is essential for initiation of innate immune response and represents a unique pattern-recognition molecule in the ECM. Previously, we demonstrated that the levels of urinary mindin in patients with type 2 diabetes were higher than those in healthy individuals. We propose that urinary mindin is a potent biomarker for the development of diabetic nephropathy.

  6. Contrast-induced nephropathy after computed tomography

    Directory of Open Access Journals (Sweden)

    Luciano da Silva Selistre

    2015-03-01

    Full Text Available Introduction: Contrast induced nephropathy is the third most prevalent preventable cause of acute kidney injury in hospitalized patients. It defined as an absolute increase in serum creatinine ≥ 0.5 mg/dL and relative ≥ 25% increase. Objective: We studied the risk factors to intravenous injection contrast nephropathy after computed tomography. Methods: We studied 400 patients prospectively. Results: The incidence of contrast induced nephropathy, with an absolute or a relative increase were 4.0% and 13.9%, respectively. Diabetes and cardiac failure were independent risk factors for CIN a relative increase de serum creatinine (O.R.: 3.5 [95% CI: 1.92-6.36], p < 0.01, 2.61 [95% CI: 1.14-6.03%], p < 0.05, respectively. Conclusions: We showed association between uses of intravenous injection contrast after computed tomography with acute injury renal, notably with diabetes and heart failure.

  7. Angiotensin receptor blockers in diabetic nephropathy

    DEFF Research Database (Denmark)

    Parving, Hans-Henrik; Andersen, Steen; Jacobsen, Peter

    2004-01-01

    The activity of the renin-angiotensin-aldosterone system (RAAS) is elevated both in the circulation and in the renal tissue of diabetic and nondiabetic nephropathies. The increased RAAS activity plays an important role in the hemodynamic and nonhemodynamic pathogenetic mechanisms involved in kidney...... with diabetic nephropathy have demonstrated that angiotensin II receptor blockers (ARB) induce favorable changes in systemic blood pressure, renal hemodynamics, and proteinuria similar to those induced by angiotensin-converting enzyme (ACE) inhibition. Studies have revealed the optimal renoprotective dose...... in diabetic nephropathy. In addition, dual RAAS blockade is safe and well tolerated. Impaired autoregulation of glomerular filtration rate (GFR); demonstrated with some blood pressure-lowering agents implies disturbances in the downstream transmission of the systemic blood pressure into the glomerulus...

  8. Combination regimen of leflunomide plus methylprednisolone in a female patient with reactive arthritis and concomitant IgA nephropathy

    Institute of Scientific and Technical Information of China (English)

    CHEN Yi-zhi; ZHAO Xue-zhi; WU Jun; MEI Chang-lin

    2010-01-01

    @@ Spondyloarthropathies (SpAs) include five categories: ankylosing spondylitis (AS),1 reactive arthritis (ReA), psoriatic arthritis (PsA), enteropathic arthritis (EA) and undifferentiated spondyloarthropathy (USpA).~2 ReA is an aseptic arthritis occurring after extra-articular infections, particularly genitourinary (GU) or gastrointestinal (GI) tracts. When arthritis is accompanied by conjunctivitis and urethritis, the diagnosis of Reiter syndrome will be suitable for this clinical triad; however, the term "ReA" has been proposed to substitute for Reiter syndrome.

  9. Childhood Cancer Statistics

    Science.gov (United States)

    ... Room Employment Feedback Contact Select Page Childhood Cancer Statistics Home > Cancer Resources > Childhood Cancer Statistics Childhood Cancer Statistics – Graphs and Infographics Number of Diagnoses Incidence Rates ...

  10. Treatment of Diabetic Nephropathy with Acupuncture

    Institute of Scientific and Technical Information of China (English)

    CHU Qin; WANG Lin; LIU Guo-zhen; HAN Chou-ping

    2007-01-01

    Objective: to observe the clinical efficacy of acupuncture on diabetic nephropathy.Methods: altogether 54 cases were randomly allocated into acupuncture group (30 cases) and control group of western medications (24 cases), the latter was treated with routine western medication, while the former was combined acupuncture based on routine western medication,and the treatment course of the two groups were both 30 days. Results: the effect of treatment group was superior to the control group (P<0.05). Conclusion: acupuncture can improve symptoms of diabetic nephropathy, lower blood glucose, urine albumin, blood urine nitrogen,and creatinine and improve the function of kidney.

  11. Remission and regression of diabetic nephropathy

    DEFF Research Database (Denmark)

    Hovind, Peter; Tarnow, Lise; Parving, Hans-Henrik

    2004-01-01

    diabetic patients with overt nephropathy, remission (decrease in albuminuria to diabetic nephropathy (rate of decline in GFR treatment. Furthermore, remission of nephrotic-range albuminuria......Diabetic kidney disease is considered to be an irreversible and inexorable progressive disease. Therefore, prevention of development of ESRD is extremely important. Animal studies have demonstrated that regression of existing renal morphologic lesions is feasible. In a sizable fraction of type 1...... in diabetic patients, an aggressive multifactorial approach, aiming at lowering blood pressure and albuminuria, and improving glycemic control, must be applied....

  12. Lymphoplasmacytic lymphoma causing light chain cast nephropathy.

    Science.gov (United States)

    Pérez, Nuria S; Garcia-Herrera, Adriana; Rosiñol, Laura; Palos, Lily; Santiago, Evelyn; Espinosa, Gerard; Solé, Manel; Campistol, Josep M; Quintana, Luis F

    2012-01-01

    Plasma cell dyscrasias are frequently associated with kidney disease through the production of monoclonal immunoglobulin but with a diverse set of pathologic renal patterns. While almost all patients with a renal biopsy showing a cast nephropathy have myeloma, kidney involvement associated with pathological immunoglobulin light chains and lymphoma is rare. To our knowledge, this is the first report of a cast nephropathy associated with lymphoplasmacytic lymphoma. We emphasize the relation between light chain deposition and renal dysfunction in this disease with production of light chains. A therapeutic approach that decreases light chain production appears to be warranted in these patients.

  13. [Physiopathology of nephropathy studied with contrast media].

    Science.gov (United States)

    Morales Buenrostro, L E; Tellez Zenteno, J F; Torre Delgadillo, A

    2000-01-01

    For the technological advances in diagnostic and therapeutic procedures, the use of intravenous contrast media in the hospital is more and more frequent. It can produce acute renal failure secondary to its nephrotoxicity known as contrast media nephropathy. This review describes the pathophysiologic mechanisms of contrast media injury, including cytotoxicity caused by hyperosmoloarity of contrast media, the hemodynamic factors and the role of the renin-angiotensin system, prostaglandins, oxygen free radicals, endothelin-1, adenosine, nitric oxide and others. The understanding of this information is of vital importance for the development of prophylactic strategies for contrast media nephropathy.

  14. SORBS1 gene, a new candidate for diabetic nephropathy

    DEFF Research Database (Denmark)

    Germain, Marine; Pezzolesi, Marcus G; Sandholm, Niina

    2015-01-01

    AIMS/HYPOTHESIS: The genetic determinants of diabetic nephropathy remain poorly understood. We aimed to identify novel susceptibility genes for diabetic nephropathy. METHODS: We performed a genome-wide association study using 1000 Genomes-based imputation to compare type 1 diabetic nephropathy...... consistently and significantly (p diabetic nephropathy. The minor rs1326934-C allele was less frequent in cases than in controls (0.34 vs 0.43) and was associated with a decreased risk for diabetic nephropathy (OR 0.70; 95% CI 0.60, 0.82). However, this association was not observed...... in a second stage with two additional diabetic nephropathy cohorts, the All Ireland-Warren 3-Genetics of Kidneys in Diabetes UK and Republic of Ireland (UK-ROI; p = 0.15) and the Finnish Diabetic Nephropathy (FinnDiane; p = 0.44) studies, totalling 2,142 cases and 2,494 controls. Altogether, the random...

  15. Interleukin (IL)-21 promotes intestinal IgA response to microbiota.

    Science.gov (United States)

    Cao, A T; Yao, S; Gong, B; Nurieva, R I; Elson, C O; Cong, Y

    2015-09-01

    Commensal microbiota-specific T helper type 17 (Th17) cells are enriched in the intestines, which can convert into T follicular helper (Tfh) in Peyer's patches, and are crucial for production of intestinal immunoglobulin A (IgA) against microbiota; however, the role of Th17 and Tfh cytokines in regulating the mucosal IgA response to enteric microbiota is still not completely known. In this study, we found that intestinal IgA was impaired in mice deficient in interleukin (IL)-17 or IL-21 signaling. IL-21, but not IL-17, is able to augment B-cell differentiation to IgA(+) cells as mediated by transforming growth factor β1 (TGFβ1) and accelerate IgA class switch recombination (CSR). IL-21 and retinoic acid (RA) induce IgA(+) B-cell development and IgA production and drives autocrine TGFβ1 production to initiate IgA CSR. Repletion of T-cell-deficient TCRβxδ(-/-) mice with Th17 cells specific for commensal bacterial antigen increased the levels of IgA(+) B cells and IgA production in the intestine, which was blocked by neutralizing IL-21. Thus IL-21 functions to strongly augment IgA production under intestinal environment. Furthermore, IL-21 promotes intestinal B-cell homing through α4β7 expression, alone or with TGFβ and RA. Together, IL-21 from microbiota-specific Th17 and/or Tfh cells contributes to robust intestinal IgA levels by enhancing IgA(+) CSR, IgA production and B-cell trafficking into the intestine.

  16. Childhood Emergencies

    Science.gov (United States)

    ... emergency physicians. They receive comprehensive training in treating childhood emergencies and have more training in pediatric emergencies than other physicians, including pediatricians. Does Your Child's School Know About Food Allergies? - 8/10/2015 The nation's emergency physician ...

  17. Childhood Craniopharyngioma

    Science.gov (United States)

    ... has any of the following: Headaches, including morning headache or headache that goes away after vomiting . Vision changes. Nausea ... Cancer Late Effects of Treatment for Childhood Cancer Adolescents and Young Adults with Cancer Children with Cancer: ...

  18. Epigenetic modifications and diabetic nephropathy

    Directory of Open Access Journals (Sweden)

    Marpadga A. Reddy

    2012-09-01

    Full Text Available Diabetic nephropathy (DN is a major complication associated with both type 1 and type 2 diabetes, and a leading cause of end-stage renal disease. Conventional therapeutic strategies are not fully efficacious in the treatment of DN, suggesting an incomplete understanding of the gene regulation mechanisms involved in its pathogenesis. Furthermore, evidence from clinical trials has demonstrated a “metabolic memory” of prior exposure to hyperglycemia that continues to persist despite subsequent glycemic control. This remains a major challenge in the treatment of DN and other vascular complications. Epigenetic mechanisms such as DNA methylation, nucleosomal histone modifications, and noncoding RNAs control gene expression through regulation of chromatin structure and function and post-transcriptional mechanisms without altering the underlying DNA sequence. Emerging evidence indicates that multiple factors involved in the etiology of diabetes can alter epigenetic mechanisms and regulate the susceptibility to diabetes complications. Recent studies have demonstrated the involvement of histone lysine methylation in the regulation of key fibrotic and inflammatory genes related to diabetes complications including DN. Interestingly, histone lysine methylation persisted in vascular cells even after withdrawal from the diabetic milieu, demonstrating a potential role of epigenetic modifications in metabolic memory. Rapid advances in high-throughput technologies in the fields of genomics and epigenomics can lead to the identification of genome-wide alterations in key epigenetic modifications in vascular and renal cells in diabetes. Altogether, these findings can lead to the identification of potential predictive biomarkers and development of novel epigenetic therapies for diabetes and its associated complications.

  19. Vesicoureteral reflux and reflux nephropathy

    Energy Technology Data Exchange (ETDEWEB)

    Thomsen, H.S.

    Vesicoureteral reflux (VUR) is mainly a primary phenomenon due to incontinence of the ureterovesical junction, mostly affecting a pediatric population. During micturition cystourethrography (MCU) reflux into the kidney - intrarenal reflux (IRR) - is occasionally seen. In areas with IRR the kidney surface may subsequently be depressed and the papillae retracted (reflux nephropathy (RN)). VUR may lead to hypertension and/or end-stage renal failure. Most commonly, VUR is discovered during evaluation for urinary tract infection, but it may also be present in patients with hypertension, toxemia of pregnancy, chronic renal failure and proteinuria, and it may be found in siblings of patients with VUR. For the time being VUR is demonstrated at radiographic MCU, whereas RN is diagnosed by demonstration of focal scars and of abnormal parenchymal thickness at urography. In children with VUR and no abnormalities of calyces or parenchymal defects standardized measurement of the parenchymal thickness at three sites may identify kidneys which are likely to develop focal scars. Quantitation of focal scarring should be performed in connection with a measure of the overall kidney size. The occurrence of IRR is dependent of the papillary morphology, intrapelvic pressure and urine flow. There may be an important relationship between renal ischemia and IRR in producing a 'vicious circle of deleterious effects' which, combined with parenchymal extravasation, may lead to RN. Treatment of VUR includes medical and surgical management. Since renal scarring may occur in infancy, prevention should focus on infants and young children. Infants and young children with severe VUR may have normal urograms. Therefore a MCU should also be performed, preferably with the recommended standardized technique. (orig.).

  20. A monoclonal anti-rat IgA antibody. Detection of IgA antibodies to ovalbumin and antigens of the parasite Trichinella spiralis in the rat

    NARCIS (Netherlands)

    van Loveren H; Osterhaus ADME; Nagel J; Hakkert B; de Klerk A; Drost G; Vos JG

    1987-01-01

    Dit rapport geeft de resultaten weer van de ontwikkeling van een monoclonaal antilichaam dat specifiek ratte IgA immunglobulinen herkent. IgA is een belangrijk immuunglobuline voor de bescherming van muscosale oppervlakken (o.a. op het niveau van de darm en de luchtwegen). Met behulp van het ontw

  1. Evaluation of monoclonal antibodies with specificity for human IgA, IgA subclasses and allotypes and secretory component. Results of an IUIS/WHO collaborative study

    NARCIS (Netherlands)

    Mestecky, J.; Hamilton, R.G.; Magnusson, C.G.M.; Jefferis, R.; Vaerman, J.P.; Goodall, M.; Lange, G.G. de; Moro, I.; Aucouturier, P.; Radl, J.; Cambiaso, C.; Silvain, C.; Preud'homme, J.L.; Kusama, K.; Carlone, G.M.; Biewenga, J.; Kobayashi, K.; Skvaril, F.; Reimer, C.B.

    1996-01-01

    51 monoclonal antibodies (McAb) with putative specificity for human IgA, the IgA subclasses, Am allotypes or secretory component (SC) were evaluated for immunoreactivity and specificity by nine laboratories employing immunodiffusion, agglutination, immunohistological assays, immunoblotting and direc

  2. Diabetic nephropathy in Surinamese South Asian subjects

    NARCIS (Netherlands)

    Chandieshaw, Prataap Kalap; Chandie Shaw, Prataap Kalap

    2008-01-01

    This thesis focuses on the incidence and risk factors for nephropathy in diabetic and non-diabetic Surinamese South Asians. The Surinamese South Asians, originally descended from the North-East India. Due to the former colonial bounds with the Netherlands, a relatively youn

  3. Diabetic nephropathy: Prescription trends in tertiary care

    Directory of Open Access Journals (Sweden)

    Devi D

    2008-01-01

    Full Text Available Diabetic nephropathy is a leading cause of end stage renal disease. Drug utilization studies could promote rational drug use. The objective of this study was to evaluate prescribing trends in hospitalized patients with diabetic nephropathy. A prospective, observational study was conducted in a tertiary care hospital. The demographic, disease and treatment data of patients with diabetic nephropathy were collected for a period of six months and analysed. Drugs were classified using World Health Organization recommended Anatomic Therapeutic Chemical classification. A total of 755 drugs (7.4 drugs per prescription were prescribed to 102 study patients, who were all hypertensive and in late stages of diabetic nephropathy. Drug classes with largest representation were those acting on gastrointestinal tract plus metabolism (37% and cardiovascular drugs (28%. Calcium channel blockers represented the largest antihypertensive drug class (41%. Almost three-fourths of patients received more than one antihypertensive agent. Approximately 37% of patients did not receive any antidiabetic medication. Of those who did, prescriptions for insulin (91% exceeded those of oral hypoglycaemic drugs (9%. Antimicrobials accounted for 10.2% of all drugs prescribed, of which 31.8% were quinolones. Drugs prescribed by generic name accounted for 11.98%. While all patients received antihypertensive therapy, more than a third were not on any antidiabetic treatment. Antihypertensive poly-therapy was observed in the majority with calcium channel blockers being most frequently prescribed antihypertensive drug class. Insulin was the preferred to hypoglycaemic drugs.

  4. Igaühele hea haridus!? / Olav Aarna

    Index Scriptorium Estoniae

    Aarna, Olav, 1942-

    2005-01-01

    Ilmunud ka: Järva Teataja 1. september lk. 2, Meie Maa 1. september lk. 2, Koit 1. september lk. 6, Severnoje Poberezhje 1. september lk. 2, Vali Uudised 2. september lk. 2, Sakala 2. september lk. 2, Hiiu Leht 2. september lk. 2, Kuulutaja 2. september lk. 4, Nädaline 8. september lk. 4. Riigikogu kultuurikomisjoni esimehe sõnul peaks igaüks sõnastama enda jaoks rahuldava vastuse küsimusele, mis on hea haridus

  5. Monoclonal IgA Antibodies for Aflatoxin Immunoassays

    Science.gov (United States)

    Ertekin, Özlem; Pirinçci, Şerife Şeyda; Öztürk, Selma

    2016-01-01

    Antibody based techniques are widely used for the detection of aflatoxins which are potent toxins with a high rate of occurrence in many crops. We developed a murine monoclonal antibody of immunoglobulin A (IgA) isotype with a strong binding affinity to aflatoxin B1 (AFB1), aflatoxin B2 (AFB2), aflatoxin G1 (AFG1), aflatoxin G2 (AFG2) and aflatoxin M1 (AFM1). The antibody was effectively used in immunoaffinity column (IAC) and ELISA kit development. The performance of the IACs was compatible with AOAC performance standards for affinity columns (Test Method: AOAC 991.31). The total binding capacity of the IACs containing our antibody was 111 ng, 70 ng, 114 ng and 73 ng for AFB1, AFB2, and AFG1 andAFG2, respectively. Furthermore, the recovery rates of 5 ng of each AF derivative loaded to the IACs were determined as 104.9%, 82.4%, 85.5% and 70.7% for AFB1, AFB2, AFG1 and AFG2, respectively. As for the ELISA kit developed using non-oriented, purified IgA antibody, we observed a detection range of 2–50 µg/L with 40 min total test time. The monoclonal antibody developed in this research is hitherto the first presentation of quadruple antigen binding IgA monoclonal antibodies in mycotoxin analysis and also the first study of their utilization in ELISA and IACs. IgA antibodies are valuable alternatives for immunoassay development, in terms of both sensitivity and ease of preparation, since they do not require any orientation effort. PMID:27187470

  6. Monoclonal IgA Antibodies for Aflatoxin Immunoassays

    Directory of Open Access Journals (Sweden)

    Özlem Ertekin

    2016-05-01

    Full Text Available Antibody based techniques are widely used for the detection of aflatoxins which are potent toxins with a high rate of occurrence in many crops. We developed a murine monoclonal antibody of immunoglobulin A (IgA isotype with a strong binding affinity to aflatoxin B1 (AFB1, aflatoxin B2 (AFB2, aflatoxin G1 (AFG1, aflatoxin G2 (AFG2 and aflatoxin M1 (AFM1. The antibody was effectively used in immunoaffinity column (IAC and ELISA kit development. The performance of the IACs was compatible with AOAC performance standards for affinity columns (Test Method: AOAC 991.31. The total binding capacity of the IACs containing our antibody was 111 ng, 70 ng, 114 ng and 73 ng for AFB1, AFB2, and AFG1 andAFG2, respectively. Furthermore, the recovery rates of 5 ng of each AF derivative loaded to the IACs were determined as 104.9%, 82.4%, 85.5% and 70.7% for AFB1, AFB2, AFG1 and AFG2, respectively. As for the ELISA kit developed using non-oriented, purified IgA antibody, we observed a detection range of 2–50 µg/L with 40 min total test time. The monoclonal antibody developed in this research is hitherto the first presentation of quadruple antigen binding IgA monoclonal antibodies in mycotoxin analysis and also the first study of their utilization in ELISA and IACs. IgA antibodies are valuable alternatives for immunoassay development, in terms of both sensitivity and ease of preparation, since they do not require any orientation effort.

  7. Monoclonal IgA Antibodies for Aflatoxin Immunoassays.

    Science.gov (United States)

    Ertekin, Özlem; Pirinçci, Şerife Şeyda; Öztürk, Selma

    2016-05-12

    Antibody based techniques are widely used for the detection of aflatoxins which are potent toxins with a high rate of occurrence in many crops. We developed a murine monoclonal antibody of immunoglobulin A (IgA) isotype with a strong binding affinity to aflatoxin B1 (AFB1), aflatoxin B2 (AFB2), aflatoxin G1 (AFG1), aflatoxin G2 (AFG2) and aflatoxin M1 (AFM1). The antibody was effectively used in immunoaffinity column (IAC) and ELISA kit development. The performance of the IACs was compatible with AOAC performance standards for affinity columns (Test Method: AOAC 991.31). The total binding capacity of the IACs containing our antibody was 111 ng, 70 ng, 114 ng and 73 ng for AFB1, AFB2, and AFG1 andAFG2, respectively. Furthermore, the recovery rates of 5 ng of each AF derivative loaded to the IACs were determined as 104.9%, 82.4%, 85.5% and 70.7% for AFB1, AFB2, AFG1 and AFG2, respectively. As for the ELISA kit developed using non-oriented, purified IgA antibody, we observed a detection range of 2-50 µg/L with 40 min total test time. The monoclonal antibody developed in this research is hitherto the first presentation of quadruple antigen binding IgA monoclonal antibodies in mycotoxin analysis and also the first study of their utilization in ELISA and IACs. IgA antibodies are valuable alternatives for immunoassay development, in terms of both sensitivity and ease of preparation, since they do not require any orientation effort.

  8. Amino acid sequence requirements in the hinge of human immunoglobulin A1 (IgA1) for cleavage by streptococcal IgA1 proteases

    DEFF Research Database (Denmark)

    Batten, MR; Senior, BW; Kilian, Mogens;

    2003-01-01

    The amino acid sequence requirements in the hinge of human immunoglobulin A1 (IgA1) for cleavage by IgA1 proteases of different species of Streptococcus were investigated. Recombinant IgA1 antibodies were generated with point mutations at proline 227 and threonine 228, the residues lying on either...... that proteases from different strains may have different amino acid requirements for cleavage. Remarkably, some streptococcal proteases appeared able to cleave the hinge at a distant alternative site if substitution prevented efficient cleavage of the original site. Hence, this study has identified key residues...... required for the recognition of the IgA1 hinge as a substrate by streptococcal IgA1 proteases, and it marks a preliminary step towards development of specific enzyme inhibitors....

  9. IgM nephropathy: Clinical picture and pathological findings in 36 patients

    Directory of Open Access Journals (Sweden)

    Ghadeer A Mokhtar

    2011-01-01

    Full Text Available Immunoglobulin M nephropathy (IgMN is an idiopathic glomerulonephritis characterized by a variable degree of mesangial hyper- cellularity and diffuse IgM deposits. This study describes the clinical presentation and the morphological findings in 36 patients, five adults and 31 children, with IgMN. The initial manifestations of the disease were the nephrotic syndrome in 32 patients, proteinuria in two, hematuria associated with proteinuria in 16 and isolated recurrent gross hematuria in two patients. The nephrotic syndrome was steroid responsive in 9% of the cases, steroid dependent in 25% and steroid resistant in 66%. Five patients were hypertensive at the onset of the disease. The mean follow- up period was 3.4 years (range, 1-7 years. One patient developed end- stage kidney disease five years after the diagnosis. On light microscopy, 24 specimens showed mild focal and segmental mesangial hyper- cellularity, 10 cases were normal in cellularity and two cases showed diffuse global mesangial hyper- cellularity. Four cases showed focal and segmental glomerulosclerosis. Mild interstitial inflammation and fibrosis were observed in 11 cases and focal tubular atrophy and hypertensive hyaline arteriosclerosis were noted in three cases. In addition to IgM, the immunofluorescence study showed C3 deposits in 12 cases, IgG in nine cases and IgA in one case. Electron dense- deposits in the mesangium and para- mesangial areas were demonstrated on electron microscopy in 22 cases. In view of its different clinical presentations and the constant findings of diffuse mesangial IgM deposition in all the cases, it is concluded that this form of nephropathy constitutes a distinct entity separate from minimal change disease or focal and segmental glomerulosclerosis. Patients with IgMN had a higher prevalence of steroid resistance and dependence in the proteinuric group.

  10. Childhood obesity.

    Science.gov (United States)

    Seth, Anju; Sharma, Rajni

    2013-04-01

    Childhood obesity is an issue of serious medical and social concern. In developing countries including India, it is a phenomenon seen in higher socioeconomic strata due to the adoption of a western lifestyle. Consumption of high calorie food, lack of physical activity and increased screen time are major risk factors for childhood obesity apart from other genetic, prenatal factors and socio-cultural practices. Obese children and adolescents are at increased risk of medical and psychological complications. Insulin resistance is commonly present especially in those with central obesity and manifests as dyslipidemia, type 2 diabetes mellitus, impaired glucose tolerance, hypertension, polycystic ovarian syndrome and metabolic syndrome. Obese children and adolescents often present to general physicians for management. The latter play a key role in prevention and treatment of obesity as it involves lifestyle modification of the entire family. This article aims at discussing the approach to diagnosis and work-up, treatment and preventive strategies for childhood obesity from a general physician's perspective.

  11. Childhood obesity

    DEFF Research Database (Denmark)

    Heitmann, Berit L; Koplan, Jeffrey; Lissner, Lauren

    2009-01-01

    Despite progress toward assuring the health of today's young population, the 21(st) century began with an epidemic of childhood obesity. There is general agreement that the situation must be addressed by means of primary prevention, but relatively little is known about how to intervene effectively....... The evidence behind the assumption that childhood obesity can be prevented was discussed critically in this roundtable symposium. Overall, there was general agreement that action is needed and that the worldwide epidemic itself is sufficient evidence for action. As the poet, writer, and scholar Wittner Bynner...... (1881-1968) wrote, "The biggest problem in the world could have been solved when it was small"....

  12. Reduced Expression of Lipoic Acid Synthase Accelerates Diabetic Nephropathy

    OpenAIRE

    Yi, Xianwen; Xu, Longquan; Hiller, Sylvia; Kim, Hyung-Suk; Nickeleit, Volker; James, Leighton R; Maeda, Nobuyo

    2011-01-01

    Oxidative stress contributes to the pathogenesis of diabetic nephropathy. In mitochondria, lipoic acid synthase produces α-lipoic acid, an antioxidant and an essential cofactor in α-ketoacid dehydrogenase complexes, which participate in glucose oxidation and ATP generation. Administration of lipoic acid abrogates diabetic nephropathy in animal models, but whether lower production of endogenous lipoic acid promotes diabetic nephropathy is unknown. Here, we crossed mice heterozygous for lipoic ...

  13. Dermatose por IgA linear induzida pela gestação Linear IgA dermatosis induced by pregnancy

    Directory of Open Access Journals (Sweden)

    Telma Kanagusuko

    2008-02-01

    Full Text Available A dermatose por IgA linear é doença bolhosa auto-imune subepidérmica rara, caracterizada pelo depósito linear de IgA na zona da membrana basal da epiderme. Nos relatos de gestação em pacientes com essa dermatose, nota-se sempre melhora do quadro clínico. Contrariando essas observações,é apresentado caso de dermatose por IgA linear induzida pela gestação, que demonstrou boa resposta terapêutica à dapsona e prednisona , sem complicações materno-fetais.Linear IgA disease is a rare autoimmune subepidermal bullous disorder characterized by linear IgA deposits at the epidermal basement membrane zone. According to the literature, in patients who have linear IgA disease and become pregnant, the disease tends to improve. We report a case of linear IgA disease induced by pregnancy, successfully treated with dapsone and prednisone with no adverse effects observed in the patient and her newborns.

  14. Smoking and progression of diabetic nephropathy in type 1 diabetes

    DEFF Research Database (Denmark)

    Hovind, Peter; Rossing, Peter; Tarnow, Lise

    2003-01-01

    OBJECTIVE: Cigarette smoking contributes to development of diabetic nephropathy. However, long-term studies on the effect of smoking on decline in kidney function in diabetic nephropathy are lacking. We assessed the impact of smoking on progression of diabetic nephropathy in type 1 diabetic...... patients enrolled in a prospective observational cohort study started in 1983. RESEARCH DESIGN AND METHODS: We identified all albuminuric type 1 diabetic patients (n = 301) followed for at least 3 years, median (range) 7 years (3-14), who underwent at least yearly measurement of glomerular filtration rate...... was not associated with decline in kidney function in type 1 diabetic patients with diabetic nephropathy....

  15. Childhood Obesity

    Centers for Disease Control (CDC) Podcasts

    2013-08-06

    In this podcast, Dr. Tom Frieden, CDC Director, discusses the decrease in childhood obesity rates and what strategies have been proven to work to help our children grow up and thrive.  Created: 8/6/2013 by National Center for Injury Prevention and Control.   Date Released: 3/6/2014.

