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Sample records for minimal-change nephrotic syndrome

  1. Sagittal sinus thrombosis in adult minimal change nephrotic syndrome.

    Science.gov (United States)

    Urch, C; Pusey, C D

    1996-02-01

    Nephrotic syndrome causes a hypercoagulable state, leading to both venous and arterial thrombosis. The mechanisms are as yet unclear, but numerous alterations in coagulant and anti-coagulant factors have been reported [Llach et al. 1985, Harris and Ismail 1994]. The most common clinical features in adults are renal vein thrombosis, femoral vein thrombosis and pulmonary embolism, although thrombosis in numerous other arterial and venous sites has been described [Cameron 1984, Llach et al. 1985]. Intracranial thrombosis is rare, although in adult nephrotic syndrome arterial thrombosis is well recognized [Fuh et al. 1991]. We report a patient with minimal change nephrotic syndrome who developed venous sinus thrombosis detected by magnetic resonance (MR) scanning.

  2. Cerebral infarction in patient with minimal change nephrotic syndrome.

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    Babu, A; Boddana, P; Robson, S; Ludeman, L

    2013-01-01

    We report a case of 68-year-old Caucasian man who presented with cerebral infarcts secondary to arterial thrombosis associated with nephrotic syndrome. His initial presentation included edema of legs, left hemiparesis, and right-sided cerebellar signs. Investigations with computed tomography and magnetic resonance imaging of brain showed multiple cerebral infarcts in middle cerebral and posterior cerebral artery territory. Blood and urine investigations also showed impaired renal function, hypercholesterolemia, hypoalbuminaemia, and nephrotic range proteinuria. Renal biopsy showed minimal change disease. Cerebral infarcts were treated with antiplatelet agents and nephrotic syndrome was treated with high dose steroids. Patient responded well to the treatment and is all well till date.

  3. [Cerebral venous thrombosis in minimal change nephrotic syndrome].

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    Hirata, M; Kuroda, M; Koni, I

    1999-06-01

    A 46-year old man presented with an eight-day history of edema and was found to be nephrotic, with a plasma albumin level of 1.1 g/dl and urine protein excretion of 13.3 g/24 hrs. The level of plasma creatinine was normal at 1.0 mg/dl. A finding of renal biopsy was consistent with minimal change glomerulopathy. On the 6th hospital day, he suddenly developed a severe headache and was noted to have bilateral papilledema. Lumbar puncture revealed an opening pressure of 250 mm of water. Magnetic resonance venography showed an irregular flow in the superior sagittal sinus and right transverse sinus, a finding consistent with thrombus. The diagnosis of cerebral venous thrombosis was made, and the patient was given both Warfarin 2 mg/day and prednisolone 60 mg/day. A complete recovery from nephrotic syndrome was achieved within eight weeks. Nephrotic syndrome causes a hypercoagulable state, leading to both venous and arterial thrombosis. The most common clinical features are renal vein thrombosis, femoral vein thrombosis, and pulmonary embolism, however, cerebral venous sinus thrombosis is rare in patients with nephrotic syndrome. It is important to be aware of this complication, since prompt treatment with anticoagulation and control of nephrotic syndrome can lead to a successful outcome.

  4. Concurrence of Bartter syndrome and minimal change nephrotic syndrome

    Institute of Scientific and Technical Information of China (English)

    SHEN Hui-jun; DAI Yu-wen; MAO Jian-hua; LIU Ai-min

    2009-01-01

    @@ Nephrotic syndrome(NS)is a common disease in children with a group of symptoms including heavy proteinuria(≥50 mg/kg per 24 hours),hypoalbuminaemia,hypercholesterolaemia and edema.Bartter syndrome(BS)is a clinically and genetically heterogenous kidney disease characterized by hypokalemia,hypochloremic metabolic alkalosis,obvious increase of rennin,angiotesin II,and normal blood pressure.

  5. [Kimura's disease: an unrecognized cause of adult-onset nephrotic syndrome with minimal change disease].

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    Shehwaro, N; Langlois, A-L; Gueutin, V; Debchi, L; Charlotte, F; Rouvier, P; Rottembourg, J; Izzedine, H

    2014-02-01

    Kimura's disease (KD) is an angiolymphoid proliferative disorder of soft tissue with eosinophilia, with a predilection for head and neck regions in young Oriental men. Kidney disease is thought to be rare in KD. About a case of adult-onset nephrotic syndrome with minimal change disease, we comment Kimura's disease and its associated kidney damage. Kimura disease should be suspected and included in the diagnosis of adult-onset nephrotic syndrome with minimal change disease.

  6. Nephrotic syndrome of minimal change disease following exposure to mercury-containing skin-lightening cream.

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    Zhang, Lin; Liu, Fuyou; Peng, Youming; Sun, Lin; Chen, Chunguo

    2014-01-01

    A 28-year-old female suffered from nephrotic syndrome after a long-term use of mercury-containing, skin-lightening cream. The blood and urinary mercury content of this patient increased with use. Renal biopsy showed minimal change disease. Her symptoms were relieved 6 months after discontinuing use of the cream and receiving sodium dimercaptosulfonate and glucocorticosteroid treatments. Proteinuria disappeared, and blood and urinary mercury levels returned to normal. Previous reports of nephrotic syndrome caused by mercury-containing, skin-lightening creams have mostly been identified as be.ing due to membranous nephropathy. Minimal change disease has been reported in a few case reports published in the English language. Here we report a case of nephrotic syndrome with minimal change disease following exposure to a mercury-containing, skin-lightening cream. We also reviewed relevant published reports to summarize clinical features and treatments and to explore the possible mechanisms involved.

  7. Glomerular involution in children with frequently relapsing minimal change nephrotic syndrome: an unrecognized form of glomerulosclerosis?

    NARCIS (Netherlands)

    Dijkman, H.B.P.M.; Wetzels, J.F.M.; Gemmink, J.H.; Baede, J.; Levtchenko, E.N.; Steenbergen, E.

    2007-01-01

    Global glomerulosclerosis can be divided in the vascular (obsolescent) type and the glomerulopathic (solidified) type. In biopsies from children with recurrent nephrotic syndrome owing to minimal change nephropathy (MCN), we noticed small, globally sclerosed glomeruli that appeared to be distinct fr

  8. A Case of Nephrotic Syndrome With Minimal-Change Disease and Waldenstrom's Macroglobulinemia.

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    Grabe, Darren W; Li, Bo; Haqqie, Syed S

    2013-12-01

    Kidney disease is a rare complication of Waldenstrom's macroglobulinemia. We report a case of nephrotic syndrome and minimal change disease in a patient with biopsy proven Waldenstrom's macroglobulinemia. The patient presented with over 12 grams of proteinuria and was successfully treated with oral prednisone over the course of 4 weeks. Repeat serum protein electrophoresis as well as serum immunoelectrophoresis revealed no paraproteins, urine analysis was negative for protein or blood by dipstick and spot urine protein was 9 mg/dL with creatinine of 101 mg/dL at time of last office visit. This case illustrates the successful treatment with corticosteroids alone with prolonged complete remission.

  9. Minimal Change Nephrotic Syndrome Which Was Most Likely Caused by Chronic Sinusitis.

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    Iwatani, Hirotsugu; Mori, Daisuke; Yamamoto, Satoko; Nakano, Chikako; Yamamoto, Ryohei; Masumura, Chisako; Shikina, Takashi; Imai, Takao; Inohara, Hidenori; Rakugi, Hiromi; Isaka, Yoshitaka

    2015-01-01

    A 33-year-old Japanese man was admitted with severe edema, and a renal biopsy confirmed minimal change nephrotic syndrome (MCNS). CT revealed his severe chronic sinusitis, and he first received antimicrobial therapy, which resulted in decreased proteinuria. The surgical operation for sinusitis resulted in the complete disappearance of proteinuria without corticosteroid or immunosuppressant therapy within one week. MCNS may be triggered by infection, but there are no previously reported cases of MCNS that is completely remitted by infection control alone. Therefore, we herein report the first case of MCNS that attained complete remission following therapy for chronic sinusitis alone, which suggests a strong etiology of chronic sinusitis for MCNS.