  16. Childhood Obesity

    Science.gov (United States)

    Yuca, Sevil Ari, Ed.

    2012-01-01

    This book aims to provide readers with a general as well as an advanced overview of the key trends in childhood obesity. Obesity is an illness that occurs due to a combination of genetic, environmental, psychosocial, metabolic and hormonal factors. The prevalence of obesity has shown a great rise both in adults and children in the last 30 years.…

  17. Childhood obesity.

    Science.gov (United States)

    Strauss, R

    1999-01-01

    Approximately 10% of children are obese. Twin and adoption studies demonstrate a large genetic component to obesity, especially in adults. However, the increasing prevalence of obesity over the last 20 years can only be explained by environmental factors. In most obese individuals, no measurable differences in metabolism can be detected. Few children engage in regular physical activity. Obese children and adults uniformly underreport the amount of food they eat. Obesity is particularly related to increased consumption of high-fat foods. BMI is a quick and easy way to screen for childhood obesity. Treating childhood obesity relies on positive family support and lifestyle changes involving the whole family. Food preferences are influenced early by parental eating habits, and when developed in childhood, they tend to remain fairly constant into adulthood. Children learn to be active or inactive from their parents. In addition, physical activity (or more commonly, physical inactivity) habits that are established in childhood tend to persist into adulthood. Weight loss is usually followed by changes in appetite and metabolism, predisposing individuals to regain their weight. However, when the right family dynamics exist--a motivated child with supportive parents--long-term success is possible.

  18. Classification and Differential Diagnosis of Diabetic Nephropathy

    Science.gov (United States)

    2017-01-01

    Diabetic nephropathy (DN) is a major cause of end-stage renal disease throughout the world in both developed and developing countries. This review briefly introduces the characteristic pathological changes of DN and Tervaert pathological classification, which divides DN into four classifications according to glomerular lesions, along with a separate scoring system for tubular, interstitial, and vascular lesions. Given the heterogeneity of the renal lesions and the complex mechanism underlying diabetic nephropathy, Tervaert classification has both significance and controversies in the guidance of diagnosis and prognosis. Applications and evaluations using Tervaert classification and indications for renal biopsy are summarized in this review according to recent studies. Meanwhile, differential diagnosis with another nodular glomerulopathy and the situation that a typical DN superimposed with a nondiabetic renal disease (NDRD) are discussed and concluded in this review. PMID:28316995

  19. Classification and Differential Diagnosis of Diabetic Nephropathy

    Directory of Open Access Journals (Sweden)

    Chenyang Qi

    2017-01-01

    Full Text Available Diabetic nephropathy (DN is a major cause of end-stage renal disease throughout the world in both developed and developing countries. This review briefly introduces the characteristic pathological changes of DN and Tervaert pathological classification, which divides DN into four classifications according to glomerular lesions, along with a separate scoring system for tubular, interstitial, and vascular lesions. Given the heterogeneity of the renal lesions and the complex mechanism underlying diabetic nephropathy, Tervaert classification has both significance and controversies in the guidance of diagnosis and prognosis. Applications and evaluations using Tervaert classification and indications for renal biopsy are summarized in this review according to recent studies. Meanwhile, differential diagnosis with another nodular glomerulopathy and the situation that a typical DN superimposed with a nondiabetic renal disease (NDRD are discussed and concluded in this review.

  20. Diabetic Nephropathy : Evaluation with Doppler Ultrasonography

    Energy Technology Data Exchange (ETDEWEB)

    Sim, Jung Suk; Kim, Seung Hyup; Kang, Heung Sik; Park, Jae Hyung; Han, Man Chung [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    1996-06-15

    To compare Doppler ultrasonography with laboratory tests in evaluation of diabetic nephropathy. Fifty-five patients (mean age = 60, M : F = 26 : 29) with diabetes mellitus underwent renal Doppler ultrasonography. Resistive indices were compared with degree of proteinuria, serum creatinine level, and creatinine clearance rate. Eighteen patients who showed no proteinuria or microscopic proteinuria had a mean resistive index (RI) of 0.72 (SD, 0.05), 16 patients with macroscopic proteinuria without nephrotic syndrome had a mean RI of 0.82 (SD, 0.13), and 21 patients with nephrotic syndrome had a mean RI of 0.90 (SD, 0.12). Renal RI correlated highly with serum creatinine level (r = 0.62) and creatinine clearance rate (r = -0.43). Renal Doppler ultrasonography provides a useful indication of renal function in diabetic nephropathy but cannot offer an advantage over conventional laboratory test

  1. Renoprotective effects of losartan in diabetic nephropathy

    DEFF Research Database (Denmark)

    Andersen, Steen; Tarnow, Lise; Cambien, Francois

    2002-01-01

    evaluated the short-term renoprotective effect in diabetic nephropathy of the angiotensin II receptor antagonist losartan in patients homozygous for the insertion or the deletion allele. METHODS: Fifty-four hypertensive type 1 diabetic patients with diabetic nephropathy homozygous for the insertion (I; N......BACKGROUND: The beneficial short- and long-term renoprotective effects of angiotensin I-converting enzyme (ACE) inhibition are lower in albuminuric diabetic patients homozygous for the deletion compared to the insertion polymorphism of the ACE gene. In an attempt to overcome this interaction, we...... = 26) or the deletion (D; N = 28) allele of the ACE/ID polymorphism were included. After four weeks of washout, the patients received losartan 50 mg daily followed by 100 mg in two treatment periods each lasting two months. Patients and investigators were blinded to ACE genotypes. At baseline...

  2. Diabetic nephropathy in Surinamese South Asian subjects

    OpenAIRE

    Chandieshaw, Prataap Kalap; Chandie Shaw, Prataap Kalap

    2008-01-01

    This thesis focuses on the incidence and risk factors for nephropathy in diabetic and non-diabetic Surinamese South Asians. The Surinamese South Asians, originally descended from the North-East India. Due to the former colonial bounds with the Netherlands, a relatively young South Asian migrant population settled in the Netherlands. South Asians have a high prevalence of central obesity and an eight-fold higher prevalence for type 2 diabetes mellitus. We found the following conclusions: 1.Sur...

  3. Diabetic nephropathy – complications and treatment

    OpenAIRE

    Lim AK

    2014-01-01

    Andy KH Lim1–3 1Department of Nephrology, Monash Medical Center, Monash Health, 2Department of General Medicine, Dandenong Hospital, Monash Health, 3Department of Medicine, Monash University, Clayton, VIC, Australia Abstract: Diabetic nephropathy is a significant cause of chronic kidney disease and end-stage renal failure globally. Much research has been conducted in both basic science and clinical therapeutics, which has enhanced understanding of the pathophysiology of diabetic ne...

  4. Linear IGA bullous dermatosis in Tunisian children : 31 cases

    Directory of Open Access Journals (Sweden)

    Kharfi Monia

    2011-01-01

    Full Text Available Background: Linear IgA bullous dermatosis (LAD of children is relatively frequent in Africa. Aim: We undertook this study to evaluate the frequency of this disease among autoimmune bullous diseases (AIBDs in Tunisian children. Materials and Methods: We present a 32-year retrospective study (January 1976 to December 2007. Children with chronic acquired bullous diseases seen at the Charles Nicolle Hospital of Tunis and for who direct immunofluorescence (DIF of the perilesional skin demonstrated linear IgA immunoglobulin deposits were included in the study population. Results: Thirty-one children with LAD were selected representing 65.9% of all AIBDs of children selected in the same period, with a mean age of 5.5 years and a sex ratio (M/F of 2.4. Most of the children had generalized eruption (28/31, more profuse on the face, pelvic region, buttocks and limbs. Mucosal lesions happened in only four children (12.9%. The mean duration of the disease was 14 months. DIF demonstrated linear IgA deposits along the dermal-epidermal junction in all patients. IgG, IgM, and complement were also seen (20/31. Indirect immunofluorescence was negative in 67% of cases. Eight patients responded to dapsone; however, prednisone had to be added in seven children to control the disease and erythromycin in four others. A long-term remission period was achieved in 76.1% of patients. Conclusion: This study confirms that LAD is the most common AIBD in children in Tunisia which frequently occurs in preschool-aged males. Independently of the used drug, a long-term remission is frequently observed.

  5. An unusual presentation of childhood vasculitis presenting in adulthood: A challenging diagnosis of Henoch-Schönlein Purpura

    Directory of Open Access Journals (Sweden)

    Charat Thongprayoon

    2014-01-01

    Full Text Available Context: Henoch-Schφnlein purpura (HSP, a systemic IgA vascultitis, is uncommon in adults, with an incidence rate of 0.1 to 1.2 per million in adults over 20 years old. This vasculitic syndrome can present as an uncommon cause of intestinal obstruction in older patients. We report a case of an older woman with HSP presenting with small bowel obstruction and vasculitic rash. Case Report: We report a 67-year-old woman who presented with small bowel obstruction and skin rash. Skin biopsy revealed leukocytoclastic vasculitis with +IgA granular deposition within the walls of superficial dermal vessels. Kidney biopsy confirmed the diagnosis of HSP with mild mesangial proliferative IgA nephropathy. Her abdominal pain and small bowel obstruction were improved with conservative treatment. She continued to do well with normal kidney function at a 3-month follow-up visit. Conclusion: HSP, a systemic IgA vasculitis, is a predominantly pediatric vasculitis and is uncommon in adults. In adults, the disease process is identical to that in children. However, gastrointestinal manifestation is less common in older patients, and bowel perforation and obstruction are rare. Intestinal obstruction with skin rash and renal involvement should raise suspicions of HSP.

  6. Histological changes of kidney in diabetic nephropathy.

    Science.gov (United States)

    Pourghasem, Mohsen; Shafi, Hamid; Babazadeh, Zahra

    2015-01-01

    Diabetes mellitus is the most common cause of chronic renal disorders and end-stage kidney disease in developed countries. It is the major cause of dialysis and transplantation. Failure in renal function causes wide disorders in the body. Diabetes results in wide range of alterations in the renal tissue. It is believed that early histological changes in diabetic nephropathy are detectable 2 years after diabetes is diagnosed. The glomerular alterations are the most important lesions in the diabetic nephropathy (DN). The Renal Pathology Society provides a new pathological classification for the detection of histopathology of DN. It divides diabetic nephropathy into four hierarchical glomerular lesions. Alloxan or streptozotocin induced diabetic rat is the one most widely used specie to study DN. Histological changes in the rat DN closely resemble the human disease and the most information of this review was obtained through the study of rat DN. All cell types of the kidney such as mesangial cells, podocytes and tubulointerstitial cells are liable to be affected in the event of DN. Severity of renal lesions is associated to the clinical aspect of renal outcome, but the aim of this article was only to review the histological changes of kidney in diabetes mellitus.

  7. Monoclonal IgA Antibodies for Aflatoxin Immunoassays

    OpenAIRE

    2016-01-01

    Antibody based techniques are widely used for the detection of aflatoxins which are potent toxins with a high rate of occurrence in many crops. We developed a murine monoclonal antibody of immunoglobulin A (IgA) isotype with a strong binding affinity to aflatoxin B1 (AFB1), aflatoxin B2 (AFB2), aflatoxin G1 (AFG1), aflatoxin G2 (AFG2) and aflatoxin M1 (AFM1). The antibody was effectively used in immunoaffinity column (IAC) and ELISA kit development. The performance of the IACs was compatible ...

  8. "Iga päev..." : [luuletused] / Doris Kareva

    Index Scriptorium Estoniae

    Kareva, Doris, 1958-

    2001-01-01

    Tekst eesti ja inglise k. D. Kareva lühibiograafia eesti ja inglise k. lk. 175. Sisu: "Iga päev..." = "Every day..." ; "Ma nägin unes - Saatan kõneles..." = "I dream that I heard Satan speak..." ; "Viib sünnieelsest unest surmaunne..." = "Rainbow-coloured confusion bears us..." ; "Vaadeldes vikerkaarlevat maailma..." = "Viewing the rainbowing world..." ; "Ei jõua kirjutada puhtandit..." = "No time to write the final draft..." ; "Põletatud luuletused..." = ""Burnt poems..." ; Fraktalia ; Müsteerium 1-5 = Enigma 1-5 ; "Jumal juhtub..." = "God happens..." ; Moira 1-7 = Wishing well 1-7 ; Concerto strumenti e voce

  9. A case of vancomycin-associated linear IgA bullous dermatosis and IgA antibodies to the α3 subunit of laminin-332.

    Science.gov (United States)

    Zenke, Y; Nakano, T; Eto, H; Koga, H; Hashimoto, T

    2014-04-01

    Linear IgA bullous dermatosis (LABD) is a rare autoimmune bullous disease, which is defined by the histopathological finding of subepidermal vesicles with neutrophilic infiltration and linear IgA deposits in the basement membrane zone, revealed by immunofluorescence study. We present a case of LABD in which vancomycin (VCM) administration triggered LABD, and immunoblot analysis showed IgA antibodies reactive to the 145- and 165-kDa α3 subunits of laminin-332. This is the first report of VCM-associated LABD in which the target antigen was laminin-332. In the present case, we were compelled to continue administration of VCM along with systemic steroids, which eventually led to the attenuation of the symptoms, normalization of the serum IgA level, and negative results on both indirect immunofluorescence of 1 mol L(-1) NaCl-split skin and immunoblot analysis.

  10. Salivary IgA response of triathletes participating in the French Iron Tour.

    Science.gov (United States)

    Libicz, S; Mercier, B; Bigou, N; Le Gallais, D; Castex, F

    2006-05-01

    Athletes are susceptible to upper respiratory tract infections (URTI) during intense training and after major competitions. Secretory IgA, which is the predominant antibody of the mucosal immune system, is the major effector of host-resistance to many microorganisms causing URTI. Previous studies have shown that salivary IgA-mediated immunity decreases after a single short distance triathlon, but the effect of repeated triathlon competitions on secretory IgA levels remains unknown. The purpose of this study was to examine the salivary IgA response of elite triathletes in repeated triathlon races during the 2001 French Iron Tour (FIT). Eight triathletes participated in this study. Saliva samples were collected daily after waking up (fasting basal state), before (pre-race) and after (post-race) each day's competition. Salivary IgA, total protein, and flow rate were measured. Salivary IgA concentrations were measured by an enzyme-linked immunosorbent assay. The salivary flow rate was significantly decreased after each race compared with the fasting basal state (p < 0.01). The salivary IgA concentration of the fasting basal state decreased over the FIT and was even lower than that of the post-race values (p < 0.05). The salivary IgA secretion rate of the fasting basal state decreased by 51.9% over the FIT (p < 0.05). Our data suggest that intense exercise repeated daily has a cumulative negative effect on basal levels of salivary IgA.

  11. Linear IgA bullous dermatosis in a patient with renal cell carcinoma

    NARCIS (Netherlands)

    Van der Waal, RIF; Van de Scheur, MR; Pas, HH; Jonkman, MF; Van Groeningen, CJ; Nieboer, C; Starink, TM

    2001-01-01

    Linear IgA bullous dermatosis (LABD) is an autoimmune subepidermal bullous disease with heterogeneous clinical manifestations, characterized by linear deposition of IgA along the epidermal basement membrane zone. We report a patient with a metastasized renal cell carcinoma who developed an extensive

  12. Childhood pancreatitis.

    Science.gov (United States)

    Uretsky, G; Goldschmiedt, M; James, K

    1999-05-01

    Acute pancreatitis is a rare finding in childhood but probably more common than is generally realized. This condition should be considered in the evaluation of children with vomiting and abdominal pain, because it can cause significant morbidity and mortality. Clinical suspicion is required to make the diagnosis, especially when the serum amylase concentration is normal. Recurrent pancreatitis may be familial as a result of inherited biochemical or anatomic abnormalities. Patients with hereditary pancreatitis are at high risk for pancreatic cancer.

  13. Childhood rhabdomyosarcoma.

    Science.gov (United States)

    Córdoba Rovira, S M; Inarejos Clemente, E J

    Rhabdomyosarcoma is the most common soft-tissue sarcoma in children; it can appear in any part of the body. Its biological behavior varies widely, and despite the absence of specific clinical or radiological characteristics, rhabdomyosarcoma should be taken into account in the differential diagnosis of solid tumors in children. This review focuses primarily on the imaging findings and anatomical distribution of the histological subtypes of childhood rhabdomyosarcoma and secondarily on the differential findings in histological studies.

  14. Risk factor control is key in diabetic nephropathy.

    Science.gov (United States)

    Lewis, Gareth; Maxwell, Alexander P

    2014-02-01

    Prolonged duration of diabetes, poor glycaemic control and hypertension are major risk factors for both diabetic nephropathy and cardiovascular disease. Optimising blood sugar control together with excellent control of blood pressure can reduce the risk of developing diabetic nephropathy. Diabetic nephropathy should be considered in any patient with diabetes when persistent albuminuria develops. Microalbuminuria is the earliest clinically detectable indicator of diabetic nephropathy risk. The majority of patients with diabetic nephropathy are appropriately diagnosed based on elevated urinary albumin excretion and/or reduced 0032-6518 renal function. Patients with type 2 diabetes should have annual urinary ACR measurements from the time of diabetes diagnosis while those with type 1 diabetes should commence five years after diagnosis. Blood pressure lowering to 130/80mmHg and reduction of proteinuria to diabetic nephropathy and reduces the number of cardiovascular events. Drugs that block the renin-angiotensin-aldosterone system (RAAS) are effective in reducing proteinuria, managing hypertension and reducing cardiovascular risk. Unless there are clear contraindications or intolerance all patients with diabetic nephropathy should be prescribed an ACEI or ARB. Stopping an ACEI or ARB during intercurrent illness or times of volume depletion is critically important. Patients with diabetic nephropathy should have at least yearly measurements of blood pressure, renal function and urinary ACR.

  15. Generalized Nets for the Diagnosis and Management of Diabetic Nephropathy

    Directory of Open Access Journals (Sweden)

    Anthony Shannon

    2009-10-01

    Full Text Available This paper develops Generalized Nets for the diagnosis and management of diabetic nephropathy. The first net accounts for the development of nephropathy from an initial increase in glomerular filtration rates through mciroalbuminuria to end stage renal disease. The second net referes to the management of diabetes mellitus to increase the probability of preventing diabetic nephropathyor minimising its effects.

  16. Default in plasma and intestinal IgA responses during acute infection by simian immunodeficiency virus

    Directory of Open Access Journals (Sweden)

    Chaoul Nada

    2012-05-01

    Full Text Available Abstract Background Conflicting results regarding changes in mucosal IgA production or in the proportions of IgA plasma cells in the small and large intestines during HIV-infection have been previously reported. Except in individuals repeatedly exposed to HIV-1 but yet remaining uninfected, HIV-specific IgAs are frequently absent in mucosal secretions from HIV-infected patients. However, little is known about the organization and functionality of mucosal B-cell follicles in acute HIV/SIV infection during which a T-dependent IgA response should have been initiated. In the present study, we evaluated changes in B-cell and T-cell subsets as well as the extent of apoptosis and class-specific plasma cells in Peyer’s Patches, isolated lymphoid follicles, and lamina propria. Plasma levels of IgA, BAFF and APRIL were also determined. Results Plasma IgA level was reduced by 46% by 28 days post infection (dpi, and no IgA plasma cells were found within germinal centers of Peyer’s Patches and isolated lymphoid follicles. This lack of a T-dependent IgA response occurs although germinal centers remained functional with no sign of follicular damage, while a prolonged survival of follicular CD4+ T-cells and normal generation of IgG plasma cells is observed. Whereas the average plasma BAFF level was increased by 4.5-fold and total plasma cells were 1.7 to 1.9-fold more numerous in the lamina propria, the relative proportion of IgA plasma cells in this effector site was reduced by 19% (duodemun to 35% (ileum at 28 dpi. Conclusion Our data provide evidence that SIV is unable to initiate a T-dependent IgA response during the acute phase of infection and favors the production of IgG (ileum or IgM (duodenum plasma cells at the expense of IgA plasma cells. Therefore, an early and generalized default in IgA production takes place during the acute of phase of HIV/SIV infection, which might impair not only the virus-specific antibody response but also IgA responses

  17. Childhood Obesity Facts

    Science.gov (United States)

    ... Breastfeeding Micronutrient Malnutrition State and Local Programs Childhood Obesity Facts Recommend on Facebook Tweet Share Compartir On ... Children (WIC) Program, 2000–2014 Prevalence of Childhood Obesity in the United States, 2011-2014 Childhood obesity ...

  18. PetIGA: A framework for high-performance isogeometric analysis

    KAUST Repository

    Dalcin, L.

    2016-05-25

    We present PetIGA, a code framework to approximate the solution of partial differential equations using isogeometric analysis. PetIGA can be used to assemble matrices and vectors which come from a Galerkin weak form, discretized with Non-Uniform Rational B-spline basis functions. We base our framework on PETSc, a high-performance library for the scalable solution of partial differential equations, which simplifies the development of large-scale scientific codes, provides a rich environment for prototyping, and separates parallelism from algorithm choice. We describe the implementation of PetIGA, and exemplify its use by solving a model nonlinear problem. To illustrate the robustness and flexibility of PetIGA, we solve some challenging nonlinear partial differential equations that include problems in both solid and fluid mechanics. We show strong scaling results on up to 40964096 cores, which confirm the suitability of PetIGA for large scale simulations.

  19. Regulation of intestinal IgA responses by dietary palmitic acid and its metabolism.

    Science.gov (United States)

    Kunisawa, Jun; Hashimoto, Eri; Inoue, Asuka; Nagasawa, Risa; Suzuki, Yuji; Ishikawa, Izumi; Shikata, Shiori; Arita, Makoto; Aoki, Junken; Kiyono, Hiroshi

    2014-08-15

    Enhancement of intestinal IgA responses is a primary strategy in the development of oral vaccine. Dietary fatty acids are known to regulate host immune responses. In this study, we show that dietary palmitic acid (PA) and its metabolites enhance intestinal IgA responses. Intestinal IgA production was increased in mice maintained on a PA-enriched diet. These mice also showed increased intestinal IgA responses against orally immunized Ag, without any effect on serum Ab responses. We found that PA directly stimulates plasma cells to produce Ab. In addition, mice receiving a PA-enriched diet had increased numbers of IgA-producing plasma cells in the large intestine; this effect was abolished when serine palmitoyltransferase was inhibited. These findings suggest that dietary PA regulates intestinal IgA responses and has the potential to be a diet-derived mucosal adjuvant.

  20. Impaired selection of IgA and intestinal dysbiosis associated with PD-1-deficiency.

    Science.gov (United States)

    Maruya, Mikako; Kawamoto, Shimpei; Kato, Lucia M; Fagarasan, Sidonia

    2013-01-01

    A major function of immunoglobulin A (IgA) is to maintain balanced bacterial communities in the gut. We have previously shown that diversification of IgA upon somatic hypermutation (SHM) is critical for IgA function yet the principles governing the selection of IgA in the gut have remained elusive. Here we discuss recent progress in understanding this process as revealed by our studies in mice that lack the inhibitory co-receptor programmed cell death-1 (PD-1). We found that PD-1 affects the dynamics of germinal center (GC) B cells by controlling the number and the nature of T helper cells in the Peyer's patches (PPs). Deregulation of the T cell compartment impacts the selection of IgA plasma cells leading to gut dysbiosis. When the PD-1-dependent checkpoint is missing, gut bacteria go beyond the mucosal barrier and induce systemic GCs that can generate antibodies with auto-reactive properties.

  1. Improved prognosis of diabetic nephropathy in type 1 diabetes

    DEFF Research Database (Denmark)

    Andrésdóttir, Gudbjörg; Jensen, Majken L; Carstensen, Bendix

    2015-01-01

    -term renin-angiotensin system inhibition), lipids, and glycemia, along with less smoking and other lifestyle and treatment advancements, is inadequately analyzed. To clarify this, we studied 497 patients with type 1 diabetes and diabetic nephropathy at the Steno Diabetes Center and compared them...... and nephropathy onset occurred later in life, mortality was reduced by 30%. Risk factors for decline in glomerular filtration rate, death, and other renal end points were generally in agreement with prior studies. Thus, with current treatment of nephropathy in type 1 diabetes, the prognosis and loss of renal......The natural history of diabetic nephropathy offered an average survival of only 5-7 years. During the past decades, multiple changes in therapy and lifestyle have occurred. The prognosis of diabetic nephropathy after implementing stricter control of blood pressure (including increased use of long...

  2. Sulodexide ameliorates early but not late kidney disease in models of radiation nephropathy and diabetic nephropathy

    OpenAIRE

    Rossini, Michele; Naito, Takashi; Yang, Haichun; Freeman, Michael; Donnert, Ellen; Ma, Li-Jun; Dunn, Stephen R.; Sharma, Kumar; Fogo, Agnes B.

    2010-01-01

    Background. Sulodexide is a glycosaminoglycan with anticoagulant and antithrombotic activities. Although sulodexide reduced albuminuria in patients with type 1 and type 2 diabetes, long-term effects on chronic renal injury are not established. We investigated sulodexide effects and mechanisms in a rat radiation nephropathy model and in the db/db mouse model of diabetic kidney disease.

  3. Membranous nephropathy with predominance of C1q: another variant of C1q nephropathy?

    NARCIS (Netherlands)

    Deurwaarder, E.S. den; Steenbergen, E.; Hoogeveen, E.K.; Wetzels, J.F.M.

    2012-01-01

    Originally described as a proliferative glomerulonephritis, C1q nephropathy is nowadays mostly recognized as a variant of focal segmental glomerulosclerosis or minimal change disease. We describe a 30-year-old male patient with nephrotic range proteinuria. Kidney biopsy demonstrated a membranous nep

  4. Determination of levels of salivary IgA subclasses in patients with minor recurrent aphthous ulcer

    Directory of Open Access Journals (Sweden)

    Ramandeep Saluja

    2012-01-01

    Full Text Available Context: Recurrent Aphthous Ulcer (RAU is an inflammatory disease characterized by recurrent, painful oral ulcers. It is of multifactorial etiology. Salivary immunoglobulins have important role in the protection of mucosal surfaces. Aim: The aim of this study was to determine salivary immunoglobulin A1 (IgA1 and IgA2 in acute and remission phases of the disease. Materials and Methods: Thirty clinically confirmed cases of RAU and 30 age-and sex-matched controls were included in the study. After detailed case history and thorough clinical examination, 2 mL of saliva was collected in both acute and remission phases of the disease. The obtained saliva samples were subjected to quantification of IgA1 and IgA2 levels using RID kit. Results: The mean IgA2 level was significantly higher (P<.001 in both acute and remission phase of the study group. The mean IgA1 level also showed a significant increase in the acute phase compared to remission as well as controls (P<.05. Females exhibited a higher level in acute phase for IgA1 and in both phases for IgA2 (P<.05. Conclusion: The results associated with clinical observations suggest that acute phase is characterized with increase in IgA2 that might reflect increased immune response as a possible result of the microbial stimulation seen in the acute phase in comparison to the remission period. IgA plays an important role in the pathogenesis of RAU and it can be used as a parameter to assess the mucosal immune status

  5. Mechanisms of diabetic nephropathy--old buddies and newcomers part 2.

    Science.gov (United States)

    Nawroth, P P; Isermann, B

    2010-11-01

    The clinical translation of established pathomechanisms of diabetic nephropathy improved the outcome in patients with diabetic nephropathy. However, they fail to halt or even reverse diabetic nephropathy, even though the feasibility of disease reversal has been established. The second part of this review summarizes recent novel insights into the mechanisms of diabetic nephropathy focusing on novel candidate mechanisms of diabetic nephropathy. These studies emphasize a crucial role of endothelial dependent mechanisms, which, however, can not be viewed as independent determinants of diabetic nephropathy. Rather, the endothelial dependent mechanisms act in concert with other cellular systems, establishing an intra-glomerular cross-talk which determines the progression of diabetic nephropathy.

  6. An Anti-EGFR IgA That Displays Improved Pharmacokinetics and Myeloid Effector Cell Engagement In Vivo

    NARCIS (Netherlands)

    Lohse, Stefan; Meyer, Saskia; Meulenbroek, Laura A P M; Jansen, J H Marco; Nederend, Maaike; Kretschmer, Anna; Klausz, Katja; Möginger, Uwe; Derer, Stefanie; Rösner, Thies; Kellner, Christian; Schewe, Denis; Sondermann, Peter; Tiwari, Sanjay; Kolarich, Daniel; Peipp, Matthias; Leusen, Jeanette H W; Valerius, Thomas

    2016-01-01

    Antibodies of IgA isotype effectively engage myeloid effector cells for cancer immunotherapy. Here, we describe preclinical studies with an Fc engineered IgA2m(1) antibody containing the variable regions of the EGFR antibody cetuximab. Compared with wild-type IgA2m(1), the engineered molecule lacked

  7. [Evaluation of the new Hevylite™ IgA assay for the diagnosis and follow-up of monoclonal gammopathies].

    Science.gov (United States)

    Lakomy, Daniela; Lemaire-Ewing, Stéphanie; Denimal, Damien; Bastie, Jean-Noël; Lafon, Ingrid; Caillot, Denis

    2013-01-01

    Multiple myeloma diagnosis and follow-up are based on monoclonal protein measurement. The estimation of monoclonal immunoglobulin production requires serum protein electrophoresis, immunoelectrophoresis and free light chain assay. However these classical assays have some limitations. Hevylite™ IgA (Binding Site) is a new nephelometric/turbidimetric assay allowing the IgA κ and IgA λ measurement. The aim of this study was to determine the performance of this assay, for the diagnosis and follow-up of myeloma patients at different stages. Sixty seven frozen sera from 26 patients were assayed. Total IgA, IgA κ, IgA λ concentrations, serum protein electrophoresis and serum immunofixation were performed at diagnosis and during follow-up. All myeloma patients had an abnormal IgA κ/IgA λ ratio at diagnosis. During disease monitoring, the IgA κ or IgA λ concentrations correlated well with the electrophoretic estimation of the monoclonal spike and the values of total IgA. Hevylite™ test was more sensitive than serum protein electrophoresis and provided numerical and reproductible assessment of the monoclonal and non-monoclonal isotype. The IgA κ/IgA λ ratio allowed early prediction of disease relapse. Hevylite™ is an interesting assay especially when the monoclonal IgA comigrates on electrophoresis with normal proteins making impossible a reliable densitometric estimation. Hevylite™ might become an important assay in the biological exploration of gammopathies.

  8. Rodent models of diabetic nephropathy: their utility and limitations.

    Science.gov (United States)

    Kitada, Munehiro; Ogura, Yoshio; Koya, Daisuke

    2016-01-01

    Diabetic nephropathy is the most common cause of end-stage renal disease. Therefore, novel therapies for the suppression of diabetic nephropathy must be developed. Rodent models are useful for elucidating the pathogenesis of diseases and testing novel therapies, and many type 1 and type 2 diabetic rodent models have been established for the study of diabetes and diabetic complications. Streptozotocin (STZ)-induced diabetic animals are widely used as a model of type 1 diabetes. Akita diabetic mice that have an Ins2+/C96Y mutation and OVE26 mice that overexpress calmodulin in pancreatic β-cells serve as a genetic model of type 1 diabetes. In addition, db/db mice, KK-Ay mice, Zucker diabetic fatty rats, Wistar fatty rats, Otsuka Long-Evans Tokushima Fatty rats and Goto-Kakizaki rats serve as rodent models of type 2 diabetes. An animal model of diabetic nephropathy should exhibit progressive albuminuria and a decrease in renal function, as well as the characteristic histological changes in the glomeruli and the tubulointerstitial lesions that are observed in cases of human diabetic nephropathy. A rodent model that strongly exhibits all these features of human diabetic nephropathy has not yet been developed. However, the currently available rodent models of diabetes can be useful in the study of diabetic nephropathy by increasing our understanding of the features of each diabetic rodent model. Furthermore, the genetic background and strain of each mouse model result in differences in susceptibility to diabetic nephropathy with albuminuria and the development of glomerular and tubulointerstitial lesions. Therefore, the validation of an animal model reproducing human diabetic nephropathy will significantly facilitate our understanding of the underlying genetic mechanisms that contribute to the development of diabetic nephropathy. In this review, we focus on rodent models of diabetes and discuss the utility and limitations of these models for the study of diabetic

  9. Luo Lingjie's Experience in Treating Chronic Nephropathy

    Institute of Scientific and Technical Information of China (English)

    Cai Min; Yang Yonghe; Luo Lingjie; Duan Shumin

    2008-01-01

    @@ In treating chronic nephropathy,Luo Lingjie,a chief physician,pays attention to regulating the balance between yin and yang,treating infection if present,and removing pathogenic factors.He prescribes gentle drugs and uses carefully strongly warming-tonifying ones,emphasizes the importance of persuading the patient to persist in treatment with medication and nurse one's health for recuperation,and is good at combined use of TCM and western medicine therapy and brings the merits of various therapies into full play,with obvious theraoeutic effects.

  10. Gold nephropathy in juvenile rheumatoid arthritis.

    Science.gov (United States)

    Husserl, F E; Shuler, S E

    1979-01-01

    A 2-year-old girl was treated with gold salts for juvenile rheumatoid arthritis. Treatment had to be discontinued when persistent proteinuria was detected. As this case report indicates, close monitoring of the urine is mandatory during treatment with gold salts to detect early signs of toxicity: hematuria followed by casts and then proteinuria as therapy is continued. Histologic examination with electron microscopy will help to differentiate the different forms of gold toxicity. When the findings are consistent with gold-induced renal involvement, therapy should be discontinued. The gold nephropathy usually resolves in time, with no permanent renal damage.

  11. Danish Registry of Childhood and Adolescent Diabetes

    DEFF Research Database (Denmark)

    Svensson, Jannet; Cerqueira, Charlotte; Kjærsgaard, Per

    2016-01-01

    classification, family history of diabetes, growth parameters, self-care, and treatment variables. The quality indicators are selected based on international consensus of measures of good clinical practice. The indicators are metabolic control as assessed by HbA1c, blood pressure, albuminuria, retinopathy...... experiencing severe hypoglycemia, diabetic nephropathy, and retinopathy. CONCLUSION: The systematic collection of data in DanDiabKids documents improved quality of care over the last 12 years, despite a substantial increase in the number of patients cared for by pediatric departments in Denmark, fulfilling......AIM: The aims of the Danish Registry of Childhood and Adolescent Diabetes (DanDiabKids) are to monitor and improve the quality of care for children and adolescents with diabetes in Denmark and to follow the incidence and prevalence of diabetes. STUDY POPULATION: The study population consists of all...

  12. Human IgA inhibits adherence of Acanthamoeba polyphaga to epithelial cells and contact lenses.

    Science.gov (United States)

    Campos-Rodríguez, Rafael; Oliver-Aguillón, Gabriela; Vega-Pérez, Luz M; Jarillo-Luna, Adriana; Hernández-Martínez, Dolores; Rojas-Hernández, Saúl; Rodríguez-Monroy, Marco A; Rivera-Aguilar, Víctor; González-Robles, Arturo

    2004-09-01

    Specific anti-Acanthamoeba IgA antibodies have been detected in the serum and tears of patients and healthy individuals. However, the role of human secretory IgA antibodies in inhibiting the adherence of Acanthamoeba had not been previously investigated. Therefore, the purpose of this study was to purify secretory IgA from human colostrum and analyze its effect on the adherence of Acanthamoeba trophozoites to contact lenses and Madin-Darby canine kidney (MDCK) cells. IgA antibodies to Acanthamoeba polyphaga in colostrum of healthy women as well as in saliva and serum of healthy subjects were analyzed by ELISA and Western blot analysis. In serum, saliva, and colostrum, we detected IgA antibodies that recognized several antigens of A. polyphaga. In addition, colostrum and IgA antibodies purified from it inhibited adherence of A. polyphaga trophozoites to contact lenses and MDCK cells. These results suggest that IgA antibodies may participate in the resistance to the amoebic infection, probably by inhibiting the adherence of the trophozoites to contact lenses and corneal epithelial cells.

  13. Preventive Effect of Salicylate and Pyridoxamine on Diabetic Nephropathy.

    Science.gov (United States)

    Abouzed, Tarek Kamal; Munesue, Seiichi; Harashima, Ai; Masuo, Yusuke; Kato, Yukio; Khailo, Khaled; Yamamoto, Hiroshi; Yamamoto, Yasuhiko

    2016-01-01

    Objective. Diabetic nephropathy is a life-threatening complication in patients with long-standing diabetes. Hemodynamic, inflammatory, and metabolic factors are considered as developmental factors for diabetic nephropathy. In this study, we evaluated whether pharmacological interventions with salicylate, compared to pyridoxamine, could prevent diabetic nephropathy in mice. Methods. Male mice overexpressing inducible nitric oxide synthase in pancreatic β-cells were employed as a diabetic model. Salicylate (3 g/kg diet) or pyridoxamine (1 g/L drinking water; ~200 mg/kg/day) was given for 16 weeks to assess the development of diabetic nephropathy. Treatment with long-acting insulin (Levemir 2 units/kg twice a day) was used as a control. Results. Although higher blood glucose levels were not significantly affected by pyridoxamine, early to late stage indices of nephropathy were attenuated, including kidney enlargement, albuminuria, and increased serum creatinine, glomerulosclerosis, and inflammatory and profibrotic gene expressions. Salicylate showed beneficial effects on diabetic nephropathy similar to those of pyridoxamine, which include lowering blood glucose levels and inhibiting macrophage infiltration into the kidneys. Attenuation of macrophage infiltration into the kidneys and upregulation of antiglycating enzyme glyoxalase 1 gene expression were found only in the salicylate treatment group. Conclusions. Treatment with salicylate and pyridoxamine could prevent the development of diabetic nephropathy in mice and, therefore, would be a potentially useful therapeutic strategy against kidney problems in patients with diabetes.

  14. Reversal of diabetic nephropathy by a ketogenic diet.

    Directory of Open Access Journals (Sweden)

    Michal M Poplawski

    Full Text Available Intensive insulin therapy and protein restriction delay the development of nephropathy in a variety of conditions, but few interventions are known to reverse nephropathy. Having recently observed that the ketone 3-beta-hydroxybutyric acid (3-OHB reduces molecular responses to glucose, we hypothesized that a ketogenic diet, which produces prolonged elevation of 3-OHB, may reverse pathological processes caused by diabetes. To address this hypothesis, we assessed if prolonged maintenance on a ketogenic diet would reverse nephropathy produced by diabetes. In mouse models for both Type 1 (Akita and Type 2 (db/db diabetes, diabetic nephropathy (as indicated by albuminuria was allowed to develop, then half the mice were switched to a ketogenic diet. After 8 weeks on the diet, mice were sacrificed to assess gene expression and histology. Diabetic nephropathy, as indicated by albumin/creatinine ratios as well as expression of stress-induced genes, was completely reversed by 2 months maintenance on a ketogenic diet. However, histological evidence of nephropathy was only partly reversed. These studies demonstrate that diabetic nephropathy can be reversed by a relatively simple dietary intervention. Whether reduced glucose metabolism mediates the protective effects of the ketogenic diet remains to be determined.

  15. BMP-7 PROTEIN EXPRESSION IS DOWNREGULATED IN HUMAN DIABETIC NEPHROPATHY.

    Science.gov (United States)

    Ivanac-Janković, Renata; Ćorić, Marijana; Furić-Čunko, Vesna; Lovičić, Vesna; Bašić-Jukić, Nikolina; Kes, Petar

    2015-06-01

    Bone morphogenetic protein-7 (BMP-7) is expressed in all parts of the normal kidney parenchyma, being highest in the epithelium of proximal tubules. It protects kidney against acute and chronic injury, inflammation and fibrosis. Diabetic nephropathy is the leading cause of chronic kidney disease, and is characterized by decreased expression of BMP-7. The aim of our study was to analyze whether the expression of BMP-7 is significantly changed in advanced stages of human diabetic nephropathy. Immunohistochemical analysis of the expression of BMP-7 was performed on archival material of 30 patients that underwent renal biopsy and had confirmed diagnosis of diabetic nephropathy. Results showed that BMP-7 was differently expressed in the cytoplasm of epithelial cells of proximal tubules and podocytes among all stages of diabetic nephropathy. At early stages of diabetic nephropathy, BMP-7 was strongly positive in proximal tubules and podocytes, while low expression was recorded in the majority of samples at advanced stages. In conclusion, increased expression of BMP-7 at initial stages of diabetic nephropathy with subsequent decrease at advanced stage highlights the role of BMP-7 in the protection of kidney structure and function. Further investigations should be focused on disturbances of BMP-7 receptors and signaling pathways in patients with diabetic nephropathy.

  16. Diabetic Nephropathy: New Risk Factors and Improvements in Diagnosis.

    Science.gov (United States)

    Tziomalos, Konstantinos; Athyros, Vasilios G

    2015-01-01

    Diabetic nephropathy is the leading cause of end-stage renal disease. Patients with diabetic nephropathy have a high cardiovascular risk, comparable to patients with coronary heart disease. Accordingly, identification and management of risk factors for diabetic nephropathy as well as timely diagnosis and prompt management of the condition are of paramount importance for effective treatment. A variety of risk factors promotes the development and progression of diabetic nephropathy, including elevated glucose levels, long duration of diabetes, high blood pressure, obesity, and dyslipidemia. Most of these risk factors are modifiable by antidiabetic, antihypertensive, or lipid-lowering treatment and lifestyle changes. Others such as genetic factors or advanced age cannot be modified. Therefore, the rigorous management of the modifiable risk factors is essential for preventing and delaying the decline in renal function. Early diagnosis of diabetic nephropathy is another essential component in the management of diabetes and its complications such as nephropathy. New markers may allow earlier diagnosis of this common and serious complication, but further studies are needed to clarify their additive predictive value, and to define their cost-benefit ratio. This article reviews the most important risk factors in the development and progression of diabetic nephropathy and summarizes recent developments in the diagnosis of this disease.

  17. Childhood sarcoidosis: Louisiana experience.

    Science.gov (United States)

    Gedalia, Abraham; Khan, Tahir A; Shetty, Avinash K; Dimitriades, Victoria R; Espinoza, Luis R

    2016-07-01

    A retrospective chart review was conducted to detect patients with sarcoidosis seen by pediatric rheumatology service from the period of 1992 to 2013 at Children's hospital of New Orleans. Twenty-seven patients were identified. The average duration of symptoms before diagnosis was 5 (range 1-120) months. Five patients had onset before the age of 5 years and were diagnosed with early-onset sarcoidosis. The most common manifestations at presentation were constitutional symptoms (62 %) followed by ocular (38 %). During the course of illness, 19/27 (70 %) had multiorgan involvement. Common manifestations included uveitis/iritis (77 %), fever (50 %), hilar adenopathy (42 %), arthritis (31 %), peripheral lympadenopathy (31 %), hepatosplenomegaly (31 %), parenchymal lung disease (27 %), and skin rash (19 %). Unusual manifestations included granulomatous bone marrow disease (3 cases), hypertension (2), abdominal aortic aneurysm (large vessel vasculitis; 1), granulomatous hepatitis (1), nephrocalcinosis (1), membranous nephropathy (1), refractory granulomatous interstitial nephritis with recurrence in transplanted kidney (1), CNS involvement (2), parotid gland enlargement (1), and sensorineural hearing loss (1). Biopsy specimen was obtained in 21/27 (77 %) patients, and demonstration of noncaseating granuloma associated with negative stains for mycobacteria and fungi was seen in 18 patients. Elevated angiotensin-converting enzyme level was seen in 74 % of patients. Treatment with oral prednisone was initiated in symptomatic patients with significant clinical improvement. Low-dose methotrexate (MTX) 10-15 mg/m(2)/week orally, as steroid-sparing agent, was administered in 14 patients. Other immunomodulators included cyclophosphamide (2 patients), etanercept (2), infliximab (2), mycophenolate mofetil (1), and tacrolimus (1). Childhood sarcoidosis is prevalent in Louisiana. Most of the affected children present with a multisystem disease associated with

  18. Smoking in diabetic nephropathy: sparks in the fuel tank?

    Science.gov (United States)

    Chakkarwar, Vishal Arvind

    2012-12-15

    Diabetic nephropathy is associated with high morbidity and mortality and the prevalence of this disease is continuously increasing worldwide. Long-term diabetes increases the likelihood of developing secondary complications like nephropathy, the most common cause of end stage renal disease. Usually, other factors like hypertension, alcoholism and smoking also partly contribute to the progression of diabetic nephropathy. Among this, cigarette smoking in diabetes has been repeatedly confirmed as an independent risk factor for the onset and progression of diabetic nephropathy. Various studies suggest that smoking is a major fuel in the development of high oxidative stress and subsequently hyperlipidemia, accumulation of advanced glycation end products, activation of the renin angiotensin system and Rho-kinase, which are observed to play a pathogenic role in the progression of diabetic nephropathy. Furthermore, cigarette smoking in diabetic patients with vascular complications produces a variety of pathological changes in the kidney, such as thickening of the glomerular basement membrane and mesangial expansion with progression in glomerulosclerosis and interstitial fibrosis, which ultimately results in end stage renal failure. Strong associations are consistently found between chronic cigarette smoking and diabetic microvascular complications. A diverse group of studies unveil potential mechanisms that may explain the role of cigarette smoking in the progression of diabetic nephropathy. Tremendous efforts are being made to control smoking mediated progression of diabetic nephropathy, but no promising therapy is yet available. The present review critically discusses the possible detrimental role of chronic cigarette smoking in the progression of diabetic nephropathy and various possible pharmacological interventions to attenuate the exacerbation of diabetic nephropathy.

  19. Management of nephropathy in patients with type 2 diabetes

    Institute of Scientific and Technical Information of China (English)

    2002-01-01

    Purpose To review evidence-based management of nephropathy in patients with type 2 diabetes. Data sources A literature search (MEDLINE 1966 to 2000) was performed using the key word “diabetic nephropathy". Relevant book chapters were also reviewed. Study selection Well-controlled, prospective landmark studies and expert review articles on diabetic nephropathy were selected. Data extraction Data and conclusions from the selected articles that provide solid evidence to the optimal management of diabetic nephropathy were extracted and interpreted in light of our clinical research experience with many thousands of Hong Kong Chinese patients. Results Hypertension, long diabetes duration, poor glycaemic control and central obesity are the most important risk factors. Microalbuminuria is a practical marker to predict overt nephropathy in type 2 diabetic patients. Risk factor modification, renal function monitoring and combined therapies are the current integrated approaches to manage patients with diabetic kidney disease. Optimal glycaemic control is the mainstay of treatment but effective antihypertensive therapy is also key to delaying the progression of diabetic nephropathy. Angiotensin-converting enzyme inhibitors and angiotensin Ⅱ receptor antagonists have important renoprotective actions independent of their blood pressure lowering actions. Conclusions Diabetic nephropathy is the leading cause of end-stage renal disease worldwide. Monitoring renal function and screening for microalbuminuria will allow the identification of patients with nephropathy at a very early stage for intervention. Tight glycaemic control and aggressive antihypertensive treatment as well as the use of renin-angiotensin system inhibitors should substantially delay the progression of nephropathy.

  20. Biomarkers in diabetic nephropathy: Present and future

    Institute of Scientific and Technical Information of China (English)

    Gemma; Currie; Gerard; Mc; Kay; Christian; Delles

    2014-01-01

    Diabetic nephropathy(DN) is the leading cause of end stage renal disease in the Western world. Microalbuminuria(MA) is the earliest and most commonly used clinical index of DN and is independently associated with cardiovascular risk in diabetic patients. Although MA remains an essential tool for risk stratification and monitoring disease progression in DN, a number of factors have called into question its predictive power. Originally thought to be predictive of future overt DN in 80% of patients, we now know that only around 30% of microalbuminuric patients progress to overt nephropathy after 10 years of follow up. In addition, advanced structural alterations in the glomerular basement membrane may already have occurred by the time MA is clinically detectable.Evidence in recent years suggests that a significant proportion of patients with MA can revert to normoalbuminuria and the concept of nonalbuminuric DN is well-documented, reflecting the fact that patients with diabetes can demonstrate a reduction in glomerular filtration rate without progressing from normo-to MA. There is an unmet clinical need to identify biomarkers with potential for earlier diagnosis and risk stratification in DN and recent developments inthis field will be the focus of this review article.

  1. A REVIEW ON DIABETIC NEUROPATHY AND NEPHROPATHY

    Directory of Open Access Journals (Sweden)

    Mohd. Muneer Ahamed

    2012-01-01

    Full Text Available Diabetes is a major public health problem. Diabetes mellitus now affects large number of people in many developing countries than western countries where only two or three percent of the population is affected. With on estimated 33 million people in India alone affected by diabetes. It is a major epidemic of the twentieth century. Diabetes is a chronic disorder, which is associated with obesity, hypertension, advancing age, accumulation of harmful agents in the vascular endothelium causing development of microangiopathies or micro vascular complications. These complications include peripheral neuropathy, nephropathy and retinopathy, which cause early death and increased morbidity. These complications vary in prevalence in different populations depending on various factors such as genetic predisposition and ethnicity. Besides these complications cardiovascular changes are also occurring. Peripheral neuropathy (PN is characterized by pain, numbness, and tingling in the extremities with slow nerve conduction. Up to 50% of all patients with diabetes develop neuropathy and the prevalence of painful neuropathy ranges from 10 to 20% of patients with diabetes. Diabetic nephropathy is characterized by increased urinary protein, loss of renal function, excessive deposition of extracellular matrix proteins in the mesangium, and clear cytoplasm of the proximal tubular epithelial cells due to excessive reabsorbed glycogen. Evaluation of diabetes and its complications is very essential for proper control and prevention of the disease associated complications.

  2. Vancomycin-associated linear IgA disease mimicking toxic epidermal necrolysis*

    Science.gov (United States)

    Pereira, Amanda Regio; de Moura, Luis Henrique Barbizan; Pinheiro, Jhonatan Rafael Siqueira; Pasin, Victor Pavan; Enokihara, Milvia Maria Simões e Silva; Porro, Adriana Maria

    2016-01-01

    Linear IgA dermatosis is a rare subepidermal autoimmune blistering disease characterized by linear deposition of IgA along the basement membrane zone. In the last three decades, many different drugs have been associated with the drug-induced form of the disease, especially vancomycin. We report a case of vancomycin-induced linear IgA disease mimicking toxic epidermal necrolysis. The aim of this work is to emphasize the need to include this differential diagnosis in cases of epidermal detachment and to review the literature on the subject and this specific clinical presentation. PMID:28300888

  3. Localized linear IgA dermatosis induced by UV light-treatment for herpes zoster.

    Science.gov (United States)

    He, Chundi; Xu, Honghui; Xiao, Ting; Geng, Long; Chen, Hong-Duo

    2007-05-01

    We report a case of localized linear IgA dermatosis (LID). The patient suffered from herpes zoster on the right waist and received three localized ultraviolet (UV) light treatments. One month later he presented with bullae on the same site. Direct immunofluorescence showed deposition of linear IgA and weak C3 along the basement membrane zone. Indirect immunofluorescence on the salt-split human skin demonstrated that IgA antibodies were bound to the epidermal side. To our knowledge, this is the first case of localized LID induced by UV light treatment for herpes zoster. It is also the third case of LID induced by UV light.

  4. Oxidative Stress in Diabetic Nephropathy with Early Chronic Kidney Disease

    OpenAIRE

    Miranda-Díaz, Alejandra Guillermina; Pazarín-Villaseñor, Leonardo; Yanowsky-Escatell, Francisco Gerardo; Andrade-Sierra, Jorge

    2016-01-01

    The increase in the prevalence of diabetes mellitus (DM) and the secondary kidney damage produces diabetic nephropathy (DN). Early nephropathy is defined as the presence of microalbuminuria (30–300 mg/day), including normal glomerular filtration rate (GFR) or a mildly decreased GFR (60–89 mL/min/1.73 m2), with or without overt nephropathy. The earliest change caused by DN is hyperfiltration with proteinuria. The acceptable excretion rate of albumin in urine is 300 mg/day. Chronic kidney disea...

  5. Long-term renoprotective effects of losartan in diabetic nephropathy

    DEFF Research Database (Denmark)

    Andersen, S.; Tarnow, Lise; Cambien, F.

    2003-01-01

    139/74 mmHg (14/8) in both genotype groups during the study (P treatment with losartan has similar beneficial renoprotective effects on progression of diabetic nephropathy in hypertensive...... of the angiotensin II subtype-1 (AT1) receptor antagonist losartan (100 mg o.d.) on kidney function in II and DD type 1 diabetic patients with diabetic nephropathy. RESEARCH DESIGN AND METHODS: A total of 54 hypertensive type 1 diabetic patients with diabetic nephropathy homozygous for the insertion (n = 26...... type 1 diabetic patients with ACE II and DD genotypes....

  6. Relationship between E-cadherin and diabetic nephropathy

    Institute of Scientific and Technical Information of China (English)

    姜洪娟

    2014-01-01

    Objective To identify novel biomarker for diabetic nephropathy(DN)by urinary proteomic methods,and to detect the expression of E-cadherin in urine and renal tissue of patients with DN.Methods Urine samples were collected from 12 cases of type 1 diabetic nephropathy patients(T1DN),12 cases of type 2 diabetic nephropathy patients(T2DN),12 cases of nephritic syndrome patients(NS),and 12 cases of healthy Controls.Comparative proteomic approach of two-dimensional gel electro-

  7. Childhood-onset bullous systemic lupus erythematosus.

    Science.gov (United States)

    Lourenço, D M R; Gomes, R Cunha; Aikawa, N E; Campos, L M A; Romiti, R; Silva, C A

    2014-11-01

    Bullous systemic lupus erythematosus has rarely been described in pediatric lupus population and the real prevalence of childhood-onset bullous systemic lupus erythematosus has not been reported. From January 1983 to November 2013, 303 childhood-onset SLE (c-SLE) patients were followed at the Pediatric Rheumatology Unit of the Childreńs Institute of Hospital das Clínicas da Faculdade de Medicina Universidade da Universidade de São Paulo, three of them (1%) diagnosed as childhood-onset bullous systemic lupus erythematosus. All three cases presented tense vesiculobullous lesions unassociated with lupus erythematosus lesions, with the median duration of 60 days (30-60). All patients fulfilled bullous systemic lupus erythematosus criteria. Two had nephritis and serositis and presented specific autoantibodies. The histological pattern demonstrated subepidermal blisters with neutrophils-predominant infiltrates within the upper dermis. Direct immunofluorescence (DIF) showed deposits of IgG and complement along the epidermal basement membrane, in the presence or absence of IgA and/or IgM. A positive indirect immunofluorescence on salt-split skin demonstrating dermal binding was observed in two cases. All of them had moderate/severe disease activity at diagnosis with median Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) of 18 (14-24). Two patients received dapsone and one with severe nephritis received immunosuppressive drugs. In conclusion, in the last 30 years the prevalence of bullous lupus in childhood-onset lupus population was low (1%) in our tertiary University Hospital. A diagnosis of SLE should always be considered in children with recurrent tense vesiculobullous lesions with or without systemic manifestations.

  8. Autophagy as a Therapeutic Target in Diabetic Nephropathy

    Directory of Open Access Journals (Sweden)

    Yuki Tanaka

    2012-01-01

    Full Text Available Diabetic nephropathy is a serious complication of diabetes mellitus, and its prevalence has been increasing worldwide. Therefore, there is an urgent need to identify a new therapeutic target to prevent diabetic nephropathy. Autophagy is a major catabolic pathway involved in degrading and recycling macromolecules and damaged organelles to maintain intracellular homeostasis. The study of autophagy in mammalian systems is advancing rapidly and has revealed that it is involved in the pathogenesis of various metabolic or age-related diseases. The functional role of autophagy in the kidneys is also currently under intense investigation although, until recently, evidence showing the involvement of autophagy in the pathogenesis of diabetic nephropathy has been limited. We provide a systematic review of autophagy and discuss the therapeutic potential of autophagy in diabetic nephropathy to help future investigations in this field.

  9. A rapid screening test for detection of IgA deficiency.

    Science.gov (United States)

    Schulenburg, B J; Plapp, F V; Rachel, J M

    1991-09-01

    A solid-phase red cell adherence (SPRCA) assay has been developed to screen blood donors for IgA deficiency, and 6117 donor sera have been screened by this method. Eighteen sera were found to be IgA deficient, which represents a frequency of 1 in 340. Seventeen of these sera were retested by passive hemagglutination inhibition, which has a sensitivity of approximately 0.1 mg per dL. Eight sera were confirmed as IgA deficient, and nine were found to contain low levels of IgA (less than 1 mg/dL). The approximate sensitivity of the SPRCA assay is 1 mg per dL. The speed, simplicity, and sensitivity of this assay make it a good alternative to conventional methods of screening.

  10. Linear IgA dermatosis associated with ulcerative colitis: complete and sustained remission after total colectomy.

    Science.gov (United States)

    Vargas, Thiago Jeunon de Sousa; Fialho, Mônica; Santos, Luiza Tavares dos; Rodrigues, Palmira Assis de Jesus Barreto; Vargas, Ana Luisa Bittencourt Sampaio Jeunon; Sousa, Maria Auxiliadora Jeunon

    2013-01-01

    Linear IgA dermatosis has been increasingly associated with inflammatory bowel diseases, particularly ulcerative colitis. A 13-year-old male patient with an 11-month history of ulcerative colitis developed vesicles, pustules and erosions on the skin of the face, trunk and buttocks and in the oral mucosa. The work-up revealed a neutrophil-rich sub-epidermal bullous disease and linear deposition of IgA along the dermoepidermal junction, establishing the diagnosis of linear IgA dermatosis. The patient experienced unsatisfactory partial control of skin and intestinal symptoms despite the use of adalimumab, mesalazine, prednisone and dapsone for some months. After total colectomy, he presented complete remission of skin lesions, with no need of medications during two years of follow-up. A review of previously reported cases of the association is provided here and the role of ulcerative colitis in triggering linear IgA dermatosis is discussed.