  10. Minimal change nephrotic syndrome after stem cell transplantation: a case report and literature review

    Directory of Open Access Journals (Sweden)

    Silva Sandra

    2007-10-01

    Full Text Available Abstract Graft-versus-host disease is one of the most frequent complications occurring after haematopoietic stem cell transplantation. Recently, renal involvement has been described as a manifestation of chronic graft-versus-host disease. Immunosuppression seems to play a major role: clinical disease is triggered by its tapering and resolution is achieved with the resumption of the immunosuppressive therapy. Prognosis is apparently favourable, but long term follow up data are lacking. We report a case of a 53-year-old man who developed nephrotic syndrome 142 days after allogeneic stem cell transplantation for acute myeloid leukaemia. Onset of nephrotic syndrome occurred after reduction of immunosuppressants and was accompanied by manifestations of chronic graft-versus-host disease. Histological examination of the kidney was consistent with Minimal Change Disease. After treatment with prednisolone and mycophenolate mofetil he had complete remission of proteinuria and improvement of graft-versus-host disease. Eighteen months after transplantation the patient keeps haematological remission and normal renal function, without proteinuria. Since patients with chronic graft-versus-host disease might be considered at risk for development of nephrotic syndrome, careful monitoring of renal parameters, namely proteinuria, is advisable.

  11. Minimal change nephrotic syndrome after stem cell transplantation: a case report and literature review.

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    Silva, Sandra; Maximino, José; Henrique, Rui; Paiva, Ana; Baldaia, Jorge; Campilho, Fernando; Pimentel, Pedro; Loureiro, Alfredo

    2007-10-30

    Graft-versus-host disease is one of the most frequent complications occurring after haematopoietic stem cell transplantation. Recently, renal involvement has been described as a manifestation of chronic graft-versus-host disease. Immunosuppression seems to play a major role: clinical disease is triggered by its tapering and resolution is achieved with the resumption of the immunosuppressive therapy. Prognosis is apparently favourable, but long term follow up data are lacking.We report a case of a 53-year-old man who developed nephrotic syndrome 142 days after allogeneic stem cell transplantation for acute myeloid leukaemia. Onset of nephrotic syndrome occurred after reduction of immunosuppressants and was accompanied by manifestations of chronic graft-versus-host disease. Histological examination of the kidney was consistent with Minimal Change Disease. After treatment with prednisolone and mycophenolate mofetil he had complete remission of proteinuria and improvement of graft-versus-host disease. Eighteen months after transplantation the patient keeps haematological remission and normal renal function, without proteinuria.Since patients with chronic graft-versus-host disease might be considered at risk for development of nephrotic syndrome, careful monitoring of renal parameters, namely proteinuria, is advisable.

  12. [A case of AKI-caused minimal change nephrotic syndrome with concomitant pleuritis].

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    Watanabe, Renya; Abe, Yasuhiro; Sasaki, Masaru; Hamauchi, Aki; Yasunaga, Tomoe; Kurata, Satoshi; Yasuno, Tetsuhiko; Ito, Kenji; Sasatomi, Yoshie; Hisano, Satoshi; Nakashima, Hitoshi

    2016-01-01

    A twenty-year-old man complaining of chest pain was diagnosed as nephrotic syndrome complicated with pleural effusion and ascites. Despite treatment with antibiotics, his fever and high inflammatory reaction persisted. After hospitalization, his urine volume decreased and renal function had deteriorated. As he was suffering from dyspnea, hemodialysis was performed together with chest drainage. His pleural effusion was exudative, and IVIG treatment was added to the antibiotic treatment. He was diagnosed as suspected developed minimal change nephrotic syndrome (MCNS) and administered prednisolone intravenously. His renal function ameliorated as a result of this treatment, enabling him to withdraw from hemodialysis. Inflammatory reaction gradually decreased and his general condition improved. The result of a renal biopsy examination carried out after the hemodialysis treatment confirmed MCNS, which suggested that MCNS had induced acute kidney injury (AKI) atypically in this case. Generally AKI is not induced by MCNS in youth, but it may occur under severe inflammatory conditions. Physicians should be aware that MCNS in young patients may lead to the development of AKI requiring hemodialysis treatment.

  13. Minimal change nephrotic syndrome associated with invasive thymoma: a case report with literature review.

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    Long, Quan; Wu, Ping; Jiang, Gengru; Zhu, Chun

    2014-04-01

    The present report describes a case of nephrotic syndrome (NS) with invasive thymoma. A male patient was hospitalized for severe edema with reduced urine output. He had a history of thymectomy and radiotherapy because of invasive thymoma 4 years before the development of NS. Renal biopsy displayed minimal change disease (MCD). Although imaging study showed probably recurrent sign of invasive thymoma, the patient still received steroid monotherapy for ~ 9 months and he got partial remission of NS at the 8th week. Therefore, we suggest that MCD should be taken into account as a pathological lesion type in old NS patients with thymoma. In spite of longer remission time, steroid monotherapy and combination therapy with immunosuppressant are effective for thymoma-associated MCD.