  11. Iga seitsmes kindlustusvõtja üritab seltsi petta / Erkki Erilaid

    Index Scriptorium Estoniae

    Erilaid, Erkki

    2003-01-01

    Kindlustusseltside hinnangul üritab iga seitsmes kahjukindlustusvõtja kas kahju suurendada või pärast õnnetust tagantjärele kindlustust vormistades seltse petta. Vt. samas: Näiteid petuskeemidest

  12. Analüütikud : EL-iga ühinemine kiirendab majanduskasvu / Erkki Erilaid

    Index Scriptorium Estoniae

    Erilaid, Erkki

    2001-01-01

    Juhtivate majandusanalüütikute hinnangul kasvab Eesti majandus tänu abiprogrammidele ja välisinvesteeringutele kiiremini juhul, kui Eesti liitub EL-iga. Vt. samas Teet Rajasalu art. Majandus ja EL

  13. Iga sündiv laps muudab pere vaesemaks / Hille Tänavsuu

    Index Scriptorium Estoniae

    Tänavsuu, Hille, 1941-2014

    2000-01-01

    Iga uue lapse sünniga tekib oht, et pere langeb allapoole vaesuspiiri, ütleb ÜRO tellimusel valminud kogumiku "Lapsed Eestis" peatoimetaja Dagmar Kutsar. Lisaks skeem : Leibkonnad - leibkondade jaotus laste arvu järgi (protsentides)

  14. Euroliidu odava aseaine tunneb ära iga riik / Urmas Paet

    Index Scriptorium Estoniae

    Paet, Urmas, 1974-

    2007-01-01

    Eesti välisminister Urmas Paet kirjutab Euroopa identiteedist, Euroopa Liidu laienemisest, selle toetamise vajalikkusest ja Türgi ning Balkanimaade perspektiivist EL-iga liitumisel, euroopalike reformide toimumisest neis riikides

  15. Rodent models of diabetic nephropathy: their utility and limitations

    Directory of Open Access Journals (Sweden)

    Kitada M

    2016-11-01

    Full Text Available Munehiro Kitada,1,2 Yoshio Ogura,2 Daisuke Koya1,2 1Division of Anticipatory Molecular Food Science and Technology, Medical Research Institute, 2Department of Diabetology and Endocrinology, Kanazawa Medical University, Uchinada, Ishikawa, Japan Abstract: Diabetic nephropathy is the most common cause of end-stage renal disease. Therefore, novel therapies for the suppression of diabetic nephropathy must be developed. Rodent models are useful for elucidating the pathogenesis of diseases and testing novel therapies, and many type 1 and type 2 diabetic rodent models have been established for the study of diabetes and diabetic complications. Streptozotocin (STZ-induced diabetic animals are widely used as a model of type 1 diabetes. Akita diabetic mice that have an Ins2+/C96Y mutation and OVE26 mice that overexpress calmodulin in pancreatic β-cells serve as a genetic model of type 1 diabetes. In addition, db/db mice, KK-Ay mice, Zucker diabetic fatty rats, Wistar fatty rats, Otsuka Long-Evans Tokushima Fatty rats and Goto-Kakizaki rats serve as rodent models of type 2 diabetes. An animal model of diabetic nephropathy should exhibit progressive albuminuria and a decrease in renal function, as well as the characteristic histological changes in the glomeruli and the tubulointerstitial lesions that are observed in cases of human diabetic nephropathy. A rodent model that strongly exhibits all these features of human diabetic nephropathy has not yet been developed. However, the currently available rodent models of diabetes can be useful in the study of diabetic nephropathy by increasing our understanding of the features of each diabetic rodent model. Furthermore, the genetic background and strain of each mouse model result in differences in susceptibility to diabetic nephropathy with albuminuria and the development of glomerular and tubulointerstitial lesions. Therefore, the validation of an animal model reproducing human diabetic nephropathy will

  16. Telmisartan in the management of diabetic nephropathy: a contemporary view.

    Science.gov (United States)

    Balakumar, Pitchai; Bishnoi, Harish K; Mahadevan, Nanjaian

    2012-05-01

    Diabetic nephropathy, a complex disorder with heterogeneous etiologies, remains one of the most threatening diseases worldwide. There were around 177 million people with diabetes mellitus worldwide, and it has been estimated to be increased to 360 million by 2030. Given that about 20-30% of these people develop diabetic nephropathy, the present treatment protocols primarily aim for an efficient glucose and blood pressure control to arrest the initiation and progression of diabetic nephropathy. The treatment of diabetic nephropathy near the beginning at microalbuminuria stage with angiotensin-II-AT1 receptor blockers (ARBs) improves blood pressure control and halts disease progression of diabetic nephropathy. In fact, ARBs exert renoprotective effects independently of their blood pressure lowering effect, as they have direct defensive action on the diabetic kidney. Indubitably, it would be better if an ARB has both glucose-lowering and blood pressure controlling potentials efficiently. Intriguingly, telmisartan has such possessions considering its dual role of AT1 receptor blocking action and peroxisome proliferator-activated receptor gamma (PPARγ) partial agonistic property. The additional PPARγ agonistic potential of telmisartan could make it a distinctive intervention in the ARB class to prevent the progression of diabetic nephropathy through activation of PPARγ-mediated insulin sensitization, and renal anti-inflammatory and anti-oxidant actions. Indeed, telmisartan reduced insulin resistance and glucose intolerance, and halted the progressive renal dysfunction associated with diabetic nephropathy by inhibiting the incidence of albuminuria, and preventing the progression of glomerulosclerosis, renal interstitial inflammation and fibrosis. This review will discuss the current status of therapeutic potentials of telmisartan in treating diabetic nephropathy.

  17. Nutritional Intervention for a Patient with Diabetic Nephropathy

    OpenAIRE

    Kim, Hee Young

    2014-01-01

    In recent years, several studies have reported that the prevalence of diabetes mellitus is increasing every year, and also the acute and chronic complications accompanying this disease are increasing. Diabetic nephropathy is one of chronic complications of diabetes mellitus, and food intake which is burden to kidney function should be limited. At the same time, diet restriction could deteriorate quality of life of patient with diabetic nephropathy. According to the results of previous studies...

  18. Rodent models of diabetic nephropathy: their utility and limitations

    OpenAIRE

    Kitada M; Ogura Y; Koya D

    2016-01-01

    Munehiro Kitada,1,2 Yoshio Ogura,2 Daisuke Koya1,2 1Division of Anticipatory Molecular Food Science and Technology, Medical Research Institute, 2Department of Diabetology and Endocrinology, Kanazawa Medical University, Uchinada, Ishikawa, Japan Abstract: Diabetic nephropathy is the most common cause of end-stage renal disease. Therefore, novel therapies for the suppression of diabetic nephropathy must be developed. Rodent models are useful for elucidating the pathogenesis of diseases and test...

  19. Food allergy and probiotics in childhood.

    Science.gov (United States)

    del Giudice, Michele Miraglia; Leonardi, Salvatore; Maiello, Nunzia; Brunese, Francesco Paolo

    2010-09-01

    Food allergy is a frequent problem in childhood and its prevalence is increasing. In most cases food allergy is an IgE-mediated hypersensitivity response that cause skin reactions as urticaria. Subacute or chronic disorders have generally a not IgE mediated mechanism. Milk is the most common food allergen in USA and UK followed by egg, peanut and walnuts. Sensitization to milk or egg in infancy is associated with an increased risk to develop house dust mite sensitization and asthma later in childhood. Commensal gut flora play a role in induction of oral tolerance and the importance of the intestinal microbiota in the development of food allergy is essential in early ages, when the mucosal barrier and immune system are still immature. Probiotics interact with the mucosal immune system by the same pathways as commensal bacteria. Recent study show that probiotic bacteria induced in vivo increased plasma levels IL-10 and total IgA in children with allergic predisposition. Many clinical studies reporting significant benefits by probiotics supplementation in food allergy prevention and management but not everyone agree on their effectiveness. These differences are probably related to differences in selected populations and in probiotic strains used.

  20. Role of Toll-like receptors in diabetic nephropathy.

    Science.gov (United States)

    Mudaliar, Harshini; Pollock, Carol; Panchapakesan, Usha

    2014-05-01

    Diabetic nephropathy is the leading cause of kidney failure and its increasing prevalence and incidence has imposed global socio-economic stress on healthcare systems worldwide. Although historically considered a metabolic disorder, recent studies have established that inflammatory responses are central to the pathogenesis of diabetic nephropathy. TLRs (Toll-like receptors) are a family of pattern recognition receptors responsible for the initiation of inflammatory and immune responses. The regulation of TLR2 and TLR4 have been implicated in the pathogenesis of various kidney diseases, and emerging evidence shows their involvement in the perpetuation of inflammation in the diabetic kidney. The present review focuses on the relative contributions of TLR2 and TLR4 in recognizing endogenous ligands relevant to diabetic nephropathy and their subsequent activation of NF-κB (nuclear factor κB), which results in the synthesis and secretion of pro-inflammatory cytokines and chemokines. Moreover, we discuss the pro-inflammatory signalling pathways of TLR2 and TLR4, in which their interruption or blockade may prove to be important therapeutic targets, potentially translated into clinical treatments for diabetic nephropathy. Currently, inhibitors to TLR2 and TLR4 are undergoing clinical trials in various inflammatory models of disease, but none in patients with diabetic nephropathy. Given the existing literature, there is a fundamental necessity to undertake trials in patients with diabetic nephropathy with a focus on renal end points.

  1. Reactivities of N-acetylgalactosamine-specific lectins with human IgA1 proteins

    DEFF Research Database (Denmark)

    Moore, J.S.; Kulhavy, R.; Tomana, M.;

    2007-01-01

    exhibit binding heterogeneity, depending on their source and methods of isolation. To characterize potential differences in recognition of terminal N-acetylgalactosamine (GalNAc) on IgA1, we evaluated the binding characteristics of several commercial preparations of GalNAc-specific lectins using a panel...... of IgA1 and, as controls, IgA2 and IgG myeloma proteins. These lectins originated from snails Helix aspersa (HAA) and Helix pomatia (HPA), and the plant Vicia villosa (VV). Only HAA and HPA bound exclusively to IgA1, with its O-linked glycans composed of GalNAc, galactose, and sialic acid. In contrast......, VV reacted with sugars of both IgA subclasses and IgG, indicating that it also recognized N-linked glycans without GalNAc. Furthermore, HAA and HPA from several manufacturers differed in their ability to bind various IgA1 myeloma proteins and other GalNAc-containing glycoproteins in ELISA and Western...

  2. [Diagnosis and management of diabetic nephropathy].

    Science.gov (United States)

    Kitada, Munehiro; Koya, Daisuke

    2015-12-01

    Diabetic nephropathy(DN) is the most common cause of chronic kidney disease, leading to end-stage renal disease (ESRD) and cardiovascular disease (CVD). The overall number of patients with DN will continue to increase in parallel with the increasing global pandemic of type 2 diabetes. The detection of albuminuria is most important for diagnosis of early stage of DN, and also estimated glomerular filtration rate (eGFR) should be assessed, because both albuminuria and reduction of GFR are recognized as the independent risk factor for progression of ESRD and initiation of CVD, respectively. Based on landmark clinical trials, both DN and CVD have become preventable by controlling conventional factors, including hyperglycemia targeting HbA1c<7.0%, hypertenstion using renin angiotensin system inhibitors, dyslipidemia using statins or fibrates, and multifactorial treatment.

  3. Computed tomographical evaluation of diabetic nephropathy

    Energy Technology Data Exchange (ETDEWEB)

    Ubara, Yoshifumi; Hara, Shigeko; Arizono, Kenji; Katori, Hideyuki; Yamada, Akira; Mimura, Nobuhide [Toranomon Hospital, Tokyo (Japan). Kidney Center; Hagura, Ryoko

    1996-06-01

    Diabetic nephropathy can be regarded mainly as a type of microangiopathy, but is a disease that may also include aspects of macroangiopathy. This is especially true of renal disease in non-insulin dependent diabetes mellitus (NIDDM), which is characterized not only by diabetic glomerulosclerosis, but also by atherosclerosis. We performed morphological studies on the kidney, using computed tomography (CT), focusing on such points as: abdominal aortic calcifications at the level of kidney, calcifications in the renal artery, and wedge-shaped defects on the renal surface. We noted that these findings became more prominent in NIDDM patients during end-stage renal failure than during normal renal function, and were significantly more common in those two NIDDM groups than in age-matched nondiabetic patients without hypertension, hyperlipidemia or gout. NIDDM patients exhibited these features more frequently than IDDM patients. (author)

  4. Treatment of membranous nephropathy in children.

    Science.gov (United States)

    Makker, Sudesh P

    2003-07-01

    Membranous nephropathy (MN) is not a common pediatric glomerular disease and not a common cause of idiopathic nephrotic syndrome (NS) in children. Because of the rarity of the disease, there is only a limited amount of uncontrolled data and no controlled data available in children regarding the treatment of MN. Older uncontrolled data indicate that nearly a quarter of children with NS, whether untreated or treated with various immunosuppressive agents, develop chronic renal failure. Current recommendations for treatment both for children presenting with or without NS therefore are based on controlled data obtained in adults with MN. All children should receive angiotensin-converting enzyme (ACE) inhibitors or angiotensin-receptor blockers (ARBs). Children with NS may be treated initially with corticosteroids. If a satisfactory response is not obtained with corticosteroids, then treatment with cyclosporine or chlorambucil can be tried. The protocols of treatment with these drugs are described in this article.

  5. BK Virus Nephropathy in Heart Transplant Recipients.

    Science.gov (United States)

    Joseph, Alin; Pilichowska, Monika; Boucher, Helen; Kiernan, Michael; DeNofrio, David; Inker, Lesley A

    2015-06-01

    Polyomavirus-associated nephropathy (PVAN) has become an important cause of kidney failure in kidney transplant recipients. PVAN is reported to affect 1% to 7% of kidney transplant recipients, leading to premature transplant loss in approximately 30% to 50% of diagnosed cases. PVAN occurring in the native kidneys of solid-organ transplant recipients other than kidney only recently has been noted. We report 2 cases of PVAN in heart transplant recipients, which brings the total of reported cases to 7. We briefly review the literature on the hypothesized causes of PVAN in kidney transplant recipients and comment on whether these same mechanisms also may cause PVAN in other solid-organ transplant recipients. PVAN should be considered in the differential diagnosis when evaluating worsening kidney function. BK viremia surveillance studies of nonkidney solid-organ recipients should be conducted to provide data to assist the transplantation community in deciding whether regular monitoring of nonkidney transplant recipients for BK viremia is indicated.

  6. Diabetic Nephropathy in Women With Preexisting Diabetes

    DEFF Research Database (Denmark)

    Ringholm, Lene; Damm, Julie Agner; Vestgaard, Marianne

    2016-01-01

    In women with preexisting diabetes and nephropathy or microalbuminuria, it is important to deliver careful preconception counselling to assess the risk for the mother and the foetus, for optimizing glycaemic status and to adjust medical treatment. If serum creatinine is normal in early pregnancy......, kidney function is often preserved during pregnancy, but complications such as severe preeclampsia and preterm delivery are still common. Perinatal mortality is now comparable with that in women with diabetes and normal kidney function. Besides strict glycaemic control before and during pregnancy, early...... and intensive antihypertensive treatment is important to optimize pregnancy outcomes. Methyldopa, labetalol, nifedipine and diltiazem are considered safe, whereas angiotensin-converting enzyme (ACE) inhibitors or angiotensin receptor blockers should be stopped before or at confirmation of pregnancy...

  7. Complex networks analysis of obstructive nephropathy data

    Science.gov (United States)

    Zanin, M.; Boccaletti, S.

    2011-09-01

    Congenital obstructive nephropathy (ON) is one of the most frequent and complex diseases affecting children, characterized by an abnormal flux of the urine, due to a partial or complete obstruction of the urinary tract; as a consequence, urine may accumulate in the kidney and disturb the normal operation of the organ. Despite important advances, pathological mechanisms are not yet fully understood. In this contribution, the topology of complex networks, based on vectors of features of control and ON subjects, is related with the severity of the pathology. Nodes in these networks represent genetic and metabolic profiles, while connections between them indicate an abnormal relation between their expressions. Resulting topologies allow discriminating ON subjects and detecting which genetic or metabolic elements are responsible for the malfunction.

  8. Advances in Murine Models of Diabetic Nephropathy

    Directory of Open Access Journals (Sweden)

    Li-li Kong

    2013-01-01

    Full Text Available Diabetic nephropathy (DN is one of the microvascular complications of both type 1 and type 2 diabetes, which is also associated with a poor life expectancy of diabetic patients. However, the pathogenesis of DN is still unclear. Thus, it is of great use to establish appropriate animal models of DN for doing research on pathogenesis and developing novel therapeutic strategies. Although a large number of murine models of DN including artificially induced, spontaneous, and genetically engineered (knockout and transgenic animal models have been developed, none of them develops renal changes sufficiently reflecting those seen in humans. Here we review the identified murine models of DN from the aspects of genetic background, type of diabetes, method of induction, gene deficiency, animal age and gender, kidney histopathology, and phenotypic alterations in the hope of enhancing our comprehension of genetic susceptibility and molecular mechanisms responsible for this disease and providing new clues as to how to choose appropriate animal models of DN.

  9. Mouse Models for Studying Diabetic Nephropathy.

    Science.gov (United States)

    Chow, Bryna S M; Allen, Terri J

    2015-06-01

    Diabetic nephropathy (DN) is a term used to describe kidney damage cause by diabetes. With DN as one of the leading causes of end-stage renal disease worldwide, there is a strong need for appropriate animal models to study DN pathogenesis and develop therapeutic strategies. To date, most experiments are carried out in mouse models as opposed to other species for several reasons including lower cost, ease of handling, and easy manipulation of the mouse genome to generate transgenic and knockout animals. This unit provides detailed insights and technical knowledge in setting up one of the most widely used models of DN, the streptozotocin (STZ)-induced model. This model has been extensively exploited to study the mechanism of diabetic renal injury. The advantages and limitations of the STZ model and the availability of other genetic models of DN are also discussed.

  10. Association of Diabetic Nephropathy and Liver Disorders

    Science.gov (United States)

    Malawadi, BN

    2016-01-01

    Introduction Liver disorder is known to be a risk factor for Diabetes Mellitus (DM) and diabetic patients are at risk of developing liver disorders. Association of liver and renal disease is less explored in the field of research; hence, we conducted a retrospective study on this. Aim To compare the renal and liver profiles of type II DM patients compared to healthy controls and find the association between the two profiles in diabetics. Materials and Methods The renal and liver profiles of 68 type II DM patients and 58 controls were compared. Estimated Glomerular Filtration Rate (GFR) (eGFR) was calculated using Modification of Diet in Renal Disease (MDRD) formula and was taken as a tool to grade different stages of diabetic nephropathy. Comparison of liver profiles between different stages of diabetic nephropathy was done. Correlations and associations were studied between eGFR and liver enzymes and Bilirubin. Results A significant elevation in Total Bilirubin (TB) (p< 0.15), Direct Bilirubin (DB) (p< 0.0035), Aspartate Amino Transferase (AST) and Alanine Amino Transferase (ALT) (p<0.0001) levels in diabetics was noted. An elevated eGFR and a significant correlation between eGFR and liver enzymes were observed. A significant association between liver and renal disease has been obtained in diabetics (p=0.0136). Conclusion Significantly, high liver function tests and low eGFR were observed in type II diabetics. A significant positive correlation between liver enzymes (AST and ALT) and eGFR suggest a possible association between liver and kidney functions in DM. PMID:27891331

  11. Exogenous kallikrein protects against diabetic nephropathy.

    Science.gov (United States)

    Liu, Wenjuan; Yang, Yeping; Liu, Yemei; Lu, Xiaolan; Guo, Shizhe; Wu, Meng; Wang, Meng; Yan, Linling; Wang, Qinghua; Zhao, Xiaolong; Tong, Xian; Hu, Ji; Li, Yiming; Hu, Renming; Stanton, Robert C; Zhang, Zhaoyun

    2016-11-01

    The kallikrein-kinin system has been shown to be involved in the development of diabetic nephropathy, but specific mechanisms are not fully understood. Here, we determined the renal-protective role of exogenous pancreatic kallikrein in diabetic mice and studied potential mechanisms in db/db type 2 diabetic and streptozotocin-induced type 1 diabetic mice. After the onset of diabetes, mice were treated with either pancreatic kallikrein (db/db+kallikrein, streptozotocin+kallikrein) or saline (db/db+saline, streptozotocin+saline) for 16 weeks, while another group of streptozotocin-induced diabetic mice received the same treatment after onset of albuminuria (streptozotocin'+kallikrein, streptozotocin'+saline). Db/m littermates or wild type mice were used as non-diabetic controls. Pancreatic kallikrein had no effects on body weight, blood glucose and blood pressure, but significantly reduced albuminuria among all three groups. Pathological analysis showed that exogenous kallikrein decreased the thickness of the glomerular basement membrane, protected against the effacement of foot process, the loss of endothelial fenestrae, and prevented the loss of podocytes in diabetic mice. Renal fibrosis, inflammation and oxidative stress were reduced in kallikrein-treated mice compared to diabetic controls. The expression of kininogen1, tissue kallikrein, kinin B1 and B2 receptors were all increased in the kallikrein-treated compared to saline-treated mice. Thus, exogenous pancreatic kallikrein both prevented and ameliorated diabetic nephropathy, which may be mediated by activating the kallikrein-kinin system.

  12. Diabetic nephropathy in Africa: A systematic review

    Institute of Scientific and Technical Information of China (English)

    2015-01-01

    AIM To determine the prevalence and incidence ofdiabetic nephropathy in Africa.METHODS: We performed a systematic narrativereview of published literature following the MOOSEGuidelines for Meta-Analysis and Systematic Reviewsof Observational Studies. We searched PubMed-MEDLINE for all articles published in English and Frenchlanguages between January 1994 and July 2014 usinga predefined strategy based on the combination ofrelevant terms and the names of each of the 54 Africancountries and African sub-regions to capture the largestnumber of studies, and hand-searched the referencelists of retrieved articles. Included studies reported onthe prevalence, incidence or determinants of chronickidney disease (CKD) in people with diabetes withinAfrican countries.RESULTS: Overall, we included 32 studies from 16countries; two being population-based studies andthe remaining being clinic-based surveys. Most of thestudies (90.6%) were conducted in urban settings.Methods for assessing and classifying CKD variedwidely. Measurement of urine protein was the mostcommon method of assessing kidney damage (62.5%of studies). The overall prevalence of CKD varied from11% to 83.7%. Incident event rates were 94.9% forproteinuria at 10 years of follow-up, 34.7% for endstagerenal disease at 5 years of follow-up and 18.4%for mortality from nephropathy at 20 years of followup.Duration of diabetes, blood pressure, advancingage, obesity and glucose control were the commondeterminants of kidney disease.CONCLUSION: The burden of CKD is importantamong people with diabetes in Africa. High quality datafrom large population-based studies with validatedmeasures of kidney function are still needed to bettercapture the magnitude and characteristics of diabeticnephropathy in Africa.

  13. Role of metabolic control on diabetic nephropathy

    Directory of Open Access Journals (Sweden)

    Macedo Célia Sperandéo

    2002-01-01

    Full Text Available OBJECTIVE: The aim of this investigation was studying the influence of glucose metabolic control on diabetic nephropathy. The authors observed the effect of acarbose, insulin, and both drugs on the metabolic control and development of mesangial enlargement of kidney glomeruli in alloxan-diabetic rats. METHODS: Five groups of Wistar rats were used: normal rats (N, non-treated alloxan-diabetic rats (D, alloxan-diabetic rats treated with acarbose (AD, alloxan-diabetic rats treated with insulin (ID, and alloxan-diabetic rats treated with insulin plus acarbose (IAD. The following parameters were evaluated: body weight; water and food intake; diuresis; blood and urine glucose levels; and the kidney lesions: mesangial enlargement and tubule cell vacuolization. Renal lesions were analysed using a semi-quantitative score 1, 3, 6, 9, and 12 months after diabetes induction. RESULTS: Diabetic rats showed a marked increase of glycemia, urinary glucose levels, diuresis, water and food intake, and weight loss, while the treated diabetic rats showed significant decreased levels of these parameters. The most satisfactory metabolic control was that of diabetic rats treated with acarbose + insulin. There was a significant mesangial enlargement in diabetic rats compared to normal rats from the third up to the 12th month after diabetes induction, with a significant difference between the animals treated with acarbose + insulin and non-treated diabetic rats. A difference between the animals treated with acarbose or insulin alone and non-treated diabetics rats was not seen. CONCLUSIONS: The authors discuss the results stressing the role of diabetic metabolic control in the prevention of diabetic nephropathy.

  14. Contrast-induced nephropathy in interventional cardiology

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    Sudarsky D

    2011-07-01

    Full Text Available Doron Sudarsky, Eugenia NikolskyCardiology Department, Rambam Health Care Campus and Technion-Israel Institute of Technology, Haifa, IsraelAbstract: Development of contrast-induced nephropathy (CIN, ie, a rise in serum creatinine by either ≥0.5 mg/dL or by ≥25% from baseline within the first 2–3 days after contrast administration, is strongly associated with both increased inhospital and late morbidity and mortality after invasive cardiac procedures. The prevention of CIN is critical if long-term outcomes are to be optimized after percutaneous coronary intervention. The prevalence of CIN in patients receiving contrast varies markedly (from <1% to 50%, depending on the presence of well characterized risk factors, the most important of which are baseline chronic renal insufficiency and diabetes mellitus. Other risk factors include advanced age, anemia, left ventricular dysfunction, dehydration, hypotension, renal transplant, low serum albumin, concomitant use of nephrotoxins, and the volume of contrast agent. The pathophysiology of CIN is likely to be multifactorial, including direct cytotoxicity, apoptosis, disturbances in intrarenal hemodynamics, and immune mechanisms. Few strategies have been shown to be effective to prevent CIN beyond hydration, the goal of which is to establish brisk diuresis prior to contrast administration, and to avoid hypotension. New strategies of controlled hydration and diuresis are promising. Studies are mixed on whether prophylactic oral N-acetylcysteine reduces the incidence of CIN, although its use is generally recommended, given its low cost and favorable side effect profile. Agents which have been shown to be ineffective or harmful, or for which data supporting routine use do not exist, include fenoldopam, theophylline, dopamine, calcium channel blockers, prostaglandin E1, atrial natriuretic peptide, statins, and angiotensin-converting enzyme inhibitors.Keywords: contrast-induced nephropathy, contrast media

  15. Differential effects of low-dose fenofibrate treatment in diabetic rats with early onset nephropathy and established nephropathy.

    Science.gov (United States)

    Kadian, Supriya; Mahadevan, Nanjaian; Balakumar, Pitchai

    2013-01-05

    We have previously shown that low-dose fenofibrate treatment has an ability to prevent diabetes-induced nephropathy in rats. We investigated here the comparative pre- and post-treatment effects of low-dose fenofibrate (30 mg/kg/day p.o.) in diabetes-induced onset of nephropathy. Rats were made diabetics by single administration of streptozotocin (STZ, 55 mg/kg i.p.). The development of diabetic nephropathy was assessed biochemically and histologically. Moreover, lipid profile and renal oxidative stress were assessed. Diabetic rats after 8 weeks of STZ-administration developed apparent nephropathy by elevating serum creatinine, blood urea nitrogen and microproteinuria, and inducing glomerular-capsular wall distortion, mesangial expansion and tubular damage and renal oxidative stress. Fenofibrate (30 mg/kg/day p.o., 4 weeks) pretreatment (4 weeks after STZ-administration) markedly prevented diabetes-induced onset of diabetic nephropathy, while the fenofibrate (30 mg/kg/day p.o., 4 weeks) post-treatment (8 weeks after STZ-administration) was less-effective. However, both pre-and post fenofibrate treatments were effective in preventing diabetes-induced renal oxidative stress and lipid alteration in diabetic rats though the pretreatment was slightly more effective. Conversely, both pre-and post fenofibrate treatments did not alter elevated glucose levels in diabetic rats. It may be concluded that diabetes-induced oxidative stress and lipid alteration, in addition to a marked glucose elevation, play a detrimental role in the onset of nephropathy in diabetic rats. The pretreatment with low-dose fenofibrate might be a potential therapeutic approach in preventing the onset of nephropathy in diabetic subjects under the risk of renal disease induction. However, low-dose fenofibrate treatment might not be effective in treating the established nephropathy in diabetic subjects.

  16. Linear IgA and IgG bullous dermatosis.

    Science.gov (United States)

    Fernandes, Karina de Almeida Pinto; Galvis, Kely Hernández; Gomes, Anndressa Camillo da Matta Setubal; Nogueira, Osvania Maris; Felix, Paulo Antônio Oldani; Vargas, Thiago Jeunon de Sousa

    2016-01-01

    Childhood linear immunoglobulin A dermatosis is a rare autoimmune vesiculobullous disease. It results in linear deposition of autoantibodies (immunoglobulin A) against antigens in the basal membrane zone, leading to subepidermal cleavage. Additional depositions of immunoglobulin G and complement-3 might occur. It is still debated whether concomitant findings of immunoglobulins A and G should be considered a subtype of this dermatosis or a new entity. Further studies are needed to recognize this clinical variant.

  17. Linear IgA and IgG bullous dermatosis*

    Science.gov (United States)

    Fernandes, Karina de Almeida Pinto; Galvis, Kely Hernández; Gomes, Anndressa Camillo da Matta Setubal; Nogueira, Osvania Maris; Felix, Paulo Antônio Oldani; Vargas, Thiago Jeunon de Sousa

    2016-01-01

    Childhood linear immunoglobulin A dermatosis is a rare autoimmune vesiculobullous disease. It results in linear deposition of autoantibodies (immunoglobulin A) against antigens in the basal membrane zone, leading to subepidermal cleavage. Additional depositions of immunoglobulin G and complement-3 might occur. It is still debated whether concomitant findings of immunoglobulins A and G should be considered a subtype of this dermatosis or a new entity. Further studies are needed to recognize this clinical variant. PMID:28300887

  18. Childhood Brain Tumors

    Science.gov (United States)

    Brain tumors are abnormal growths inside the skull. They are among the most common types of childhood ... still be serious. Malignant tumors are cancerous. Childhood brain and spinal cord tumors can cause headaches and ...

  19. Mechanisms of diabetic nephropathy--old buddies and newcomers part 1.

    Science.gov (United States)

    Nawroth, P P; Isermann, B

    2010-10-01

    Diabetic nephropathy is the most frequent cause of terminal kidney failure in industrialized countries. In addition, the manifestation of diabetic nephropathy is associated with a poor prognosis for affected patients. Current therapies are based on established pathophysiological models. However, despite reflecting significant progress in our understanding of diabetic nephropathy, the translational efforts fell short their expectations. The current review summarizes recent studies which provided new insights into established mechanisms (part 1) and studies identifying new candidate mechanisms (part 2) underlying diabetic nephropathy.

  20. Angiotensin converting enzyme gene polymorphism in type II diabetics with nephropathy

    OpenAIRE

    Naresh, V. V. S.; Reddy, A. L. K.; Sivaramakrishna, G.; Sharma, P. V. G. K.; Vardhan, R. V.; Kumar, V. Siva

    2009-01-01

    Nephropathy is an important and a frequent complication of long-term type II diabetic nephropathy. Strong evidence exists that genetic predisposition plays a major role in the development of diabetic nephropathy. Recent studies have implicated association between angiotensin converting enzyme (ACE) insertion/deletion (I/D) gene polymorphism and nephropathy. The deletion gene polymorphism of ACE gene has been shown to be associated with increased activity of this enzyme. This study examines th...

  1. Improvement of the method of obtaining human IgA Fc-fragments

    Directory of Open Access Journals (Sweden)

    O. Y. Galkin

    2015-02-01

    Full Text Available To address a number of fundamental and applied problems in immunology, molecular and cellular biology and biotechnology it is necessary to obtain Fc-fragments of immunoglobulins. Fc-fragments may be used for studying of the effector functions of antibodies which are mediated by these areas. They are often used as an immunogen to produce anti-specie (based on so-called secondary antibody conjugate in the development of serological tests for diagnostics (predominantly such conjugate based on monoclonal antibodies. The work is aimed to develop improved methods of obtaining and allocation of Fc-fragments of human IgA. To achieve this objective, optimization of hydrolysis of IgA with subsequent purification of Fс-fragments have been carried out. Improved method of obtaining Fc-fragments of IgA provides: papain hydrolysis of immunoglobulin in the environment of nitrogen for 4 h, allowing to achieve maximum output of Fc-fragments without their further degradation: isolation and purification of Fc-fragments of human IgA by one-stage gel filtration on sephadex G-100; control of purity of the target product by electrophoresis in polyacrylamide gel with sodium dodecyl sulfate and Ouchterlony immunodiffusion. Enzymatic hydrolysis was carried out at the optimal temperature of papain (37 °C. As the oxygen in the air may have inhibitory effect on enzymatic hydrolysis reaction, the reaction mixture was incubated in the nitrogen atmosphere to prevent inactivation of papain. To reduce the incident degradation of immunoglobulin molecules, papain hydrolysis was carried out without using an enzyme activator (cysteine. Usage of the proposed scheme allows obtaining Fc-fragments of human IgA of high purity. Outcome of Fc-fragments after all stages of purification was about 18% of the initial amount of IgA in the preparation. Molecular weight from Fc-fragments of human IgA was equal to approximately 70 kDa.

  2. Effects of astaxanthin-enriched yeast on mucosal IgA induction in the jejunum and ileum of weanling mice.

    Science.gov (United States)

    Nagayama, Tatsuhiko; Sugimoto, Miki; Ikeda, Shuntaro; Kume, Shinichi

    2014-04-01

    The present study was conducted to clarify the effects of astaxanthin-enriched yeast on the concentration of immunoglobulin A (IgA), the numbers of IgA antibody-secreting cells (ASC) and the messenger RNA (mRNA) expression of IgA C-region in the jejunum and ileum of weanling mice. Weanling mice were fed rodent feed or astaxanthin-enriched yeast-supplemented rodent feed for 7, 14 or 21 days. Supplemental astaxanthin-enriched yeast increased the numbers of IgA ASC in the jejunum and ileum after 7, 14 and 21 days of treatment. Supplemental astaxanthin-enriched yeast increased IgA concentrations in the jejunum after 21 days of treatment, but IgA concentrations in the ileum were not affected by the treatment. The mRNA expressions of IgA C-region in the jejunum after 14 and 21 days of treatment and the ileum after 14 days of treatment were enhanced by supplementation of astaxanthin-enriched yeast. These results indicate that supplementation of astaxanthin-enriched yeast is effective to enhance the numbers of IgA ASC in the jejunum and ileum and IgA concentrations in the ileum of weanling mice.

  3. Evaluation of the diagnostic accuracy of a new dengue IgA capture assay (Platelia Dengue IgA Capture, Bio-Rad for dengue infection detection.

    Directory of Open Access Journals (Sweden)

    Sophie De Decker

    2015-03-01

    Full Text Available Considering the short lifetime of IgA antibodies in serum and the key advantages of antibody detection ELISAs in terms of sensitivity and specificity, Bio-Rad has just developed a new ELISA test based on the detection of specific anti-dengue IgA. This study has been carried out to assess the performance of this Platelia Dengue IgA Capture assay for dengue infection detection. A total of 184 well-characterized samples provided by the French Guiana NRC sera collection (Laboratory of Virology, Institut Pasteur in French Guiana were selected among samples collected between 2002 and 2013 from patients exhibiting a dengue-like syndrome. A first group included 134 sera from confirmed dengue-infected patients, and a second included 50 sera from non-dengue infected patients, all collected between day 3 and day 15 after the onset of fever. Dengue infection diagnoses were all confirmed using reference assays by direct virological identification using RT-PCR or virus culture on acute sera samples or on paired acute-phase sera samples of selected convalescent sera. This study revealed: i a good overall sensitivity and specificity of the IgA index test, i.e., 93% and 88% respectively, indicating its good correlation to acute dengue diagnosis; and ii a good concordance with the Panbio IgM capture ELISA. Because of the shorter persistence of dengue virus-specific IgA than IgM, these results underlined the relevance of this new test, which could significantly improve dengue diagnosis accuracy, especially in countries where dengue virus is (hyper- endemic. It would allow for additional refinement of dengue diagnostic strategy.

  4. Evaluation of the diagnostic accuracy of a new dengue IgA capture assay (Platelia Dengue IgA Capture, Bio-Rad) for dengue infection detection.

    Science.gov (United States)

    De Decker, Sophie; Vray, Muriel; Sistek, Viridiana; Labeau, Bhety; Enfissi, Antoine; Rousset, Dominique; Matheus, Séverine

    2015-03-01

    Considering the short lifetime of IgA antibodies in serum and the key advantages of antibody detection ELISAs in terms of sensitivity and specificity, Bio-Rad has just developed a new ELISA test based on the detection of specific anti-dengue IgA. This study has been carried out to assess the performance of this Platelia Dengue IgA Capture assay for dengue infection detection. A total of 184 well-characterized samples provided by the French Guiana NRC sera collection (Laboratory of Virology, Institut Pasteur in French Guiana) were selected among samples collected between 2002 and 2013 from patients exhibiting a dengue-like syndrome. A first group included 134 sera from confirmed dengue-infected patients, and a second included 50 sera from non-dengue infected patients, all collected between day 3 and day 15 after the onset of fever. Dengue infection diagnoses were all confirmed using reference assays by direct virological identification using RT-PCR or virus culture on acute sera samples or on paired acute-phase sera samples of selected convalescent sera. This study revealed: i) a good overall sensitivity and specificity of the IgA index test, i.e., 93% and 88% respectively, indicating its good correlation to acute dengue diagnosis; and ii) a good concordance with the Panbio IgM capture ELISA. Because of the shorter persistence of dengue virus-specific IgA than IgM, these results underlined the relevance of this new test, which could significantly improve dengue diagnosis accuracy, especially in countries where dengue virus is (hyper-) endemic. It would allow for additional refinement of dengue diagnostic strategy.

  5. Endothelial nitric oxide synthase gene haplotypes and diabetic nephropathy among Asian Indians

    DEFF Research Database (Denmark)

    Ahluwalia, Tarun Veer Singh; Ahuja, Monica; Rai, Taranjit Singh

    2008-01-01

    .23-16.31). Haplotype with all the wild alleles (T-b-G) was found to be associated with a decreased risk of nephropathy (OR: 0.68, P = 0.005) and haplotype with all mutant alleles (C-a-T) was associated with higher risk of diabetic nephropathy as compared to diabetes without nephropathy group (OR: 2.6, P = 0...

  6. Testament's ability in Balkan endemic nephropathy

    Directory of Open Access Journals (Sweden)

    Novaković Milan

    2009-01-01

    Full Text Available Introduction Testament is a solemn, authentic instrument in writing, by which a person declares his or her will as to disposal of his or her estate, and it has a psychopathological, lawful and ethical importance to a person, family and society. The aim of the study was to assess if the ability to make a testament was more damaged in patients with Balkan Endemic Nephropathy (BEN than in patients with other diseases that resulted in Chronic Renal Failure in Bosnia and Herzegovina in the period from the 1st January 2001 to 31st December 2006. Material and methods The 753 respondents were divided into two groups in the study: BEN group (n=150 and control group made of patients with other diseases resulting in CRF (n=150. In a multicentric longitudinal study we used: adapted questionnaire from the Renal Register of Bosnia and Herzegovina, Hamilton Depression Rating Scale, and Mini-Mental State Examination. Descriptive analysis, discriminative function and regression model have been done statistically. Results In BEN group, heirs are mostly mentioned - 84.0% (t=14.391; P=0.001, and in control group: heirs - 66.6%, relatives - 43.3% (t=7.751; P=0.003, carers - 44.0% (t= 6.678 P=0.032, and institutions 10.0% (t=5.147, P=0.061. The discriminative function shows differences between BEN and control group: canonical correlation (rc =0.827, Wilkinson lambda (lnj =0.871, Chi-square test =141.575 and significance (P=0.001. The regression course of the analysis can be used for prediction of the ability to make testament for the patients on dialysis: [y=-0.95x + 15.715, and OR = 0.785, (95% for CI = -0.997 - -0.375; Can Fanc r2=0.861; Significance is P=0.002]. Conclusion The ability to make a testament is more damaged in patients from the nephropathy group than in the patients from the control group who are on dialysis in Bosnia and Herzegovina. This has been confirmed by socio-demographic and psychological parameters, and it is very important for preservation

  7. Autophagy: A Novel Therapeutic Target for Diabetic Nephropathy.

    Science.gov (United States)

    Kume, Shinji; Koya, Daisuke

    2015-12-01

    Diabetic nephropathy is a leading cause of end stage renal disease and its occurance is increasing worldwide. The most effective treatment strategy for the condition is intensive treatment to strictly control glycemia and blood pressure using renin-angiotensin system inhibitors. However, a fraction of patients still go on to reach end stage renal disease even under such intensive care. New therapeutic targets for diabetic nephropathy are, therefore, urgently needed. Autophagy is a major catabolic pathway by which mammalian cells degrade macromolecules and organelles to maintain intracellular homeostasis. The accumulation of damaged proteins and organelles is associated with the pathogenesis of diabetic nephropathy. Autophagy in the kidney is activated under some stress conditions, such as oxidative stress and hypoxia in proximal tubular cells, and occurs even under normal conditions in podocytes. These and other accumulating findings have led to a hypothesis that autophagy is involved in the pathogenesis of diabetic nephropathy. Here, we review recent findings underpinning this hypothesis and discuss the advantages of targeting autophagy for the treatment of diabetic nephropathy.

  8. Paracetamol and analgesic nephropathy: Are you kidneying me?

    Directory of Open Access Journals (Sweden)

    Waddington F

    2014-12-01

    Full Text Available Freya Waddington, Mark Naunton, Jackson Thomas Faculty of Health, University of Canberra, Canberra, ACT, Australia Introduction: Analgesic nephropathy is a disease resulting from the frequent use of combinations of analgesic medications over many years, leading to significant impairment of renal function. The observation of a large number of cases of renal failure in patients abusing analgesic mixtures containing phenacetin led to the initial recognition of the nephrotoxicity from the use of analgesics. Phenacetin was subsequently exclusively blamed for this disease. However, the role of a single analgesic as a sole cause of analgesic nephropathy was challenged, and a number of researchers have since attempted to determine the extent of involvement of other analgesics including nonsteroidal anti-inflammatory drugs (NSAIDs, aspirin, and paracetamol. Case presentation: We present the case of an 83-year-old woman with a history of NSAID-induced nephropathy with poor pain control and reluctance to use paracetamol. We attempt to briefly review the evidence of paracetamol being implicated in the development of analgesic-induced nephropathy. Conclusion: There is a lack of concrete data regarding causative analgesics, including paracetamol. Patients should therefore not be withheld paracetamol, an effective and commonly recommended agent, for fear of worsening renal function. Keywords: kidney, paracetamol, nephropathy, phenacetin

  9. Vitamin D receptor and its protective role in diabetic nephropathy

    Institute of Scientific and Technical Information of China (English)

    Guan Xiaoling; Yang Huajie; Zhang Wei; Wang Huanjun; Liao Lin

    2014-01-01

    Objective To review the advances of studies on vitamin D receptor and its role in the pathogenesis of diabetic nephropathy.Data sources A comprehensive search of the PubMed literatures without restriction on the publication date was carried out using keywords such as vitamin D receptor and diabetic nephropathy.Study selection Articles related to vitamin D receptor and diabetic nephropathy were selected and carefully analyzed.Results The ligands as well as construction and tissue distribution of vitamin D receptor were summarized.Pathogenesis of diabetic nephropathy was analyzed.The mechanisms underlying the renoprotective role of vitamin D receptor including inhibition of renin-angiotensin system,anti-inflammation,anti-fibrosis and the reduction of proteinuria were reviewed.Mounting evidences from animal and clinical studies have suggested that vitamin D therapy has beneficial effects on the renal systems and the underlying renoprotective mechanisms of the vitamin D receptor-mediated signaling pathways is a hot research topic.Conclusion Our study suggests that vitamin D receptor has a great potential for preventing the progression of diabetic nephropathy via multiple mechanisms.

  10. Vascular endothelial dysfunction: a tug of war in diabetic nephropathy?

    Science.gov (United States)

    Balakumar, Pitchai; Chakkarwar, Vishal Arvind; Krishan, Pawan; Singh, Manjeet

    2009-03-01

    Vascular endothelium regulates vascular tone and maintains free flow of blood in vessels. Vascular endothelial dysfunction (VED) results in reduced activation of endothelial nitric oxide synthase (eNOS), reduced generation and bioavailability of nitric oxide (NO) and increased production of reactive oxygen species (ROS). The eNOS uncoupling in VED leads to eNOS mediated production of ROS that further damage the endothelial cells by upregulating the proinflammatory mediators and adhesion molecules. VED has been associated in the pathogenesis of hypertension, atherosclerosis, coronary artery diseases, diabetes mellitus and nephropathy. Diabetes is a chronic metabolic disorder characterized by hyperglycemia followed by micro and macrovascular complications. A correlation between diabetes and VED has been demonstrated in various studies. The downregulation of eNOS in diabetes has been noted to accelerate diabetic nephropathy. Moreover, various endogenous vasoconstrictors are also upregulated in diabetic nephropathy. VED has been shown to be involved in diabetic nephropathy by inducing nodular glomerulosclerosis followed by glomerular basement membrane thickness and mesangial expansion, which ultimately decline glomerular filtration rate (GFR). Thus it is suggested that diabetes-induced VED could be one of the culprits involved in the pathogenesis of diabetic nephropathy.

  11. Prevalence of IgA deficiency in children with juvenile rheumatoid arthritis.

    Science.gov (United States)

    Moradinejad, Mohammad Hasan; Rafati, Ali Hoseinpoor; Ardalan, Maryam; Rabiei, Mahnaz; Farghadan, Maryam; Ashtiani, Mohammad Taghi Haghi; Pourpak, Zahra; Moin, Mostafa

    2011-03-01

    The purpose of this study was to investigate any association between IgA deficiency (IgAD) and juvenile rheumatoid arthritis (JRA) among Iranian children.This case-control study was carried out on 83 children who were diagnosed as JRA according to American College of Rheumatology (ACR) criteria; Patients were admitted at the rheumatology clinic of Children's Medical Center, (Tehran). Serum immunoglobulins concentrations were determined by nephelometry method. Control group was 112 healthy children who were matched for age and gender. Informed consent obtained from all parents.Selective IgA deficiency (sIgAD) was found only in a boy (1.2%) among JRA children; however, partial IgA deficiency was found in 6(7.1%) of patients with JRA and in 12(10.7%) of control subjects, this difference was not statistically significant (p=0.46). Immunoglobulins levels in patients with JRA (IgM: 126.7±57.2, IgG: 1182.3±351 and IgA:169.3±98) were significantly higher than their controls (IgM: 104±52, IgG:802±220 and IgA: 94.6±47) (pJRA patients and their control counterpart; this might be partly due to the high rate of consanguineous marriages in Iran that resulted in increased prevalence of clinically undiagnosed partial IgAD in general population. Hence, future epidemiological studies are warranted to make it clear.

  12. Phagocytosis of IgA Immune Complexes by Human U937 Cells

    Institute of Scientific and Technical Information of China (English)

    郭彩云; 崔薇; 张伟

    2003-01-01

    In order to study FcαR Ⅰ mediated phagocytosis ot lgA immune complexes by U937 cells, antigen 8.9NIP/BSA was labeled with FITC and reacted with anti-NIP IgA or anti-NIP IgG antibody to form immune complexes (ICs). They were then incubated with phorbol 12-myristate B-acetate (PMA) stimulated U937 cells. The phagocytosed ICs were quantified by flow cytometry. The results was that the expression of FcαR Ⅰ on U937 cells was higher than that of FcγR Ⅰ , FcyR Ⅱ and FcγR Ⅲ. After stimulation by PMA, expression of FcαR Ⅰ on U937 cells was markedly upregulated and the phagocytosis of IgA ICs was enhanced. FcαR Ⅰ mediated specific IgA phagocytosis was stronger than FcγR Ⅰ and FcγR Ⅱ mediated IgG phagocytosis. Complement receptors, CR1 and CR3, enhanced U937 cell phagocytosis of IgA ICs. It concludes that FcγR Ⅰ mediated strong phagocytosis of IgA ICs.

  13. Prominent role for plasmacytoid dendritic cells in mucosal T cell-independent IgA induction.

    Science.gov (United States)

    Tezuka, Hiroyuki; Abe, Yukiko; Asano, Jumpei; Sato, Taku; Liu, Jiajia; Iwata, Makoto; Ohteki, Toshiaki

    2011-02-25

    Although both conventional dendritic cells (cDCs) and plasmacytoid dendritic cells (pDCs) are present in the gut-associated lymphoid tissues (GALT), the roles of pDCs in the gut remain largely unknown. Here we show a critical role for pDCs in T cell-independent (TI) IgA production by B cells in the GALT. When pDCs of the mesenteric lymph nodes (MLNs) and Peyer's patches (PPs) (which are representative GALT) were cultured with naive B cells to induce TI IgA class switch recombination (CSR), IgA production was substantially higher than in cocultures of these cells with cDCs. IgA production was dependent on APRIL and BAFF production by pDCs. Importantly, pDC expression of APRIL and BAFF was dependent on stromal cell-derived type I IFN signaling under steady-state conditions. Our findings provide insight into the molecular basis of pDC conditioning to induce mucosal TI IgA production, which may lead to improvements in vaccination strategies and treatment for mucosal-related disorders.

  14. High fecal IgA is associated with reduced Clostridium difficile colonization in infants.

    Science.gov (United States)

    Bridgman, Sarah L; Konya, Tedd; Azad, Meghan B; Guttman, David S; Sears, Malcolm R; Becker, Allan B; Turvey, Stuart E; Mandhane, Piush J; Subbarao, Padmaja; Scott, James A; Field, Catherine J; Kozyrskyj, Anita L

    2016-09-01

    Colonization of infants with Clostridium difficile is on the rise. Although better tolerated by infants than adults, it is a risk factor for future allergic disease. The present study describes associations between infant fecal immunoglobulin A (IgA) and colonization with C. difficile in 47 infants enrolled in the Canadian Healthy Infant Longitudinal Development (CHILD) study. C. difficile colonization was observed in over half (53%) of the infants. Median IgA was lower in infants colonized with C. difficile (10.9 μg versus 25.5 μg per g protein; p = 0.18). A smaller proportion of infants with IgA in the highest tertile were colonized with C. difficile compared to the other tertiles (31.3% versus 64.5%, p = 0.03). In unadjusted analysis, odds of colonization with C. difficile was reduced by 75% (OR 0.25 95% CI 0.07, 0.91 p = 0.04) among infants with IgA in the highest tertile compared to those in the other tertiles. Following adjustment for parity, birth mode and breastfeeding, this association was even stronger (aOR 0.17, 95% CI 0.03, 0.94, p = 0.04). Our study provides evidence that high fecal IgA, independent of breastfeeding, is associated with reduced likelihood of C. difficile colonization in infancy.

  15. New therapeutic agents in diabetic nephropathy

    Science.gov (United States)

    Kim, Yaeni; Park, Cheol Whee

    2017-01-01

    Studies investigating diabetic nephropathy (DN) have mostly focused on interpreting the pathologic molecular mechanisms of DN, which may provide valuable tools for early diagnosis and prevention of disease onset and progression. Currently, there are few therapeutic drugs for DN, which mainly consist of antihypertensive and antiproteinuric measures that arise from strict renin-angiotensin-aldosterone system inactivation. However, these traditional therapies are suboptimal and there is a clear, unmet need for treatments that offer effective schemes beyond glucose control. The complexity and heterogeneity of the DN entity, along with ambiguous renal endpoints that may deter accurate appraisal of new drug potency, contribute to a worsening of the situation. To address these issues, current research into original therapies to treat DN is focusing on the intrinsic renal pathways that intervene with intracellular signaling of anti-inflammatory, antifibrotic, and metabolic pathways. Mounting evidence in support of the favorable metabolic effects of these novel agents with respect to the renal aspects of DN supports the likelihood of systemic beneficial effects as well. Thus, when translated into clinical use, these novel agents would also address the comorbid factors associated with diabetes, such as obesity and risk of cardiovascular disease. This review will provide a discussion of the promising and effective therapeutic agents for the management of DN. PMID:28049280

  16. Histone Lysine Methylation in Diabetic Nephropathy

    Directory of Open Access Journals (Sweden)

    Guang-dong Sun

    2014-01-01

    Full Text Available Diabetic nephropathy (DN belongs to debilitating microvascular complications of diabetes and is the leading cause of end-stage renal diseases worldwide. Furthermore, outcomes from the DCCT/EDIC study showed that DN often persists and progresses despite intensive glucose control in many diabetes patients, possibly as a result of prior episode of hyperglycemia, which is called “metabolic memory.” The underlying mechanisms responsible for the development and progression of DN remain poorly understood. Activation of multiple signaling pathways and key transcription factors can lead to aberrant expression of DN-related pathologic genes in target renal cells. Increasing evidence suggests that epigenetic mechanisms in chromatin such as DNA methylation, histone acetylation, and methylation can influence the pathophysiology of DN and metabolic memory. Exciting researches from cell culture and experimental animals have shown that key histone methylation patterns and the related histone methyltransferases and histone demethylases can play important roles in the regulation of inflammatory and profibrotic genes in renal cells under diabetic conditions. Because histone methylation is dynamic and potentially reversible, it can provide a window of opportunity for the development of much-needed novel therapeutic potential for DN in the future. In this minireview, we discuss recent advances in the field of histone methylation and its roles in the pathogenesis and progression of DN.

  17. Effect of loganin on experimental diabetic nephropathy.

    Science.gov (United States)

    Jiang, Wang-Lin; Zhang, Shu-Ping; Hou, Jian; Zhu, Hai-Bo

    2012-02-15

    Connective tissue growth factor (CTGF) plays a pathogenic role in diabetic nephropathy (DN). Loganin, an iridoid glucoside compound was isolated from Cornus officinalis Sieb. et Zucc. This study was conducted to investigate the efficacy of loganin on DN and to elucidate the potential mechanism. High glucose (HG) stimulated cultured human renal proximal tubular epithelial cells (HK-2) analyzed CTGF expression by Western blotting and investigated whether extracellular signal-regulated kinase (ERK) signaling pathway was involved. Streptozotocin (STZ)-induced experimental DN, randomized to receive intragastric (i.g.) of loganin. Renal tissue, blood and urine samples were collected to determine and analyze. In vitro study, loganin reduced CTGF excretion in HG-induced HK-2 cells through the ERK signaling pathway. In vivo study, I.g. of loganin 5 mg/kg or 10 mg/kg significantly ameliorated renal function and increased body weight. Meanwhile, loganin reduced renal CTGF expression by immunohistochemical staining, reduced serum levels of CTGF. Besides, there were no significant differences in blood sugar levels between the loganin groups compared to the STZ-treated group. Furthermore, loganin ameliorated renal pathology. These results suggested that loganin exerts an early renal protective role to DN. Inhibition of CTGF may be a potential target in DN therapy, which highlights the possibility of using loganin to treat DN.

  18. Diabetic nephropathy: changes after diabetes surgery?

    Directory of Open Access Journals (Sweden)

    S. Ros Ruiz

    2013-01-01

    Full Text Available Introduction: Obesity, as a central piece inside metabolic syndrome, is associated with early chronic kidney disease (CKD. In addition, several observational, cross sectional, and longitudinal studies have demonstrated that obesity is as an independent risk factor for the onset, aggravated course, and poor outcomes of CKD including diabetic nephropathy. This implies that when obesity is reversed, many CKD risk factors and CKD itself could be favorably influenced. So all measures aimed at weight loss are recommended to minimize risks from obesity-related conditions and generate improvements in the metabolic profile. Recent evidence shows that bariatric surgery (BS can revert or improve proteinuria and CKD in morbidly obese patients. Objectives and methods: The present review is aimed to provide the evidence regarding the beneficial effects of weight loss after BS in different stages of CKD including kidney transplant recipients, with an special focus on the beneficial effect in reducing or improving proteinuria and renal failure. Furthermore, this updated systematic review of the literature analyzes potential adverse effects that BS could induce not only on renal function but also on morbidity and mortality risk in perioperative and postoperative period. Conclusions: Results from the different case reports, meta analysis as well as systematic review of clinical trials show that obesity treatment by way of lifestyle changes, pharmacotherapies and BS can reduce proteinuria and help to prevent loss of renal function. Also BS may reduce complications, and allow obese patients with end-stage renal disease to undergo kidney transplantation with good results.

  19. Role of Neuropilin-1 in Diabetic Nephropathy

    Directory of Open Access Journals (Sweden)

    Tzvetanka Bondeva

    2015-06-01

    Full Text Available Diabetic nephropathy (DN often develops in patients suffering from type 1 or type 2 diabetes mellitus. DN is characterized by renal injury resulting in proteinuria. Neuropilin-1 (NRP-1 is a single-pass transmembrane receptor protein devoid of enzymatic activity. Its large extracellular tail is structured in several domains, thereby allowing the molecule to interact with multiple ligands linking NRP-1 to different pathways through its signaling co-receptors. NRP-1’s role in nervous system development, immunity, and more recently in cancer, has been extensively investigated. Although its relation to regulation of apoptosis and cytoskeleton organization of glomerular vascular endothelial cells was reported, its function in diabetes mellitus and the development of DN is less clear. Several lines of evidence demonstrate a reduced NRP-1 expression in glycated-BSA cultured differentiated podocytes as well as in glomeruli from db/db mice (a model of type 2 Diabetes and in diabetic patients diagnosed with DN. In vitro studies of podocytes implicated NRP-1 in the regulation of podocytes’ adhesion to extracellular matrix proteins, cytoskeleton reorganization, and apoptosis via not completely understood mechanisms. However, the exact role of NRP-1 during the onset of DN is not yet understood. This review intends to shed more light on NRP-1 and to present a link between NRP-1 and its signaling complexes in the development of DN.

  20. [Case of MMF monotherapy for membranous nephropathy].

    Science.gov (United States)

    Kobayashi, Mioko; Kojima, Chiari; Sugiura, Hidekazu; Aoki, Asuka; Itabashi, Mitsuyo; Tsukada, Misao; Takei, Takashi; Uchida, Keiko; Nitta, Kosaku

    2010-01-01

    We report the case of a 58-year-old male patient who visited our hospital for the management of edema and proteinuria. He was diagnosed as having nephrotic syndrome, with serum total protein and albumin levels of 4.6 g/dL and 2.1 g/dL, respectively, and a urinary protein excretion level of 6.0 g/day. A percutaneous renal biopsy showed features of membranous glomerulonephritis, with capillary-wall granular deposits of IgG and C3 on immunofluorescence and subepithelial immune complex deposits on electron microscopy. No other secondary cause of membranous glomerulopathy was found even after extensive investigations. The patient was started on mycophenolate mofetil (MMF) monotherapy (1,500 mg/day), and 18 months after the start of this therapy, the proteinuria decreased to 0.5 g/day, with return to a normal serum albumin level. No digestive symptoms, kidney function worsening or increase in blood pressure were noted during treatment. These findings suggest that MMF monotherapy is effective and safe for the treatment of membranous nephropathy.

  1. Membranous nephropathy: from models to man

    Science.gov (United States)

    Beck, Laurence H.; Salant, David J.