  14. Influence of low birth weight on minimal change nephrotic syndrome in children, including a meta-analysis

    NARCIS (Netherlands)

    N. Teeninga (Nynke); M.F. Schreuder (Michiel); A. Bökenkamp (Arend); H.A.D.V.D. Waal; J.A.E.V. Wijk

    2008-01-01

    textabstractBackground. Low birth weight (LBW) has been shown to lead to a low nephron endowment with subsequent glomerular hyperfiltration. Additional renal disease can therefore be expected to have a more severe course. Minimal change nephrotic syndrome (MCNS) is a common chronic illness in childh

  15. Long-term outcome after cyclophosphamide treatment in children with steroid-dependent and frequently relapsing minimal change nephrotic syndrome.

    NARCIS (Netherlands)

    Kyrieleis, H.A.; Levtchenko, E.N.; Wetzels, J.F.M.

    2007-01-01

    BACKGROUND: Seventy percent of children with minimal change nephrotic syndrome (MCNS) have a steroid-dependent or frequent relapsing course of the disease, and most are treated with cyclophosphamide. We describe the clinical course of children with biopsy-proven MCNS treated with cyclophosphamide fo

  16. Minimal change nephrotic syndrome in an 82 year old patient following a tetanus-diphteria-poliomyelitis-vaccination

    Directory of Open Access Journals (Sweden)

    Clajus Christian

    2009-08-01

    Full Text Available Abstract Background The most common cause of idiopathic nephrotic syndrome in children and younger adults is the minimal change nephrotic syndrome (MCNS. In the elderly MCNS is relatively uncommon. Over the last decade some reports suggest a rare but possible association with the administration of various vaccines. Case presentation A 82-year old Caucasian female presented with pronounced nephrotic syndrome (proteinuria of 7.1 g/d, hypoproteinemia of 47 g/l. About six weeks prior to admission, she had received a combination vaccination for tetanus, diphtheria and poliomyelitis as a booster-vaccination from her general practitioner. The renal biopsy revealed typical minimal change lesions. She responded well to the initiated steroid treatment. As through physical examination as well as extensive laboratory and imaging studies did neither find any evidence for malignancies nor infections we suggest that the minimal change nephrotic syndrome in this patient might be related to the activation of the immune system triggered by the vaccination. Conclusion Our case as well as previous anecdotal reports suggests that vaccination and the resulting stimulations of the immune system might cause MCNS and other severe immune-reactions. Increased awareness in that regard might help to expand the database of those cases.

  17. Nephrotic Syndrome without Hematuria due to Infection-Related Glomerulonephritis Mimicking Minimal-Change Disease in a Child.

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    Iwafuchi, Yoichi; Morioka, Tetsuo; Morita, Takashi; Watanabe, Kanako; Oyama, Yuko; Narita, Ichiei

    2016-01-01

    Nephrotic syndrome without hematuria due to infection-related glomerulonephritis is uncommon. The present report describes a case of nephrotic syndrome due to infection-related glomerulonephritis without hematuria and hypertension in an older child. A 14-year-old boy was referred to our hospital because of a 5-day history of fever, nausea, weight gain and recent leg edema without hypertension. Laboratory data showed nephrotic-range proteinuria, hypoalbuminemia, mild hypocomplementemia and acute renal injury without hematuria. Although, due to the clinical presentation, minimal-change nephrotic syndrome was mostly suspected, a renal biopsy showed endocapillary hypercellularity mainly of mononuclear cells with segmental mesangiolytic changes. Fine granular IgG and C3 deposits were noted by an immunofluorescent study; many relatively small electron-dense deposits were observed electron-microscopically. These findings led to the diagnosis of nephrotic syndrome due to infection-related endocapillary proliferative glomerulonephritis, although the causative organism of his nephritis was not detected. He recovered with rest and dietary cure. When we examine an acute nephrotic child, infection-related glomerulonephritis should be considered as the differential diagnosis to avoid unnecessary use of corticosteroids.