    2014-01-01

    As recently as 2002, most cases of primary membranous nephropathy (MN), a relatively common cause of nephrotic syndrome in adults, were considered idiopathic. We now recognize that MN is an organ-specific autoimmune disease in which circulating autoantibodies bind to an intrinsic antigen on glomerular podocytes and form deposits of immune complexes in situ in the glomerular capillary walls. Here we define the clinical and pathological features of MN and describe the experimental models that enabled the discovery of the major target antigen, the M-type phospholipase A2 receptor 1 (PLA2R). We review the pathophysiology of experimental MN and compare and contrast it with the human disease. We discuss the diagnostic value of serological testing for anti-PLA2R and tissue staining for the redistributed antigen, and their utility for differentiating between primary and secondary MN, and between recurrent MN after kidney transplant and de novo MN. We end with consideration of how knowledge of the antigen might direct future therapeutic strategies. PMID:24892704

  2. Diabetic nephropathy and arterial hypertension. The effect of antihypertensive treatment

    DEFF Research Database (Denmark)

    Parving, H H; Andersen, A R; Smidt, U M

    1983-01-01

    in arterial blood pressure to a hypertensive level is an early feature; 43% of the patients had diastolic blood pressure greater than 100 mm Hg. Early and aggressive antihypertensive treatment reduces both albuminuria and the rate of decline in GFR in young patients with diabetic nephropathy.......Our longitudinal study of urinary albumin excretion rate in long-term insulin-dependent diabetics without proteinuria (negative albustix) suggests that early detection of patients at high and low risk of developing persistent proteinuria, i.e., diabetic nephropathy, is possible by using a sensitive...... method for albumin determination. Our prospective studies in young insulin-dependent diabetics with diabetic nephropathy show that the rate of decline in glomerular filtration rate (GFR) varies considerably, with a mean of 0.75 ml/min/mo and a range from 0.1 to 1.50 ml/min/mo, and that an increase...

  3. Treatment and impact of dyslipidemia in diabetic nephropathy.

    Science.gov (United States)

    Toyama, Tadashi; Shimizu, Miho; Furuichi, Kengo; Kaneko, Shuichi; Wada, Takashi

    2014-04-01

    Recent epidemiological research revealed that dyslipidemia is a risk factor for development and progression of diabetic nephropathy. Results from interventional studies revealed the possibility that anti-hyperlipidemic agents have a better effect on diabetic nephropathy through improvement of albuminuria and loss of renal function. In addition, dyslipidemia may be a consequence of albuminuria and renal dysfunction, thereby perpetuating kidney damage. Today, the proportion of diabetic patients receiving statins is increasing due to their beneficial effect on cardiovascular mortality. However, treatment for patients should be determined based on consideration of the risk and benefit of the treatment. More insight into the pathogenesis of diabetic nephropathy and the effects of life-style changes is required.

  4. Herbs and hazards: risk of aristolochic acid nephropathy in Iran.

    Science.gov (United States)

    Ardalan, Mohammad Reza; Khodaie, Laleh; Nasri, Hamid; Jouyban, Abolghasem

    2015-01-01

    Herbs are usually considered as inherently harmless products. Nonetheless, various renal injuries have been reported in association with several herbs. The best-known herb-induced chronic kidney disease is aristolochic acid nephropathy. Aristolochic acid is found in Chinese slim herbs. Balkan endemic nephropathy is nowadays considered as an aristolochic acid nephropathy. Plants of Aristolochiaceae (also known as birthwort, dutchman's pipe, and somersworth) is named zaravand or chopoghak in Persian and it grows in different mountainous and rural areas of Iran. The fruit and the steam of the Aristolochiacae are named zaravand gerd (nokhod alvand) and zaravand dearaz, respectively, and have different usage in Iranian teadirional such as treatment of headache, back pain, and anxiety. Some patients with end-stage renal disease and bilateral small kidneys have a history of exposure to some herbal remedies. We need to consider the possibility of environmental toxins and even Aristolochia nephrotoxicity as a potential danger in Iran.

  5. Immunoglobulins in nasal secretions of healthy humans: structural integrity of secretory immunoglobulin A1 (IgA1) and occurrence of neutralizing antibodies to IgA1 proteases of nasal bacteria

    DEFF Research Database (Denmark)

    Kirkeby, L; Rasmussen, TT; Reinholdt, Jesper;

    2000-01-01

    healthy humans, with a focus on IgA, and at the same time have characterized and quantified IgA1 protease-producing bacteria in the nasal flora of the subjects. Samples in the form of nasal wash were collected by using a washing liquid that contained lithium as an internal reference. Dilution factors and...

  6. Association of IgA multiple myeloma with pre-existing disease

    Energy Technology Data Exchange (ETDEWEB)

    Schafer, A.I.; Miller, J.B.

    1979-01-01

    A retrospective analysis of 153 patients with multiple myeloma was performed for evaluation of the possible significance of pre-existing disease. 37% of the group had no significant antecedent disorder. The most common prior illnesses were peptic ulcer disease and gallbladder disease. Of 12 patients in the group who had prior biliary tract disease and for whom immunoelectrophoretic studies were available, eight (66.7%) had IgA paraproteins. This figure is statistically higher than the 14.1% of prevalence of IgA paraproteins in those myeloma patients without biliary disease. We conclude that prior inflammatory gastrointestinal, pulmonary, and, particularly, biliary disease may be implicated in the pathogenesis of the IgA subset of multiple myeloma.

  7. Proteobacteria-specific IgA regulates maturation of the intestinal microbiota.

    Science.gov (United States)

    Mirpuri, Julie; Raetz, Megan; Sturge, Carolyn R; Wilhelm, Cara L; Benson, Alicia; Savani, Rashmin C; Hooper, Lora V; Yarovinsky, Felix

    2014-01-01

    The intestinal microbiota changes dynamically from birth to adulthood. In this study we identified γ-Proteobacteria as a dominant phylum present in newborn mice that is suppressed in normal adult microbiota. The transition from a neonatal to a mature microbiota was in part regulated by induction of a γ-Proteobacteria-specific IgA response. Neocolonization experiments in germ-free mice further revealed a dominant Proteobacteria-specific IgA response triggered by the immature microbiota. Finally, a role for B cells in the regulation of microbiota maturation was confirmed in IgA-deficient mice. Mice lacking IgA had persistent intestinal colonization with γ-Proteobacteria that resulted in sustained intestinal inflammation and increased susceptibility to neonatal and adult models of intestinal injury. Collectively, these results identify an IgA-dependent mechanism responsible for the maturation of the intestinal microbiota.

  8. Adaptive changes in renal mitochondrial redox status in diabetic nephropathy

    Energy Technology Data Exchange (ETDEWEB)

    Putt, David A.; Zhong, Qing; Lash, Lawrence H., E-mail: l.h.lash@wayne.edu

    2012-01-15

    Nephropathy is a serious and common complication of diabetes. In the streptozotocin (STZ)-treated rat model of diabetes, nephropathy does not typically develop until 30 to 45 days post-injection, although hyperglycemia occurs within 24 h. We tested the hypothesis that chronic hyperglycemia results in a modest degree of oxidative stress that is accompanied by compensatory changes in certain antioxidants and mitochondrial redox status. We propose that as kidneys progress to a state of diabetic nephropathy, further adaptations occur in mitochondrial redox status. Basic parameters of renal function in vivo and several parameters of mitochondrial function and glutathione (GSH) and redox status in isolated renal cortical mitochondria from STZ-treated and age-matched control rats were examined at 30 days and 90 days post-injection. While there was no effect of diabetes on blood urea nitrogen, measurement of other, more sensitive parameters, such as urinary albumin and protein, and histopathology showed significant and progressive worsening in diabetic rats. Thus, renal function is compromised even prior to the onset of frank nephropathy. Changes in mitochondrial respiration and enzyme activities indicated existence of a hypermetabolic state. Higher mitochondrial GSH content and rates of GSH transport into mitochondria in kidneys from diabetic rats were only partially due to changes in expression of mitochondrial GSH carriers and were mostly due to higher substrate supply. Although there are few clear indicators of oxidative stress, there are several redox changes that occur early and change further as nephropathy progresses, highlighting the complexity of the disease. Highlights: ►Adaptive changes in renal mitochondrial and redox status in diabetic rats. ►Modest renal dysfunction even prior to onset of nephropathy. ►Elevated concentrations of mitochondrial GSH in diabetic kidneys. ►Change in GSH due partly to increased protein expression of transporter.

  9. Towards microRNA-based therapeutics for diabetic nephropathy.

    Science.gov (United States)

    Alvarez, M L; DiStefano, J K

    2013-03-01

    There is no cure for diabetic nephropathy and the molecular mechanisms underlying disease aetiology remain poorly understood. While current paradigms for clinical management of diabetic nephropathy are useful in delaying disease onset and preventing its progression, they do not do so for a significant proportion of diabetic individuals, who eventually end up developing renal failure. Thus, novel therapeutic targets are needed for the treatment and prevention of the disease. MicroRNAs (miRNAs), a class of non-coding RNAs that negatively regulate gene expression, have recently been identified as attractive targets for therapeutic intervention. It is widely recognised that dysregulation of miRNA expression or action contributes to the development of a number of different human diseases, and evidence of a role for miRNAs in the aetiology of diabetic nephropathy is emerging. The discovery that modulation of miRNA expression in vivo is feasible, combined with recent results from successful clinical trials using this technology, opens the way for future novel therapeutic applications. For instance, inhibition of miRNAs that are commonly upregulated in diabetic nephropathy decreases albuminuria and mesangial matrix accumulation in animal models, suggesting that a therapeutic agent against these molecules may help to prevent the development of diabetic nephropathy. Certain challenges, including the development of safe and reliable delivery systems, remain to be overcome before miRNA-based therapeutics become a reality. However, the findings accumulated to date, in conjunction with newly emerging results, are expected to yield novel insights into the complex pathogenesis of diabetic nephropathy, and may eventually lead to the identification of improved therapeutic targets for treatment of this disease.

  10. Prevalence of IgA Deficiency in Children with Juvenile Rheumatoid Arthritis

    Directory of Open Access Journals (Sweden)

    Mohammad Hasan Moradinejad

    2011-03-01

    Full Text Available The purpose of this study was to investigate any association between IgA deficiency (IgAD and juvenile rheumatoid arthritis (JRA among Iranian children.This case-control study was carried out on 83 children who were diagnosed as JRA according to American College of Rheumatology (ACR criteria; Patients were admitted at the rheumatology clinic of Children's Medical Center, (Tehran. Serum immunoglobulins concentrations were determined by nephelometry method. Control group was 112 healthy children who were matched for age and gender. Informed consent obtained from all parents.Selective IgA deficiency (sIgAD was found only in a boy (1.2% among JRA children; however, partial IgA deficiency was found in 6(7.1% of patients with JRA and in 12(10.7% of control subjects, this difference was not statistically significant (p=0.46. Immunoglobulins levels in patients with JRA (IgM: 126.7±57.2, IgG: 1182.3±351 and IgA:169.3±98 were significantly higher than their controls (IgM: 104±52, IgG:802±220 and IgA: 94.6±47 (pPatients with growth failure had higher IgM, IgG and IgA levels in comparison with patients without growth failure; however, this difference was significant about IgM and IgG levels (p<0.05.In contrast to other similar studies, the number of IgAD did not differ significantly between JRA patients and their control counterpart; this might be partly due to the high rate of consanguineous marriages in Iran that resulted in increased prevalence of clinically undiagnosed partial IgAD in general population. Hence, future epidemiological studies are warranted to make it clear.

  11. Increased proportions of bacteria capable of cleaving IgA1 in the pharynx of infants with atopic disease

    DEFF Research Database (Denmark)

    Kilian, M; Husby, S; Høst, A;

    1995-01-01

    children showed a statistically significant increase in proportions of IgA1 protease-producing bacteria in the pharynx with increasing age. IgA1 protease-producing bacteria detected included Streptococcus mitis biovar 1, Haemophilus influenzae, Haemophilus parahaemolyticus, Streptococcus pneumoniae...

  12. Secretory IgA as a diagnostic tool for Pseudomonas aeruginosa respiratory colonization

    DEFF Research Database (Denmark)

    Aanaes, Kasper; Johansen, Helle Krogh; Poulsen, Steen Seier;

    2012-01-01

    BACKGROUND: Pseudomonas aeruginosa sinusitis may be the focus for intermittent lung colonization in patients with cystic fibrosis (CF). The sinusitis may induce elevated IgA levels in nasal secretion and saliva against P. aeruginosa. METHODS: 120 CF patients chronically infected, intermittently...... secretions and saliva than those without P. aeruginosa in the lungs, indicating that P. aeruginosa sinusitis may precede intermittent colonization and chronic infection of the lungs. CONCLUSIONS: Specific IgA against P. aeruginosa in nasal secretions and saliva can contribute to differentiation between...

  13. Childhood trauma and childhood urbanicity in relation to psychotic disorder

    NARCIS (Netherlands)

    Frissen, Aleida; Lieverse, Ritsaert; Drukker, Marjan; van Winkel, Ruud; Delespaul, Philippe; Cahn, W

    2015-01-01

    BACKGROUND: Urban upbringing and childhood trauma are both associated with psychotic disorders. However, the association between childhood urbanicity and childhood trauma in psychosis is poorly understood. The urban environment could occasion a background of social adversity against which any effect

  14. Quantification of β-region IgA monoclonal proteins - should we include immunochemical Hevylite® measurements? Point.

    Science.gov (United States)

    Evans, Josie A R; Jenner, Ellen L; Carr Smith, Hugh D; Berlanga, Oscar; Harding, Stephen J

    2016-06-01

    Accurate measurement of IgA monoclonal proteins presents a significant challenge to laboratory staff. IgA heavy/light chain (Hevylite, HLC) analysis is an alternative methodology for monoclonal protein assessment, giving an independent measure of IgAκ and IgAλ concentrations. Clonality is assessed by calculating the ratio of involved immunoglobulin to background uninvolved immunoglobulin concentrations (e.g. IgAκ/IgAλ in an IgAκ patient). Here we discuss the challenges faced by the laboratory in IgA monoclonal protein assessment, and compare the performance of Hevylite assays with electrophoresis and total IgA results. We present data which validates the use of Hevylite for response assessment: in most cases, Hevylite provides comparable response assignment to that provided by serum protein electrophoresis (SPE) and total IgA; in other cases Hevylite provides additional information, such as detection of residual disease or relapse.

  15. Characterization of the ligand binding site of the bovine IgA Fc receptor (bFc alpha R).

    Science.gov (United States)

    Morton, H Craig; Pleass, Richard J; Woof, Jenny M; Brandtzaeg, Per

    2004-12-24

    Recently, we identified a bovine IgA Fc receptor (bFc alpha R), which shows high homology to the human myeloid Fc alpha R, CD89. IgA binding has previously been shown to depend on several specific residues located in the B-C and F-G loops of the membrane-distal extracellular domain 1 of CD89. To compare the ligand binding properties of these two Fc alpha Rs, we have mapped the IgA binding site of bFc alpha R. We show that, in common with CD89, Tyr-35 in the B-C loop is essential for IgA binding. However, in contrast to earlier observations on CD89, mutation of residues in the F-G loop did not significantly inhibit IgA binding.

  16. Metadherin facilitates podocyte apoptosis in diabetic nephropathy

    Science.gov (United States)

    Liu, Wen-Ting; Peng, Fen-Fen; Li, Hong-Yu; Chen, Xiao-Wen; Gong, Wang-Qiu; Chen, Wen-Jing; Chen, Yi-Hua; Li, Pei-Lin; Li, Shu-Ting; Xu, Zhao-Zhong; Long, Hai-Bo

    2016-01-01

    Apoptosis, one of the major causes of podocyte loss, has been reported to have a vital role in diabetic nephropathy (DN) pathogenesis, and understanding the mechanisms underlying the regulation of podocyte apoptosis is crucial. Metadherin (MTDH) is an important oncogene, which is overexpressed in most cancers and responsible for apoptosis, metastasis, and poor patient survival. Here we show that the expression levels of Mtdh and phosphorylated p38 mitogen-activated protein kinase (MAPK) are significantly increased, whereas those of the microRNA-30 family members (miR-30s) are considerably reduced in the glomeruli of DN rat model and in high glucose (HG)-induced conditionally immortalized mouse podocytes (MPC5). These levels are positively correlated with podocyte apoptosis rate. The inhibition of Mtdh expression, using small interfering RNA, but not Mtdh overexpression, was shown to inhibit HG-induced MPC5 apoptosis and p38 MAPK pathway, and Bax and cleaved caspase 3 expression. This was shown to be similar to the effects of p38 MAPK inhibitor (SB203580). Furthermore, luciferase assay results demonstrated that Mtdh represents the target of miR-30s. Transient transfection experiments, using miR-30 microRNA (miRNA) inhibitors, led to the increase in Mtdh expression and induced the apoptosis of MPC5, whereas the treatment with miR-30 miRNA mimics led to the reduction in Mtdh expression and apoptosis of HG-induced MPC5 cells in comparison with their respective controls. Our results demonstrate that Mtdh is a potent modulator of podocyte apoptosis, and that it represents the target of miR-30 miRNAs, facilitating podocyte apoptosis through the activation of HG-induced p38 MAPK-dependent pathway. PMID:27882943

  17. Apelin retards the progression of diabetic nephropathy.

    Science.gov (United States)

    Day, Robert T; Cavaglieri, Rita C; Feliers, Denis

    2013-03-15

    Apelin and its receptor APJ have pleiotropic effects in mice and humans and play a protective role in cardiovascular diseases at least partially by inhibiting oxidative stress. Our objective was to study the effect of apelin on the progression of kidney disease in mice with established type 1 diabetes. Ove26 mice with type 1 diabetes received daily subcutaneous injections of apelin for 2 or 14 wk. APJ localizes in the glomeruli and blood vessels of kidneys. Renal APJ expression was reduced in diabetic mice but increased after treatment with apelin. Apelin treatment did not affect glycemia, body weight, or blood pressure in diabetic mice. Whole kidney and glomerular hypertrophy, as well as renal inflammation, including monocyte chemoattractant protein 1 and vascular cell adhesion molecule 1 expression, NF-κB activation, and monocyte infiltration, was inhibited after short and long treatment with apelin. Apelin administration significantly reduced albuminuria at 6 mo. Short treatment with apelin was sufficient to reverse the downregulation of the antioxidant enzyme catalase. Expression of angiotensin II and angiotensin type 1 receptor (AT1) in kidneys from diabetic mice treated was not affected by apelin. These findings show for the first time that apelin exerts a protective effect on the diabetic kidney. Short administration is sufficient to reduce kidney and glomerular hypertrophy as well as renal inflammation, but prolonged treatment is required to improve albuminuria. This effect was independent of the activation of the renin angiotensin system but correlated with upregulation of the antioxidant catalase. Apelin may represent a novel tool to treat diabetic nephropathy.

  18. Prevention of iodinated contrast-induced nephropathy

    Institute of Scientific and Technical Information of China (English)

    LI Jian-hua; HE Neng-shu

    2011-01-01

    Objective To lessen the occurrence of contrast-induced nephropathy (CIN),the preventive measures of CIN were reviewed.Data sources The data used in this review were from PubMed with relevant English articles and from Chinese Knowledge Information (CNKI) published from 1989 to 2009.The search terms were “contrast medium”,“contrast-induced nephropathy” and “prevention”.Articles involved in prevention of CIN were selected.Study selection CIN is the third most common cause of acute kidney injury and is associated with an unfavorable prognosis.The best treatment is prophylaxis because CIN can not be reversed or ameliorated.Results Thirty articles were included.Among various preventive measures,pericatheterization hydration is almost universally accepted as an appropriate and safe measure to prevent CIN,although there is no agreement as to composition,amount,and timing of hydration.Based on the use of concomitant nephrotoxic agents or high doses of contrast medium (CM) is one of risk factors for CIN,discontinuation of potentially nephrotoxic drugs 2-3 days before and after the procedure until renal function recover,and using the lowest possible dose of CM can decrease the risk of CIN.It is promising that removing the majority of CM from the coronary sinus,before it enters the systemic circulation,during coronary angiography can reduce the risk for CIN in animal studies and in limited clinical trials.Inconsistent data exist with respect to application of some vasodilators (endothelin antagonists and adenosine antagonists) and antioxidants (N-acetylcysteine and statins) in preventing CIN in high-risk patients,and new vasodilators and antioxidants continue to be tested.Conclusions Pericatheterization hydration,discontinuation of nephrotoxic drugs,and using the lowest possible dose of CM are effective measures to lessen the risk for CIN.Other prophylactic strategies and some drugs are promising,but further confirmation is required.

  19. Validation and reevaluation of the pathology classification in IgA nephropathy%IgA肾病病理分型的验证与再评价

    Institute of Scientific and Technical Information of China (English)

    师素芳; 张宏

    2013-01-01

    IgA肾病的病理表现复杂多样,一直以来没有一个被广为接受的病理分型.IgA肾病牛津病理分型的制定过程非常严谨和科学,自2009 年发表至今引起了广泛关注,不同国家对该分型进行了验证,但由于入选病例临床表现不同、随访过程中治疗方法不同、种族的差异,以及多为小样本、少终点事件的回顾性研究,因此导致目前验证研究的结果之间存在很大差异.最新的研究通过对目前已有的12 项验证研究进行的荟萃分析,发现系膜细胞增生病变、节段性硬化或粘连病变及肾小管萎缩或间质纤维化病变是影响IgA肾病肾脏预后的独立病理指标,内皮细胞增生病变不是影响肾脏预后的病理指标.IgA肾病病理分型的临床意义和应用价值有待进一步的大样本、多中心、前瞻性研究进行证实.

  20. Immunohistochemical Aspects of Cell Death in Diabetic Nephropathy

    Directory of Open Access Journals (Sweden)

    Bălăşescu Elena

    2016-03-01

    Full Text Available Introduction. Diabetes Mellitus causes ultrastructural changes triggered by partially clarified cellular mechanisms. Since cell death is an important mechanism in the appearance and progression of diabetic nephropathy, we studied alteration of several markers of apoptotic pathways signaling in renal tissue of diabetic or prediabetic patients.

  1. Case report: analgesic nephropathy: a soda and a powder.

    Science.gov (United States)

    Appel, R G; Bleyer, A J; McCabe, J C

    1995-10-01

    Analgesic nephropathy has long been considered a potentially preventable cause of renal disease. Early reports were described in patients who consumed analgesics containing phenacetin. In recent data, the removal of phenacetin from analgesic preparations resulted in a reduction in analgesic-induced end stage renal disease in Europe and Australia. However, a reduction in the incidence of analgesic nephropathy has not occurred uniformly, suggesting that phenacetin is not the sole cause. Current data raise concerns regarding adverse renal effects of acetaminophen and nonsteroidal antiinflammatory drugs. Aspirin taken alone may be of least concern. The diagnosis of analgesic nephropathy is suggested in subjects with chronic renal failure, a history of daily consumption of analgesic preparations, small bumpy kidneys, and renal papillary necrosis or chronic interstitial nephritis. However, the spectrum of disease may be changing, because these agents also may increase the risk of cardiovascular disease and chronic renal disease due to nephrosclerosis, glomerulonephritis, and diabetes mellitus. Potential pathogenetic mechanisms in analgesic nephropathy include direct cellular injury induced by analgesics, prostaglandin inhibition with reduction or redistribution of renal blood flow, and interesting new concepts regarding the role of caffeine in increasing oxygen demand and reducing oxygen supply in the medulla. The primary goal of therapy is discontinuation of analgesic consumption. Because of the association between analgesic intake and uroepithelial tumors, surveillance of patients for neoplasm is suggested.

  2. Environmental renal disease: Lead, cadmium and Balkan endemic nephropathy

    Energy Technology Data Exchange (ETDEWEB)

    Wedeen, R.P. (VA Medical Center, East Orange, NJ (United States))

    1991-11-01

    The similarity of lead and cadmium nephropathy to Balkan endemic nephropathy warrants careful reevaluation of the possibility that these nephrotoxic metals contribute to the production of the endemic renal disease. Low-level environmental exposure may result in a relationship between the concentration of the metals in tissue storage sites and biological fluids that differs from that encountered after occupational exposure. Urine and blood concentrations may therefore be inadequate measures of exposure. Lead is accumulated in the skeleton and cadmium in the liver and kidneys with biological half lives approximating a decade. Non-invasive in vivo x-ray fluorescence or neutron activation analysis can therefore be used to measure cumulative tissue stores. Multiple regression analysis of epidemiologic data could reveal the relative contribution of causal factors, including lead and cadmium, and help to distinguish Balkan endemic nephropathy from other renal diseases using rigorous diagnostic criteria. As long as Balkan endemic nephropathy remains a diagnosis of exclusion, the accuracy of the diagnosis of other renal disease determines the reliability of identification of the endemic disease.31 references.

  3. Chronic constipation causing obstructive nephropathy in a delayed toddler.

    LENUS (Irish Health Repository)

    Barrett, Michael Joseph

    2012-01-01

    Chronic constipation causing obstructive nephropathy is very rare in children. However, it can cause urinary tract obstruction with acute impairment of renal function with a need for emergent disimpaction. The authors discuss a 2 years 4 months old child who presented to our emergency department with acute renal failure due to faecal impaction.

  4. Membranous nephropathy in the older adult: epidemiology, diagnosis and management.

    NARCIS (Netherlands)

    Deegens, J.K.J.; Wetzels, J.F.M.

    2007-01-01

    Membranous nephropathy is the most important cause of the nephrotic syndrome in elderly patients (aged >65 years). The clinical presentation is similar in older and younger patients, although elderly patients more often present with renal failure. Notably, glomerular filtration rate (GFR) is usua

  5. [The role of ramipril in the therapy of diabetic nephropathy].

    Science.gov (United States)

    Dézsi, Csaba András

    2014-02-16

    In the past two decades the number of diabetic patients has increased dramatically. According to the data of the International Diabetes Federation published in 2012, more than 371 million people suffer from diabetes mellitus, which is responsible for the death of 4.8 million people yearly. Diabetic nephropathy is the most frequent cause of terminal renal failure. The first stage of its development is microalbuminuria. Without an efficient treatment 20-40% of the patients with microalbuminuria suffering from type 2 diabetes mellitus develop chronic renal failure, but only 20% of them become uremic because most of them die beforehand mainly due to cardiovascular disease. The renin-angiotensin-system, which is one of the most important elements of the regulation of blood pressure and water-salt metabolism, plays an important role in the development of diabetic nephropathy. Drugs affecting the function of this system are of great significance in the treatment of hypertension. The author rewiews the results of several important studies and animal experiments to demonstrate the role of ramipril in the therapy of diabetic nephropathy. The author concludes that ramipril is one of the angiotensin-converting enzyme inhibitors with the highest number of evidence based beneficial results. Apart from its blood pressure decreasing effect, ramipril protects target organs and it proved to be effective in the treatment of diabetic nephropathy according to most international multicenter clinical trials. Orv. Hetil., 2014, 155(7), 263-269.

  6. Current concepts in the management of diabetic nephropathy

    NARCIS (Netherlands)

    Waanders, F.; Visser, F. W.; Gans, R. O. B.

    2013-01-01

    Although much progress has been made in slowing the progression of diabetic nephropathy, renal dysfunction and development of end-stage renal disease (ESRD) remain major concerns in diabetes. In addition, diabetic patients with microalbuminuria have an increased cardiovascular mortality. Therefore,

  7. Calorie restriction mimicking effects of roflumilast prevents diabetic nephropathy.

    Science.gov (United States)

    Tikoo, Kulbhushan; Lodea, Saritha; Karpe, Pinakin Arun; Kumar, Sandeep

    2014-08-08

    Little is known about role of PDE4 in the development and progression of diabetic nephropathy. Here, we investigated the effect of roflumilast, a selective PDE 4 inhibitor in type 1 diabetic nephropathy. Diabetes was induced in male Sprague-Dawley rats using streptozotocin (55 mg/kg). Diabetic rats showed elevated plasma glucose, blood urea nitrogen, creatinine and decrease in plasma albumin confirming signs of nephropathy. Roflumilast at 2 and 3mg/kg normalized these alterations. Roflumilast also suppressed oxidative stress and deposition of an extracellular matrix protein such as fibronectin and collagen in kidney of diabetic rats. TUNEL assay revealed apoptosis in diabetic kidney than control and that roflumilast prevents this effect. We show that kidney of diabetic rats displayed a state of p-AMPK and SIRT1 deficiency and that roflumilast, interestingly, was able to restore their levels. Further, roflumilast prevented an increase in HO-1 and loss in the FoxO1 expression in diabetes. However, it did not improve the reduced NRF2 levels in diabetes. This is the first report to show that, like resveratrol and other SIRT1 activators, roflumilast also mimics calorie restriction effects through activation of AMPK/SIRT1 and protects against diabetic nephropathy. This study unveils the unexplored potential of roflumilast which can be used in treatment of metabolic disorders.

  8. Overexpression of Mafb in podocytes protects against diabetic nephropathy.

    Science.gov (United States)

    Morito, Naoki; Yoh, Keigyou; Ojima, Masami; Okamura, Midori; Nakamura, Megumi; Hamada, Michito; Shimohata, Homare; Moriguchi, Takashi; Yamagata, Kunihiro; Takahashi, Satoru

    2014-11-01

    We previously showed that the transcription factor Mafb is essential for podocyte differentiation and foot process formation. Podocytes are susceptible to injury in diabetes, and this injury leads to progression of diabetic nephropathy. In this study, we generated transgenic mice that overexpress Mafb in podocytes using the nephrin promoter/enhancer. To examine a potential pathogenetic role for Mafb in diabetic nephropathy, Mafb transgenic mice were treated with either streptozotocin or saline solution. Diabetic nephropathy was assessed by renal histology and biochemical analyses of urine and serum. Podocyte-specific overexpression of Mafb had no effect on body weight or blood glucose levels in either diabetic or control mice. Notably, albuminuria and changes in BUN levels and renal histology observed in diabetic wild-type animals were ameliorated in diabetic Mafb transgenic mice. Moreover, hyperglycemia-induced downregulation of Nephrin was mitigated in diabetic Mafb transgenic mice, and reporter assay results suggested that Mafb regulates Nephrin directly. Mafb transgenic glomeruli also overexpressed glutathione peroxidase, an antioxidative stress enzyme, and levels of the oxidative stress marker 8-hydroxydeoxyguanosine decreased in the urine of diabetic Mafb transgenic mice. Finally, Notch2 expression increased in diabetic glomeruli, and this effect was enhanced in diabetic Mafb transgenic glomeruli. These data indicate Mafb has a protective role in diabetic nephropathy through regulation of slit diaphragm proteins, antioxidative enzymes, and Notch pathways in podocytes and suggest that Mafb could be a therapeutic target.

  9. Progression of nephropathy in type 2 diabetic patients

    DEFF Research Database (Denmark)

    Rossing, Kasper; Christensen, Per Klitgaard; Hovind, Peter

    2004-01-01

    BACKGROUND: Nephropathy in type 2 diabetes is the single most common cause of end-stage renal disease (ESRD), but the decline in kidney function varies considerably between individuals, and determinants of renal function loss, early in the course of renal disease, have not been clearly identified...

  10. Impaired Podocyte Autophagy Exacerbates Proteinuria in Diabetic Nephropathy.

    Science.gov (United States)

    Tagawa, Atsuko; Yasuda, Mako; Kume, Shinji; Yamahara, Kosuke; Nakazawa, Jun; Chin-Kanasaki, Masami; Araki, Hisazumi; Araki, Shin-Ichi; Koya, Daisuke; Asanuma, Katsuhiko; Kim, Eun-Hee; Haneda, Masakazu; Kajiwara, Nobuyuki; Hayashi, Kazuyuki; Ohashi, Hiroshi; Ugi, Satoshi; Maegawa, Hiroshi; Uzu, Takashi

    2016-03-01

    Overcoming refractory massive proteinuria remains a clinical and research issue in diabetic nephropathy. This study was designed to investigate the pathogenesis of massive proteinuria in diabetic nephropathy, with a special focus on podocyte autophagy, a system of intracellular degradation that maintains cell and organelle homeostasis, using human tissue samples and animal models. Insufficient podocyte autophagy was observed histologically in patients and rats with diabetes and massive proteinuria accompanied by podocyte loss, but not in those with no or minimal proteinuria. Podocyte-specific autophagy-deficient mice developed podocyte loss and massive proteinuria in a high-fat diet (HFD)-induced diabetic model for inducing minimal proteinuria. Interestingly, huge damaged lysosomes were found in the podocytes of diabetic rats with massive proteinuria and HFD-fed, podocyte-specific autophagy-deficient mice. Furthermore, stimulation of cultured podocytes with sera from patients and rats with diabetes and massive proteinuria impaired autophagy, resulting in lysosome dysfunction and apoptosis. These results suggest that autophagy plays a pivotal role in maintaining lysosome homeostasis in podocytes under diabetic conditions, and that its impairment is involved in the pathogenesis of podocyte loss, leading to massive proteinuria in diabetic nephropathy. These results may contribute to the development of a new therapeutic strategy for advanced diabetic nephropathy.

  11. Transforming growth factor-β in diabetic nephropathy

    Directory of Open Access Journals (Sweden)

    Karima Y. Ahmed

    2013-01-01

    Conclusion Serum TGF-β level increases in patients of both type 1 and type 2 diabetes and in those with diabetic nephropathy. TGF-β is considered one of the major mediators of diabetic renal fibrogenesis that Results in end-stage renal disease.

  12. Linear IgA bullous dermatosis: report of an exuberant case.

    Science.gov (United States)

    Souza, Beatriz Cavalcanti de; Fregonesi, Nádire Cristina Freire Pontes; Tebcherani, Antônio José; Sanchez, Ana Paula Galli; Aoki, Valéria; Fernandes, Juliana Christien

    2013-01-01

    Linear immunoglobulin A dermatosis is a rare autoimmune bullous disease, but the most common autoimmune bullous dermatosis in children. We report a typical exuberant case of linear IgA dermatosis in a ten-month old child, who showed good response to treatment with corticosteroids and dapsone.

  13. Linear IgA bullous dermatosis: report of an exuberant case*

    OpenAIRE

    Souza,Beatriz Cavalcanti de; Fregonesi, Nádire Cristina Freire Pontes; Tebcherani,Antônio José; Sanchez,Ana Paula Galli; AOKI, Valéria; Fernandes, Juliana Christien

    2013-01-01

    Linear immunoglobulin A dermatosis is a rare autoimmune bullous disease, but the most common autoimmune bullous dermatosis in children. We report a typical exuberant case of linear IgA dermatosis in a ten-month old child, who showed good response to treatment with corticosteroids and dapsone.

  14. Iga tööotsija saab isikliku tegutsemiskava / Eva Kübar

    Index Scriptorium Estoniae

    Kübar, Eva

    2006-01-01

    Alates 2006. aastast saab iga töötu isikliku tööotsimiskava, mille täitmist hakkab jälgima tööturuamet. Lisa: Individuaalne tööotsimiskava. Ilmunud ka: ; Postimees : na russkom jazõke 4. jaan. lk. 3

  15. Inducement of IGA/SCC in Inconel 600 steam generator tubing during unit outages

    Energy Technology Data Exchange (ETDEWEB)

    Durance, D.; Sedman, K. [Bruce Power, Tiverton, Ontario (Canada); Roberts, J. [CANTECH Associates Ltd., Burlington, Ontario (Canada); King, P. [Babcock and Wilcox Canada, Cambridge, Ontario (Canada); Gorman, J. [Dominion Engineering, Reston, VA (United States); Allen, R. [Kinectrics, Inc., Toronto, Ontario (Canada)

    2008-07-01

    The degradation of Unit 4 SG tubing by IGA/SCC has limited both the operating period and end of life predictions for Unit 4 since restart in late 2003. The circumferential IGA/SCC has been most significant in SG4 with substantial increases in both initiation and growth rates from 2005 through the spring of 2007. A detailed review of the occurrence of circumferential OD IGA/SCC at the RTZ in the HL TTS region of Bruce 4 steam generator tubes has led a conclusion that it is probable that the IGA/SCC has been the result of attack by partially reduced sulfur species such as tetrathionates and thiosulfates during periods of low temperature exposure. It is believed that attack of this type has mostly likely occurred during startup evolutions following outages as the result the development of aggressive reduced sulfur species in the TTS region during periods when the boilers were fully drained for maintenance activities. The modification of outage practices to limit secondary side oxygen ingress in the spring of 2007 has apparently arrested the degradation and has had significant affects on the allowable operating interval and end of life predictions for the entire unit. (author)

  16. Amphibians have immunoglobulins similar to ancestral IgD and IgA from Amniotes.

    Science.gov (United States)

    Estevez, Olivia; Garet, Elina; Olivieri, David; Gambón-Deza, Francisco

    2016-01-01

    We studied the immunoglobulin genes from either the genomes or RNAs of amphibians. In particular, we obtained data from one frog genome (Nanorana parkeri) and three transcriptomes of the Caudata order (Andrias davidianus, Notophthalmus viridescens and Cynops pyrrhogaster). Apart from the immunoglobulins IgM and IgY previously described, we identified several IgD related immunoglobulins. The species N. parkeri, N. viridescens and C. pyrrhogaster have two IgD genes, while Andrias davidianus has three such genes. The three Caudata species have long IgD immunoglobulins similar to IgD of reptiles, and could be an ancient relic from the common ancestor of IgD of all mammals and reptiles. We also found two IgA isotypes. The results suggest that one of the IgA may be the ancestor of IgA in crocodiles and birds, while the other could be the ancestor IgA found in mammals. These results provide information that could help understand the evolution of immunoglobulins in terrestrial vertebrates.

  17. Selective IgA deficiency%选择性IgA缺乏症

    Institute of Scientific and Technical Information of China (English)

    张凡; 涂平; 张艳; 周炜; 唐福林; 邝捷

    2012-01-01

    报告1例选择性IgA缺乏症.患儿男,10岁.因皮下结节2年余就诊.血清IgA含量持续≤50 mg/L,皮损组织病理检查显示慢性炎症性改变,个别小血管纤维蛋白样变性,管腔狭窄、闭塞,红细胞外渗.免疫组化染色显示CD20少量表达,符合选择性IgA缺乏症的诊断.该病临床多并发反复肺部感染、自身免疫性疾病及肿瘤等.%A case of selective IgA deficiency is reported. A 10-year-old boy presented with subcutaneous nodules for 2 years. Laboratory study showed a stable lower concentration of serum IgA (≤50mg/L). Histological examination on skin lesions revealed chronic cutaneous inflammation, with fibrinoid degeneration of blood vessels and the extravasation of erythrocytes. The narrow and even the closure of the vessels could be seen. Immunohistochemical study showed a mild positive staining for CD20. These findings are consistent with the selective IgA deficiency. There is a tendency in the patients with selective IgA deficiency to develop recurrent pulmonary infections, autoimmune disorders and malignancies.

  18. Immunoglobulin M Nephropathy in a Patient with Wilson's Disease.

    Science.gov (United States)

    Ul Abideen, Zain; Sajjad, Zoya; Haroon Khan, Asna; Mamoon, Nadira; Bilal, Muhammad; Mujtaba Quadri, Khaja Hameeduddin

    2016-12-13

    Immunoglobulin M nephropathy (IgMN) is characterized by the deposition of immunoglobulin M in a dominant distribution in the renal glomeruli. Primary immunoglobulin M nephropathy is diagnosed after consistent light microscopy (LM), immunofluorescence (IF), electron microscopy (EM) results, and exclusion of known systemic disorders causing immunoglobulin M deposition in the glomeruli. The secondary disease has been reported with a few conditions though it has never been reported with any primary disease of the liver. We report the case of an adolescent male patient who presented with nausea, vomiting, diarrhea, and worsening anasarca. He was found to have nephrotic-range proteinuria that did not respond to conventional corticosteroid treatment. He was subjected to a renal biopsy which revealed a diagnosis of immunoglobulin M nephropathy. His liver function tests were deranged and an ultrasound scan of the abdomen revealed a coarse irregular liver. Workup revealed elevated urine copper excretion and a low ceruloplasmin level. He was diagnosed as a case of Wilson's disease and started on penicillamine and pyridoxine. He was also started on intravenous cyclophosphamide for the corticosteroid-resistant nephrotic syndrome to which he responded remarkably well. His edema settled, proteinuria resolved, and liver functions normalized. Currently, he is in remission and enjoying good health. To the best of our knowledge, we report the first known association between IgM nephropathy and Wilson's disease. It is presently not clear if causation can necessarily be established. This may be the result of defective IgM clearance by the liver or an altered metabolism of the antibody or immune complexes, as with hepatic-associated immunoglobulin M (IgM) nephropathy. Further studies are needed to elucidate the exact mechanism of this disease.

  19. The Evaluation of Psychological Factor and Salivary Cortisol and IgA Levels in

    Directory of Open Access Journals (Sweden)

    Fateme Arbabi-Kalati

    2014-07-01

    Full Text Available Background: Oral lichen planus (OLP is a chronic immunological disorder with unknown etiology. The aim of this study was to determine psychological factors and salivary cortisol, IgA level in patients with oral lichen planus. Materials and Methods: In this experimental study 20 patients with OLP and healthy person were admitted to this study. Saliva samples were collected between - Am. saliva cortisol, IgA level was detected by ELIZA method. In this study, patients with anxiety and depression were measured using the SCL-90 questionnaire. Data analyzed by t-test. Results: The mean salivary cortisol level in patients with OLP was 3.2±1.9 ng/mL and the mean saliva cortisol level in healthy person was 3.5±1.9 ng/mL. Significant difference was observed in the salivary cortisol levels in the 2 study groups (p=0.04. The mean salivary IgA level in patients with OLP was 0.69±0.29 ng/mL and the mean saliva IgA level in healthy person was 0.9±0.43 ng/mL but no significant difference was observed in the salivary cortisol levels in the 2 study groups. Results showed that anxiety levels in patients with oral lichen planus were slightly higher than controls but there was no significant difference between healthy subjects. Conclusion: Finding revealed the mean salivary cortisol level in patient with OLP less than healthy persons. Significant difference was observed in the salivary cortisol levels in the 2 study groups. Based on the t-student test, no significant difference was observed in the salivary IgA levels in the 2 study groups. Anxiety levels in patients with oral lichen planus were slightly higher than controls.

  20. Levels and complexity of IgA antibody against oral bacteria in samples of human colostrum.

    Science.gov (United States)

    Petrechen, L N; Zago, F H; Sesso, M L T; Bertoldo, B B; Silva, C B; Azevedo, K P; de Lima Pereira, S A; Geraldo-Martins, V R; Ferriani, V P L; Nogueira, R D

    2015-01-01

    Streptococcus mutans (SM) have three main virulence antigens: glucan binding protein B (gbpB), glucosyltransferase (Gtf) and antigens I/II (Ag I/II) envolved in the capacity of those bacteria to adhere and accumulate in the dental biofilm. Also, the glycosyltransferases 153 kDa of Streptococcus gordonii (SGO) and 170kDa of Streptococcus sanguinis (SSA) were important antigens associated with the accumulation of those bacterias. Streptococcus mitis (SMI) present IgA1 protease of 202 kDa. We investigated the specificity and levels IgA against those antigens of virulence in samples of human colostrum. This study involved 77 samples of colostrum that were analyzed for levels of immunoglobulian A, M and G by Elisa. The specificity of IgA against extracts of SM and initials colonizators (SSA, SMI, SGO) were analyzed by the Western blot. The mean concentration of IgA was 2850.2 (±2567.2) mg/100 mL followed by IgM and IgG (respectively 321.8±90.3 and 88.3±51.5), statistically different (pbacteria antigens and theirs virulence antigens. To SM, the GbpB was significantly lower detected than others antigens of SM (p0.4). So, the breast milk from first hours after birth presented significant levels of IgA specific against important virulence of antigens those oral streptococci, which can disrupt the installation and accumulation process of these microorganisms in the oral cavity.

  1. IgG, IgA, and lysozyme in Martina Franca donkey jennies and their foals.

    Science.gov (United States)

    Veronesi, Maria C; Dall'Ara, Paola; Gloria, Alessia; Servida, Francesco; Sala, Elisabetta; Robbe, Domenico

    2014-04-01

    Because immune transfer from jenny to donkey foal is mostly unknown, the aim of the present study was to evaluate, from 5 days before to 10 days after foaling, immunoglobulin (Ig)G, IgA, and lysozyme peripartal concentrations in serum and mammary secretions of 10 healthy, spontaneously foaling Martina Franca jennies and in serum of their mature, viable, healthy foals, in the first 10 days after birth. The results showed that, in jennies, mammary secretion of IgG levels (ranging between 16 and 75 mg/mL) and IgA (0.9-2 mg/mL), and IgG (6.8-13.5 mg/mL) and IgA (0.5-2.4 mg/mL) serum concentrations were not different along the time of study. Also, IgG concentrations in serum of foals did not show significant differences although a high level was observed at 12 hours after birth (8 mg/mL), and IgA concentrations in serum of foals did not show any significant difference, although a high level was observed at 12 hours after birth (1.2 mg/mL). Lysozyme increased significantly at Day 2 after parturition in mammary secretions of jennies (551.9 μg/mL) and at 12 hours in serum of foals (25.9 μg/mL). The study demonstrated that the pattern of passive immune transfer in donkey foals seems to be similar to that reported for the horse foal, with IgG predominating IgA in serum and mammary secretions of the jenny and also in serum of foals. The most significant early increase in foals' serum concerns lysozyme, which probably plays an important role in the innate immunity of the donkey foal in the first challenging hours after birth.

  2. Childhood Roots of Schizophrenia

    Science.gov (United States)

    Watt, Norman F.; Lubensky, Amy W.

    1976-01-01

    Earlier project reports compared childhood social behavior of nonmigratory schizophrenics and normal classmates by analyzing teachers' comments in school records. This article expands the sample to include migratory schizophrenics and analyzes childhood intellectual functioning. Behavioral differences indicated emotional immaturity and social…

  3. Childhood Overweight and Obesity

    Science.gov (United States)

    ... Home Prevention and Wellness Food and Nutrition Healthy Food Choices Childhood Overweight and Obesity Childhood Overweight and Obesity Family HealthFood and NutritionHealthy Food ChoicesKids and TeensPrevention and WellnessWeight Loss and Diet ...

  4. Undiagnosed asthma in childhood

    NARCIS (Netherlands)

    van Gent, R.

    2008-01-01

    Asthma is the most prevalent chronic disease in childhood. To study the actual prevalence and impact of undiagnosed childhood asthma in daily life (i.e. quality of life, participation in physical and school activities) we performed a survey in schoolchildren (aged 7-10 years) in the southern part of

  5. Effects of pregnancy on the onset and progression of diabetic nephropathy and of diabetic nephropathy on pregnancy outcomes.

    Science.gov (United States)

    Young, Esther Cytrynbaum; Pires, Maria Lucia Elias; Marques, Luiz Paulo José; de Oliveira, José Egídio Paulo; Zajdenverg, Lenita

    2011-01-01

    Controversy exists regarding the effect of pregnancy on the development and course of diabetic nephropathy. This study followed 43 pregnant women with previous diabetes mellitus, 32 without nephropathy (Group I) and 11 with nephropathy (Group II). Urinary albumin excretion (UAE), serum creatinine (Cr) and creatinine clearance (CCr) in the pre-pregnancy (Pre-P), first trimester (1T), third trimester (3T) and 1 year postpartum (PP) were evaluated. In both groups there were an increase in 3T compared to Pre-P of CCr (137 vs. 98 ml/min and 110 vs. 81 ml/min, p=0.0001, respectively) and UAE (7.78 vs. 3.15 mg/24 h and 592 vs. 119 mg/24 h, p=0.0001, respectively). Increase of Cr in the PP compared to 1T in Group II (0.88 vs. 0.70 mg/dL, p=0.031) was observed. There were no difference in UAE, CCr and Cr in the PP when compared to pre-P as well variance over time between groups. Group II showed higher prevalence of chronic hypertension (72.7 vs. 21.9%, p=0.004), preeclampsia (63.6 vs. 6.3%, p=0.0003) and lower gestational age at birth (36 vs. 38 weeks, p=0.003). We conclude that pregnancy was not associated with development and progression of diabetic nephropathy in women with or without mild renal dysfunction. The presence of diabetic nephropathy was associated with increased risk of perinatal complications.

  6. PetIGA-MF: a multi-field high-performance toolbox for structure-preserving B-splines spaces

    KAUST Repository

    Sarmiento, A.F.

    2016-10-01

    We describe a high-performance solution framework for isogeometric discrete differential forms based on B-splines: PetIGA-MF. Built on top of PetIGA, an open-source library we have built and developed over the last decade, PetIGA-MF is a general multi-field discretization tool. To test the capabilities of our implementation, we solve different viscous flow problems such as Darcy, Stokes, Brinkman, and Navier-Stokes equations. Several convergence benchmarks based on manufactured solutions are presented assuring optimal convergence rates of the approximations, showing the accuracy and robustness of our solver.

  7. Lack of Association between TLR4 Genetic Polymorphisms and Diabetic Nephropathy in a Chinese Population

    Directory of Open Access Journals (Sweden)

    Danfeng Peng

    2014-01-01

    Full Text Available Objective. Toll-like receptor 4 (TLR4 plays a central role in innate immunity. Activation of innate immune response and subsequent chronic low-grade inflammation are thought to be involved in the pathogenesis of diabetic nephropathy. In this study, we aimed to investigate whether TLR4 variants are associated with diabetic nephropathy in the Chinese population. Methods. Seven tagging single nucleotide polymorphisms (SNPs of TLR4 based on HapMap Chinese data were genotyped in 1,455 Chinese type 2 diabetic patients. Of these patients, 622 were diagnosed with diabetic nephropathy and 833 were patients with diabetes for over 5 years but without diabetic nephropathy. Results. None of the SNPs and haplotypes showed significant association to diabetic nephropathy in our study. No association between the SNPs and quantitative traits was observed either. Conclusion. We concluded that common variants within TLR4 genes were not associated with diabetic nephropathy in the Chinese type 2 diabetes patients.

  8. The role of IL-18 in type 1 diabetic nephropathy: The problem and future treatment.

    Science.gov (United States)

    Elsherbiny, Nehal M; Al-Gayyar, Mohammed M H

    2016-05-01

    Diabetic vascular complication is a leading cause of diabetic nephropathy, a progressive increase in urinary albumin excretion coupled with elevated blood pressure leading to declined glomerular filtration and eventually end stage renal failure. There is growing evidence that activated inflammation is contributing factor to the pathogenesis of diabetic nephropathy. Meanwhile, IL-18, a member of the IL-1 family of inflammatory cytokines, is involved in the development and progression of diabetic nephropathy. However, the benefits derived from the current therapeutics for diabetic nephropathy strategies still provide imperfect protection against renal progression. This imperfection points to the need for newer therapeutic agents that have potential to affect primary mechanisms contributing to the pathogenesis of diabetic nephropathy. Therefore, the recognition of IL-18 as significant pathogenic mediators in diabetic nephropathy leaves open the possibility of new potential therapeutic targets.

  9. Decreased ADP-Ribosyl Cyclase Activity in Peripheral Blood Mononuclear Cells from Diabetic Patients with Nephropathy

    Directory of Open Access Journals (Sweden)

    Michio Ohtsuji

    2008-01-01

    Results. ADPRCA negatively correlated with the level of HbA1c (=.040, 2=.073, although ADPRCA showed no significant correlation with gender, age, BMI, blood pressure, level of fasting plasma glucose and lipid levels, as well as type, duration, or medication of diabetes. Interestingly, patients with nephropathy, but not other complications, presented significantly lower ADPRCA than those without nephropathy (=.0198 and diabetes (=.0332. ANCOVA analysis adjusted for HbA1c showed no significant correlation between ADPRCA and nephropathy. However, logistic regression analyses revealed that determinants for nephropathy were systolic blood pressure and ADPRCA, not HbA1c. Conclusion/interpretation. Decreased ADPRCA significantly correlated with diabetic nephropathy. ADPRCA in PBMCs would be an important marker associated with diabetic nephropathy.

  10. Nephropathy in type 1 diabetes is associated with increased circulating activated platelets and platelet hyperreactivity

    DEFF Research Database (Denmark)

    Tarnow, Inge; Michelson, Alan D.; Barnard, Marc R.;

    2009-01-01

    Patients with diabetes mellitus (DM) have increased platelet activation compared to non-diabetic controls. Platelet hyperreactivity has been associated with adverse cardiovascular outcomes in Type 2 DM, and with diabetic nephropathy. We investigated the relationship between platelet activation...... and nephropathy in Type 1 DM. Patients with Type 1 DM and diabetic nephropathy (n = 35), age- and sex-matched Type 1 DM patients with persistent normoalbuminuria (n = 51), and healthy age- and sex-matched controls (n = 30) were studied. Platelet surface P-selectin, platelet surface activated GPIIb/IIIa, monocyte...... to 0.5 or 20 microM ADP) was higher in nephropathy patients compared with normoalbuminuric patients (P = 0.027), and non-diabetic controls (P = 0.0057). NPAs were higher in nephropathy patients compared to normoalbuminuric patients (P = 0.0088). MPAs were higher in nephropathy patients compared to non-diabetic...

  11. IgA production in the large intestine is modulated by a different mechanism than in the small intestine: Bacteroides acidifaciens promotes IgA production in the large intestine by inducing germinal center formation and increasing the number of IgA+ B cells.

    Science.gov (United States)

    Yanagibashi, Tsutomu; Hosono, Akira; Oyama, Akihito; Tsuda, Masato; Suzuki, Ami; Hachimura, Satoshi; Takahashi, Yoshimasa; Momose, Yoshika; Itoh, Kikuji; Hirayama, Kazuhiro; Takahashi, Kyoko; Kaminogawa, Shuichi

    2013-04-01

    It has been demonstrated that intestinal commensal bacteria induce immunoglobulin (Ig) A production by promoting the development of gut-associated lymphoid tissues in the small intestine. However, the precise mechanism whereby these bacteria modulate IgA production in the large intestine, which harbors the majority of intestinal commensals, is poorly understood. In addition, it is not known which commensal bacteria induce IgA production in the small intestine and which induce production in the large intestine. To address these issues, we generated gnotobiotic mice mono-associated with different murine commensal bacteria by inoculating germ-free (GF) mice with Lactobacillus johnsonii or Bacteroides acidifaciens. In GF mice, IgA production was barely detectable in the small intestine and was not detected in the large intestine. Interestingly, total IgA secretion in the large intestinal mucosa of B. acidifaciens mono-associated (BA) mice was significantly greater than that of GF and L. johnsonii mono-associated (LJ) mice. However, there was no difference in total IgA production in the small intestine of GF, LJ and BA mice. In addition, in the large intestine of BA mice, the expression of IgA(+) cells and germinal center formation were more remarkable than in GF and LJ mice. Furthermore, B. acidifaciens-specific IgA was detected in the large intestine of BA mice. These results suggest that the production of IgA in the large intestine may be modulated by a different mechanism than that in the small intestine, and that B. acidifaciens is one of the predominant bacteria responsible for promoting IgA production in the large intestine.

  12. Prevalence and Determinants of Diabetic Nephropathy in Korea: Korea National Health and Nutrition Examination Survey

    OpenAIRE

    Jae Hee Ahn; Ji Hee Yu; Seung-Hyun Ko; Hyuk-Sang Kwon; Dae Jung Kim; Jae Hyeon Kim; Chul Sik Kim; Kee-Ho Song; Jong Chul Won; Soo Lim; Sung Hee Choi; Kyungdo Han; Bong-Yun Cha; Nan Hee Kim

    2014-01-01

    Background Diabetic nephropathy is a leading cause of end stage renal disease and is associated with an increased risk of cardiovascular mortality. It manifests as albuminuria or impaired glomerular filtration rate (GFR), and the prevalence of diabetic nephropathy varies with ethnicity. The prevalence of diabetic nephropathy and its determinants in Korean adults have not previously been studied at the national level. This cross-sectional study was undertaken to ascertain the prevalence and de...

  13. The Protective Role of Nrf2 in Streptozotocin-Induced Diabetic Nephropathy

    OpenAIRE

    Jiang, Tao; Huang, Zheping; Lin, Yifeng; Zhang, Zhigang; Fang, Deyu; Zhang, Donna D.

    2010-01-01

    OBJECTIVE Diabetic nephropathy is one of the major causes of renal failure, which is accompanied by the production of reactive oxygen species (ROS). Nrf2 is the primary transcription factor that controls the antioxidant response essential for maintaining cellular redox homeostasis. Here, we report our findings demonstrating a protective role of Nrf2 against diabetic nephropathy. RESEARCH DESIGN AND METHODS We explore the protective role of Nrf2 against diabetic nephropathy using human kidney ...

  14. Concomitant macro and microvascular complications in diabetic nephropathy

    Directory of Open Access Journals (Sweden)

    Alwakeel Jamal

    2009-01-01

    Full Text Available To determine the prevalence of concomitant microvascular and macrovascular complica-tions of diabetic nephropathy we retrospectively reviewed the medical records of all 1,952 type 2 dia-betic patients followed-up at Security Forces Hospital, Riyadh, Saudi Arabia from January 1989 to December 2004. There were 626 (32.1% patients (294 (47% were males who developed diabetic nephropathy. Their mean age was 66.9 ± 11.4 years, mean duration of diabetes was 15.4 ± 7.5 years, mean age at the onset of nephropathy was 61.5 ± 12.4 years, and mean duration of nephropathy was 3.9 ± 3.8 years. Concomitant diabetic complications included cataract (38.2%, acute coronary syndrome (36.1%, peripheral neuropathy (24.9%, myocardial infarction (24.1%, background retinopathy (22.4%, stroke (17.6%, proliferative retinopathy (11.7%, foot infection (7.3%, limb amputation (3.7% and blindness (3%. Hypertension was documented in 577 (92.2% patients, dyslipidemia in 266 (42.5% and mortality from all causes in 86 (13.7%. There were 148 (23.6% patients with one complication, 81 (12.9% with two, 83 (13.3% with three, and 61 (9.7% with four or more. Dete-rioration of glomerular filtration rate was observed in 464 (74% patients and doubling of serum creatinine in 250 (39.9%, while 95 (15.2% developed end-stage renal disease (ESRD at the end of study and 79 (12.6% required dialysis. Complications were significantly more prevalent among males with greater number reaching ESRD level than females (P< 0.05. Relative risks of developing com-plications were significant after the onset of nephropathy; ACS (1.41, MI (1.49, stroke (1.48, diabetic foot (1.6, amputation (1.58 and death (1.93. We conclude that complications of diabetes are aggre-ssive and progressive including high prevalence of diabetic nephropathy. Careful monitoring and proper institution of management protocols should be implemented to identify diabetic patients at high risk for complications and mitigate progression

  15. Efek Probiotik terhadap Mortalitas, Derajat Inflamasi Intestinal, dan Kadar IgA pada Mencit Model Sepsis

    Directory of Open Access Journals (Sweden)

    Yoseph Indrayanto

    2013-03-01

    Full Text Available Sepsis results in changes to the microbiology of the gastrointestinal tract, leading to a loss of commensal flora and an overgrowth of potentially pathogenic bacteria. Administering probiotics to sepsis patients may restore balance to the microbiota and have positive effects on immune function and gastrointestinal structure and function. This study was aimed to evaluate the effect of probiotic on mortality, intestinal inflammation and immunoglobulin A (IgA serum level in mice model of sepsis. This study was an experimental research laboratory, with 36 male Balb/C mice were divided into: control, sepsis, and sepsis+probiotic groups. The study was conducted at Histology and Biomedical Laboratory, School of Medicine, Sebelas Maret University, Surakarta, in January to April 2012. Sepsis was induced in the male Balb/C mice using an intraperitoneally (i.p injection of cecal inoculum (200 mg/ kgBW, for sepsis mice model and sepsis with probiotic. Control mice were not inoculation during the study. Detection of intestinal inflammation with hematoxylin and eosin (HE staining on 8th day. Mortality perceived until 8th day. IgA serum level measurement with enzyme-linked immunosorbent assay. Two-tailed Fisher exact test for the analysis of mortality, Kruskal-Wallis test for the intestinal inflammation, one way analysis of variance for IgA serum level. Probiotic showed no significantly mortality (10% vs 40%, p=0.065 compared to sepsis group. Probiotic showed significantly decreased intestinal inflammation compared to sepsis group (p<0.001. Control mice showed levels of IgA 35.82±4.55 ng/mL. Probiotic showed significantly increased IgA levels (65.07±34.97 ng/mL vs 6.20±5.80 ng/mL, p<0.001 compared to sepsis group. In conclusions, probiotic decrease intestinal inflammation and mortality, on the other hand increase IgA level in mice model of sepsis.

  16. Nlrp3-inflammasome activation in non-myeloid-derived cells aggravates diabetic nephropathy.

    Science.gov (United States)

    Shahzad, Khurrum; Bock, Fabian; Dong, Wei; Wang, Hongjie; Kopf, Stefan; Kohli, Shrey; Al-Dabet, Moh'd Mohanad; Ranjan, Satish; Wolter, Juliane; Wacker, Christian; Biemann, Ronald; Stoyanov, Stoyan; Reymann, Klaus; Söderkvist, Peter; Groß, Olaf; Schwenger, Vedat; Pahernik, Sascha; Nawroth, Peter P; Gröne, Herman-Josef; Madhusudhan, Thati; Isermann, Berend

    2015-01-01

    Diabetic nephropathy is a growing health concern with characteristic sterile inflammation. As the underlying mechanisms of this inflammation remain poorly defined, specific therapies targeting sterile inflammation in diabetic nephropathy are lacking. Intriguingly, an association of diabetic nephropathy with inflammasome activation has recently been shown, but the pathophysiological relevance of this finding remains unknown. Within glomeruli, inflammasome activation was detected in endothelial cells and podocytes in diabetic humans and mice and in glucose-stressed glomerular endothelial cells and podocytes in vitro. Abolishing Nlrp3 or caspase-1 expression in bone marrow-derived cells fails to protect mice against diabetic nephropathy. Conversely, Nlrp3-deficient mice are protected against diabetic nephropathy despite transplantation of wild-type bone marrow. Pharmacological IL-1R antagonism prevented or even reversed diabetic nephropathy in mice. Mitochondrial reactive oxygen species (ROS) activate the Nlrp3 inflammasome in glucose or advanced glycation end product stressed podocytes. Inhibition of mitochondrial ROS prevents glomerular inflammasome activation and nephropathy in diabetic mice. Thus, mitochondrial ROS and Nlrp3-inflammasome activation in non-myeloid-derived cells aggravate diabetic nephropathy. Targeting the inflammasome may be a potential therapeutic approach to diabetic nephropathy.

  17. Heat Stress Nephropathy From Exercise-Induced Uric Acid Crystalluria: A Perspective on Mesoamerican Nephropathy.

    Science.gov (United States)

    Roncal-Jimenez, Carlos; García-Trabanino, Ramón; Barregard, Lars; Lanaspa, Miguel A; Wesseling, Catharina; Harra, Tamara; Aragón, Aurora; Grases, Felix; Jarquin, Emmanuel R; González, Marvin A; Weiss, Ilana; Glaser, Jason; Sánchez-Lozada, Laura G; Johnson, Richard J

    2016-01-01

    Mesoamerican nephropathy (MeN), an epidemic in Central America, is a chronic kidney disease of unknown cause. In this article, we argue that MeN may be a uric acid disorder. Individuals at risk for developing the disease are primarily male workers exposed to heat stress and physical exertion that predisposes to recurrent water and volume depletion, often accompanied by urinary concentration and acidification. Uric acid is generated during heat stress, in part consequent to nucleotide release from muscles. We hypothesize that working in the sugarcane fields may result in cyclic uricosuria in which uric acid concentrations exceed solubility, leading to the formation of dihydrate urate crystals and local injury. Consistent with this hypothesis, we present pilot data documenting the common presence of urate crystals in the urine of sugarcane workers from El Salvador. High end-of-workday urinary uric acid concentrations were common in a pilot study, particularly if urine pH was corrected to 7. Hyperuricemia may induce glomerular hypertension, whereas the increased urinary uric acid may directly injure renal tubules. Thus, MeN may result from exercise and heat stress associated with dehydration-induced hyperuricemia and uricosuria. Increased hydration with water and salt, urinary alkalinization, reduction in sugary beverage intake, and inhibitors of uric acid synthesis should be tested for disease prevention.

  18. The lesional skin of linear IgA bullous dermatosis expresses growth-regulated peptide (GRO)-alpha.

    Science.gov (United States)

    Tsunemi, Yuichiro; Ihn, Hironobu; Saeki, Hidehisa; Tamaki, Kunihiko

    2004-07-01

    The patient was a 62-year-old man with erythema with tense vesiculobullae and erosions on the bilateral elbows, right knee, and one buttock. A skin biopsy specimen revealed subepidermal blister formation with a predominant infiltration of neutrophils and papillary neutrophilic microabscesses. Direct immunofluorescence study showed linear deposition of IgA and weak deposition of IgG at the basement membrane zone of the lesional skin, and indirect immunofluorescence study showed linear deposition of IgA at the epidermal side of the 1M NaCl-separated normal skin. He was diagnosed with linear IgA bullous dermatosis. Immunohistochemical study revealed that the lesional and perilesional keratinocytes expressed growth-regulated peptide (GRO) -alpha, a potent chemoattractant for neutrophils. This suggests that GRO-alpha plays a role in the infiltration of neutrophils into the lesional skin and in bulla formation in linear IgA bullous dermatosis.

  19. Iga neljas kontrollitud noorsõdur tuli püssi alla narkopohmellis / Rasmus Kagge, Dagne Hanschmidt

    Index Scriptorium Estoniae

    Kagge, Rasmus, 1977-

    2008-01-01

    Ilmunud ka: Postimees : na russkom jazõke 2. okt. lk. 5. Politsei korraldatud reidil Kuperjanovi üksik-jalaväepataljoni saabunud uutel ajateenijatel tuvastati, et iga neljas noorsõdur oli tarvitanud narkootikume. Vt. samas: Ajateenijad

  20. [DIABETIC NEPHROPATHY AS A CAUSE OF CHRONIC KIDNEY DISEASE].

    Science.gov (United States)

    Kos, Ivan; Prkačin, Ingrid

    2014-12-01

    Diabetic nephropathy is the leading cause of end-stage chronic kidney disease in most developed countries. Hyperglycemia, hypertension and genetic predisposition are the main risk factors for the development of diabetic nephropathy. Elevated serum lipids, smoking habits, and the amount and origin of dietary protein also seem to play a role as risk factors. Clinical picture includes a progressive increase in albuminuria, decline in glomerular filtration, hypertension, and a high risk of cardiovascular morbidity and mortality. Screening for albuminuria should be performed yearly, starting 5 years after diagnosis in type 1 diabetes or earlier in the presence of adolescence or poor metabolic control. In patients with type 2 diabetes, screening should be performed at diagnosis and yearly thereafter. Patients with albuminuria should undergo evaluation regarding the presence of associated comorbidities, especially retinopathy and macrovascular disease. Achieving the best metabolic control (HbA1c diabetes.

  1. CLINICAL GUIDELINE FOR THE TREATMENT OF DIABETIC NEPHROPATHY

    Directory of Open Access Journals (Sweden)

    R. A. Nadeeva

    2015-01-01

    Full Text Available Due to the high prevalence of diabetes the annual increase of the number of patients with diabetic nephropathy is evidenced. The progressive course of this sequellae and a high percentage of end-stage kidney disease requires a clear approach of early diagnosis, the development of methods of prevention and early treatment from the perspective of evidence-based medicine. This review provides recommendations on glucose-lowering treatment, monitoring of blood pressure and proteinuria, hyperlipidemia. Defi ned individual targets of the correction of hyperglycaemia, depending on the level of albuminuria excretion and the severity of the patient. Indicated the possibilities of applications of certain antidiabetic drugs, depending on the level of glomerular fi ltration rate. Drugs of the fi rst and second line are marked for the selection of antihypertensive treatment. Showed the possible ways to reduce the level of albuminuria. Presented recommendations for the management of patients, depending on the stage of nephropathy.

  2. Chemical substances as risk factors of nephropathy in diabetes mellitus

    Directory of Open Access Journals (Sweden)

    Zofia Marchewka

    2009-12-01

    Full Text Available Although diabetes mellitus, a metabolic disease, does not fall into the group of diseases induced by toxic substances or environmental pollution, there is much evidence that some chemicals have considerable importance in its development. Exposure to substances with potential renal toxicity is especially dangerous for diabetics because it accelerates and intensifies diabetic nephropathy. This paper discusses the relationship between the xenobiotics and the development of diabetes mellitus and diabetic nephropathy with particular emphasis on those substances that causes the greatest damage to the kidneys. These are cadmium, iron, lead, arsenic, polychlorinated organic compounds, nitrogen compounds, and contrast agents. In addition, the mechanisms of diabetes mellitus induction or kidney damage by these xenobiotics are described.

  3. Features of endothelial dysfunction in early diabetic nephropathy

    DEFF Research Database (Denmark)

    Jensen, T; Bjerre-Knudsen, J; Feldt-Rasmussen, B

    1989-01-01

    ); group II (n = 11), incipient diabetic nephropathy (30-300 mg albumin excreted per 24 h); and group III (n = 10), clinical diabetic nephropathy (more than 300 mg albumin excreted per 24 h). Nine non-diabetic men served as controls. The rise in tPA antigen with exercise was similar in the controls......The release of tissue plasminogen activator (tPA) by vascular endothelial cells during exercise was studied in forty men with insulin-dependent diabetes. Three groups, matched for age and diabetes duration, were defined as: group I (n = 19), normal urinary albumin excretion (less than 30 mg/24 h.......01) and II (difference not significant, p = 0.06) than in group I and normal controls. These findings suggest that insulin-dependent diabetic patients with only slightly raised urinary albumin excretion have general endothelial cell dysfunction or damage. It is not yet clear whether these changes...

  4. Light-chain nephropathy in patient with renal carcinoma.

    OpenAIRE

    Enia, G.; Maringhini, S; L'Abbate, A; Zoccali, C.; Maggiore, Q

    1981-01-01

    Paraneoplastic syndromes are often associated with renal parenchymal tumours. This report describes a case of renal-cell carcinoma with kappa-chain nephropathy. The patient, a 60-year-old man, had renal tubular dysfunction, shown by low serum concentrations of urate and phosphate. Kappa-chains were found in both serum and urine, but no lambda-chains were found. Investigations showed a clear-cell carcinoma, and the patient underwent a radical nephrectomy. Two years after operation serum phosph...

  5. A Multivariate Analysis of Risk Factors for Diabetic Nephropathy

    Directory of Open Access Journals (Sweden)

    Anthony Shannon

    2007-03-01

    Full Text Available This paper uses multivariate methods on actual data from 267 patients with noninsulin- dependent (Type 2 diabetes mellitus in order to see how the various risk factors can affect the progression of diabetic nephropathy. The approach succeeds in identifying preliminary risk factors such as smoking for males, although the females had higher fasting blood glucose at diagnosis. Not surprisingly, hypertension is common among patients of both sexes and it has an association with proteinuria in female patients in the sample.

  6. Paracetamol and analgesic nephropathy: Are you kidneying me?

    OpenAIRE

    Waddington F; Naunton M; Thomas J

    2014-01-01

    Freya Waddington, Mark Naunton, Jackson Thomas Faculty of Health, University of Canberra, Canberra, ACT, Australia Introduction: Analgesic nephropathy is a disease resulting from the frequent use of combinations of analgesic medications over many years, leading to significant impairment of renal function. The observation of a large number of cases of renal failure in patients abusing analgesic mixtures containing phenacetin led to the initial recognition of the nephrotoxicity from the use of...

  7. Idiopathic membranous nephropathy complicated with malignant hypertension: a case report

    Institute of Scientific and Technical Information of China (English)

    TAO Jian-ling; LI Hang; WEN Yu-bing; LI Xue-wang

    2007-01-01

    @@ Membranous nephropathy (MN) is the most common cause of nephrotic syndrome in adults. Its insidious onset and progression often hinder timely renal biopsy and early diagnosis delaying treatment while worsening prognosis. The complication of malignant hypertension(MHT) is rarely seen in idiopathic MN. To provide a better understanding of the disease we report a case of idiopathic MN diagnosed by biopsy six years after onset.

  8. Extract of Adenanthera pavonina L. seed reduces development of diabetic nephropathy in streptozotocin-induced diabetic rats

    Directory of Open Access Journals (Sweden)

    Ramdas Pandhare

    2012-09-01

    Conclusion: These results suggested that APSAE has reduced development of diabetic nephropathy in streptozotocin-induced diabetic rats and could have beneficial effect in reducing the progression of diabetic nephropathy.

  9. A Study on Clinical and Pathologic Features in Lupus Nephritis with Mainly IgA Deposits and a Literature Review

    OpenAIRE

    2013-01-01

    Objective. To study the clinical and pathologic features of systemic lupus erythematosus (SLE) that has atypical lupus nephritis (LN) with mainly IgA deposits. Methods. We searched the SLE patients who had nephritis with mainly IgA deposits in our hospital and selected the information including clinical manifestations, laboratory tests, treatments, and prognosis. Results. From January 2009 to June 2012, 5 patients were definitely diagnosed as SLE according to both 1982 and 2009 ACR classifica...

  10. Lectin-based analysis of fucose and sialic acid expressions on human amniotic IgA during normal pregnancy.

    Science.gov (United States)

    Orczyk-Pawiłowicz, Magdalena; Augustyniak, Daria; Hirnle, Lidia; Kątnik-Prastowska, Iwona

    2013-08-01

    The sugar moiety of IgA is known to provide a link between the innate and adaptive immune systems. Terminally located glycotopes on IgA are potential ligands engaged in the interactions which may modulate the biological activities of IgA. In the present work the expressions of Maackia amurensis (MAA), Sambucus nigra (SNA), Lens culinaris (LCA), Tetragonolobus purpureus (LTA), and Ulex europaeus (UEA) reactive glycotopes on maternal plasma and amniotic IgA were evaluated in relation to the progression of a normal human pregnancy, from the 2nd trimester, throughout the 3rd trimester, perinatal period, post-date pregnancy and delivery, by lectin-IgA-ELISA, using specific biotinylated lectins. The amniotic and maternal plasma IgA concentrations and a degree of SNA and LCA reactivity of maternal plasma IgA were almost unaltered during the normal pregnancy. The amniotic IgA from the 2nd trimester was decorated by MAA-, SNA-reactive and LCA-, LTA-, and UEA-reactive glycotopes. At the turn of the 2nd and 3rd trimesters the expression of MAA-, SNA-, LTA-, and UEA-reactive glycotopes, except for LCA-reactive, increased and remained almost at unaltered levels throughout the perinatal period and delivery. However, in the post-date pregnancy the expression of LCA-, LTA-, and UEA-reactive and SNA-reactive glycotopes were significantly higher. The unique fucosylated and sialylated glycovariants of amniotic IgA associated with the progression of the normal pregnancy may illustrate a general importance of carbohydrate-lectin receptor interactions in the control and modulation of biological events to ensuring homeostasis during pregnancy, protection and well-being of fetus.

  11. Thioredoxin is involved in endothelial cell extracellular transglutaminase 2 activation mediated by celiac disease patient IgA.

    Directory of Open Access Journals (Sweden)

    Cristina Antonella Nadalutti

    Full Text Available PURPOSE: To investigate the role of thioredoxin (TRX, a novel regulator of extracellular transglutaminase 2 (TG2, in celiac patients IgA (CD IgA mediated TG2 enzymatic activation. METHODS: TG2 enzymatic activity was evaluated in endothelial cells (HUVECs under different experimental conditions by ELISA and Western blotting. Extracellular TG2 expression was studied by ELISA and immunofluorescence. TRX was analysed by Western blotting and ELISA. Serum immunoglobulins class A from healthy subjects (H IgA were used as controls. Extracellular TG2 enzymatic activity was inhibited by R281. PX12, a TRX inhibitor, was also employed in the present study. RESULTS: We have found that in HUVECs CD IgA is able to induce the activation of extracellular TG2 in a dose-dependent manner. Particularly, we noted that the extracellular modulation of TG2 activity mediated by CD IgA occurred only under reducing conditions, also needed to maintain antibody binding. Furthermore, CD IgA-treated HUVECs were characterized by a slightly augmented TG2 surface expression which was independent from extracellular TG2 activation. We also observed that HUVECs cultured in the presence of CD IgA evinced decreased TRX surface expression, coupled with increased secretion of the protein into the culture medium. Intriguingly, inhibition of TRX after CD IgA treatment was able to overcome most of the CD IgA-mediated effects including the TG2 extracellular transamidase activity. CONCLUSIONS: Altogether our findings suggest that in endothelial cells CD IgA mediate the constitutive activation of extracellular TG2 by a mechanism involving the redox sensor protein TRX.

  12. Generalized Shape and Gauge Decoupling Load Distribution Optimization Based on IGA for Tandem Cold Mill

    Institute of Scientific and Technical Information of China (English)

    PENG Peng; YANG Quan

    2009-01-01

    Load distribution is the foundation of shape control and gauge control, in which it is necessary to take into account the shape control ability of TCM (tandem cold mill) for strip shape and gauge quality. First, the objective function of generalized shape and gauge decoupling load distribution optimization was established, which considered the rolling force characteristics of the first and last stands in TCM, the relative power, and the TCM shape control ability. Then, IGA (immune genetic algorithm) was used to accomplish this multi-objective load distribution optimization for TCM. After simulation and comparison with the practical load distribution strategy in one tandem cold mill, general-ized shape and gauge decoupling load distribution optimization on the basis of IGA approved good ability of optimizing shape control and gauge control simultaneously.

  13. Ocular Involvement and Blindness Secondary to Linear IgA Dermatosis

    Directory of Open Access Journals (Sweden)

    Cinthya Ramos-Castellón

    2010-01-01

    Full Text Available A 43-year-old man with linear immunoglobulin A (IgA dermatosis associated with gluten intolerance presented with progressive vision loss, pain and photosensitivity in both eyes. His visual acuity was light perception (LP in both eyes. A physical examination revealed bullous, papular lesions with erythematous borders in periocular tissues, limbs, and thorax. Slit-lamp examination showed conjunctival hyperemia, fibrosis, corneal opacification, and vascularization with epithelial defects. Immunofluorescent skin and corneal surface biopsy studies showed linear IgA deposits. The patient was treated with keratolimbal allogenic transplantation and cryopreserved amniotic membrane in the right eye. Regardless of the treatment he persisted with torpid evolution developing retinal and choroidal detachments. After these events he was started on intravenous immune globulin (IVIG and showed very slight improvement in ocular surface. These types of blistering diseases are rare in the eye. Even when adequate local treatment is given, systemic treatment is mandatory and ocular prognosis can be unsatisfactory.

  14. Inspection of circumferential IGA/SCC in Bruce Unit 4 SGs

    Energy Technology Data Exchange (ETDEWEB)

    Fluit, S.; Myderwyk, H.; King, P. [Babcock and Wilcox Canada Limited, Cambridge, Ontario (Canada); Durance, D.; Sedman, K. [Bruce Power, Tiverton, Ontario (Canada)

    2008-07-01

    Circumferential IGA/SCC has been detected in the roll-transition zone of tubes in Bruce A SGs for over a decade, but recently became more pronounced in Unit 4 BO4 in 2006 and the spring of 2007. A number of ET probes have been developed to detect and characterize this degradation, including C-3/8, +Point and X-Probe. Comparisons of the inspection results from these probes and metallurgical results from removed tubes have provided insight into the strengths and limitations of each probe and how the probes can complement each other. This paper will discuss the evolution of the ET inspection approach for circumferential IGA/SCC and how the results have been used in recent FFS assessments in the Bruce Unit 4 SGs. (author)

  15. Oxidative Stress in Diabetic Nephropathy with Early Chronic Kidney Disease

    Directory of Open Access Journals (Sweden)

    Alejandra Guillermina Miranda-Díaz

    2016-01-01

    Full Text Available The increase in the prevalence of diabetes mellitus (DM and the secondary kidney damage produces diabetic nephropathy (DN. Early nephropathy is defined as the presence of microalbuminuria (30–300 mg/day, including normal glomerular filtration rate (GFR or a mildly decreased GFR (60–89 mL/min/1.73 m2, with or without overt nephropathy. The earliest change caused by DN is hyperfiltration with proteinuria. The acceptable excretion rate of albumin in urine is 300 mg/day. Chronic kidney disease (CKD is characterized by abnormalities in renal function that persist for >3 months with health implications. Alterations in the redox state in DN are caused by the persistent state of hyperglycemia and the increase in advanced glycation end products (AGEs with ability to affect the renin-angiotensin system and the transforming growth factor-beta (TGF-β, producing chronic inflammation and glomerular and tubular hypertrophy and favoring the appearance of oxidative stress. In DN imbalance between prooxidant/antioxidant processes exists with an increase in reactive oxygen species (ROS. The overproduction of ROS diminishes expression of the antioxidant enzymes (manganese superoxide dismutase, glutathione peroxidase, and catalase. The early detection of CKD secondary to DN and the timely identification of patients would permit decreasing its impact on health.

  16. Urinary exosomal microRNAs in incipient diabetic nephropathy.

    Science.gov (United States)

    Barutta, Federica; Tricarico, Marinella; Corbelli, Alessandro; Annaratone, Laura; Pinach, Silvia; Grimaldi, Serena; Bruno, Graziella; Cimino, Daniela; Taverna, Daniela; Deregibus, Maria Chiara; Rastaldi, Maria Pia; Perin, Paolo Cavallo; Gruden, Gabriella

    2013-01-01

    MicroRNAs (miRNAs), a class of small non-protein-encoding RNAs, regulate gene expression via suppression of target mRNAs. MiRNAs are present in body fluids in a remarkable stable form as packaged in microvesicles of endocytic origin, named exosomes. In the present study, we have assessed miRNA expression in urinary exosomes from type 1 diabetic patients with and without incipient diabetic nephropathy. Results showed that miR-130a and miR-145 were enriched, while miR-155 and miR-424 reduced in urinary exosomes from patients with microalbuminuria. Similarly, in an animal model of early experimental diabetic nephropathy, urinary exosomal miR-145 levels were increased and this was paralleled by miR-145 overexpression within the glomeruli. Exposure of cultured mesangial cells to high glucose increased miR-145 content in both mesangial cells and mesangial cells-derived exosomes, providing a potential mechanism for diabetes-induced miR-145 overexpression. In conclusion, urinary exosomal miRNA content is altered in type 1 diabetic patients with incipient diabetic nephropathy and miR-145 may represent a novel candidate biomarker/player in the complication.

  17. Urinary exosomal microRNAs in incipient diabetic nephropathy.

    Directory of Open Access Journals (Sweden)

    Federica Barutta

    Full Text Available MicroRNAs (miRNAs, a class of small non-protein-encoding RNAs, regulate gene expression via suppression of target mRNAs. MiRNAs are present in body fluids in a remarkable stable form as packaged in microvesicles of endocytic origin, named exosomes. In the present study, we have assessed miRNA expression in urinary exosomes from type 1 diabetic patients with and without incipient diabetic nephropathy. Results showed that miR-130a and miR-145 were enriched, while miR-155 and miR-424 reduced in urinary exosomes from patients with microalbuminuria. Similarly, in an animal model of early experimental diabetic nephropathy, urinary exosomal miR-145 levels were increased and this was paralleled by miR-145 overexpression within the glomeruli. Exposure of cultured mesangial cells to high glucose increased miR-145 content in both mesangial cells and mesangial cells-derived exosomes, providing a potential mechanism for diabetes-induced miR-145 overexpression. In conclusion, urinary exosomal miRNA content is altered in type 1 diabetic patients with incipient diabetic nephropathy and miR-145 may represent a novel candidate biomarker/player in the complication.

  18. Spontaneous remission of nephrotic syndrome in idiopathic membranous nephropathy.

    Science.gov (United States)

    Polanco, Natalia; Gutiérrez, Elena; Covarsí, Adelardo; Ariza, Francisco; Carreño, Agustín; Vigil, Ana; Baltar, José; Fernández-Fresnedo, Gema; Martín, Carmen; Pons, Salvador; Lorenzo, Dolores; Bernis, Carmen; Arrizabalaga, Pilar; Fernández-Juárez, Gema; Barrio, Vicente; Sierra, Milagros; Castellanos, Ines; Espinosa, Mario; Rivera, Francisco; Oliet, Aniana; Fernández-Vega, Francisco; Praga, Manuel

    2010-04-01

    Spontaneous remission is a well known characteristic of idiopathic membranous nephropathy, but contemporary studies describing predictors of remission and long-term outcomes are lacking. We conducted a retrospective, multicenter cohort study of 328 patients with nephrotic syndrome resulting from idiopathic membranous nephropathy that initially received conservative therapy. Spontaneous remission occurred in 104 (32%) patients: proteinuria progressively declined after diagnosis until remission of disease at 14.7 +/- 11.4 months. Although spontaneous remission was more frequent with lower levels of baseline proteinuria, it also frequently occurred in patients with massive proteinuria: 26% among those with baseline proteinuria 8 to 12 g/24 h and 22% among those with proteinuria >12 g/24 h. Baseline serum creatinine and proteinuria, treatment with angiotensin-converting enzyme inhibitors or angiotensin receptor antagonists, and a >50% decline of proteinuria from baseline during the first year of follow-up were significant independent predictors for spontaneous remission. Only six patients (5.7%) experienced a relapse of nephrotic syndrome. The incidence of death and ESRD were significantly lower among patients with spontaneous remission. In conclusion, spontaneous remission is common among patients with nephrotic syndrome resulting from membranous nephropathy and carries a favorable long-term outcome with a low incidence of relapse. A decrease in proteinuria >50% from baseline during the first year predicts spontaneous remission.

  19. Oxidative Stress in Diabetic Nephropathy with Early Chronic Kidney Disease

    Science.gov (United States)

    Andrade-Sierra, Jorge

    2016-01-01

    The increase in the prevalence of diabetes mellitus (DM) and the secondary kidney damage produces diabetic nephropathy (DN). Early nephropathy is defined as the presence of microalbuminuria (30–300 mg/day), including normal glomerular filtration rate (GFR) or a mildly decreased GFR (60–89 mL/min/1.73 m2), with or without overt nephropathy. The earliest change caused by DN is hyperfiltration with proteinuria. The acceptable excretion rate of albumin in urine is 300 mg/day. Chronic kidney disease (CKD) is characterized by abnormalities in renal function that persist for >3 months with health implications. Alterations in the redox state in DN are caused by the persistent state of hyperglycemia and the increase in advanced glycation end products (AGEs) with ability to affect the renin-angiotensin system and the transforming growth factor-beta (TGF-β), producing chronic inflammation and glomerular and tubular hypertrophy and favoring the appearance of oxidative stress. In DN imbalance between prooxidant/antioxidant processes exists with an increase in reactive oxygen species (ROS). The overproduction of ROS diminishes expression of the antioxidant enzymes (manganese superoxide dismutase, glutathione peroxidase, and catalase). The early detection of CKD secondary to DN and the timely identification of patients would permit decreasing its impact on health. PMID:27525285

  20. A case of membranous nephropathy associated with chronic sinusitis.

    Science.gov (United States)

    Kanazawa, Ippei; Yano, Shozo; Takase, Hirofumi; Yamane, Yuko; Yamaguchi, Toru; Sugimoto, Toshitsugu

    2009-01-01

    Here we report a case of a 71-year-old Japanese woman with membranous nephropathy complicated with chronic sinusitis. The patient visited our hospital for treatment of edema and proteinuria, and was diagnosed as nephrotic syndrome with serum total protein 7.5 g/dL, albumin 2.2 g/dL and urine protein 3.7 g/day. Renal biopsy revealed membranous glomerulonephritis with tubulointerstitial nephritis. She had suffered from chronic sinusitis for several years. Laboratory tests showed that immunoglobulin G (IgG) and soluble interleukin-2 receptor (sIL-2R) were markedly increased (3,233 mg/dL and 5,110 U/mL, respectively) and Gallium scintigraphy showed abnormal accumulation to the paranasal sinus. After operation for chronic sinusitis, the levels of IgG and sIL-2R were decreased, and nephrotic syndrome was improved without any other specific treatments. It is known that various diseases, including membranous nephropathy and sinusitis, are caused by T-cell dysfunction. Thus, we suspect the pathogenetic link between membranous nephropathy and chronic sinusitis in this case, most probably through T-cell dysfunction.