  18. The treatment of relapse in adults with minimal change nephrotic syndrome: Myths and facts

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    Mazin M.T. Shigidi

    2011-01-01

    Full Text Available Few controlled trials have studied the treatment of relapse in adults with minimal change disease. Repeated courses of steroids, cyclophosphamide, cyclosporine and even mycophe-nolate mofetil (MMF, all seem to play a role. The aim of this study was to review and critically analyze the literature regarding the use of immunosuppressive therapy for the treatment of relapse in adults with minimal change nephrotic syndrome (MCNS. An intensive search was done for pub-lished trials in the general medical database. Retrieved studies were further sorted according to spe-cific inclusion and exclusion criteria. Selected trials were critically analyzed and evaluated using the Oxford Centre for Evidence-based Medicine Levels of Evidence, 2009 rating. Six studies were selected and systematically reviewed. One randomized controlled trial compared the use of cyclo-phosphamide versus cyclosporine (11 adults and showed that both drugs are effective in the treatment of frequent relapses [level 1b evidence (grade B]. Three trials (total of 20 patients tested the use of cyclosporine therapy and showed that cyclosporine, though effective in the treatment of relapse, is associated with an extremely high incidence of subsequent relapses following drug with-drawal. A long-term follow-up cross-sectional study of 95 patients, with 69 relapsers, supported the use of steroids in the treatment of occasional relapses [level 2c evidence (grade C]. One case series described the benefits of MMF [level 4 evidence (grade C]. Most of the the clinical trials studied were heterogeneous, underpowered by small adult populations, open-labelled, non-randomized, with poor statistical analysis, validity and utility. We conclude that there is poor evidence that successful treatment of the first relapse of adult MCNS can be achieved with a second course of steroids or cyclosporine. Also, there is weak evidence that frequent relapses can be treated using cyclophosphamide, cyclosporine or MMF

  19. Epithelioid pleural mesothelioma concurrently associated with miliary pulmonary metastases and minimal change nephrotic syndrome - A hitherto undescribed case.

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    Tsukamoto, Yoshitane; Otsuki, Taiichiro; Hao, Hiroyuki; Kuribayashi, Kozo; Nakano, Takashi; Kida, Aritoshi; Nakanishi, Takeshi; Funatsu, Eriko; Noguchi, Chihiro; Yoshihara, Shunya; Kaku, Koji; Hirota, Seiichi

    2015-12-01

    Malignant pleural mesothelioma (MPM) is the aggressive disease typically spreading along the pleural surface and encasing the lung, leading to respiratory failure or cachexia. Rare cases with atypical clinical manifestation or presentation have been reported in MPM. We experienced a unique case of MPM concurrently associated with miliary pulmonary metastases and nephrotic syndrome. A 73-year-old Japanese man with past history of asbestos exposure was referred to our hospital for the investigation of the left pleural effusion. Chest computed tomography showed thickening of the left parietal pleura. Biopsy specimen of the pleura showed proliferating epithelioid tumor cells, leading to the pathological diagnosis of epithelioid MPM with the aid of immunohistochemistry. After the diagnosis of MPM, chemotherapy was performed without effect. Soon after the clinical diagnosis of progressive disease with skull metastasis, edema and weight gain appeared. Laboratory data met the criteria of nephrotic syndrome, and renal biopsy with electron microscopic examination revealed the minimal change disease. Steroid therapy was started but showed no effect. Around the same time of onset of nephrotic syndrome, multiple miliary lung nodules appeared on chest CT. Transbronchial biopsy specimen of the nodules showed the metastatic MPM in the lung. The patient died because of the worsening of the general condition. To our knowledge, this is the first case of MPM concurrently associated with multiple miliary pulmonary metastases and nephrotic syndrome.

  20. A case of minimal change nephrotic syndrome with acute renal failure complicating Hashimotoâs disease.

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    Iwazu, Y; Nemoto, J; Okuda, K; Nakazawa, E; Hashimoto, A; Fujio, Y; Sakamoto, M; Ando, Y; Muto, S; Kusano, E

    2008-01-01

    A 63-year-old man was admitted to our hospital for evaluation of generalized edema. Coexistence of severe hypothyroidism and nephrotic syndrome was detected by laboratory examination. High titer of both antimicrosomal antibody and antithyroid peroxidase antibody indicated Hashimotoâs disease. Renal biopsy showed minimal change glomerular abnormality, but no findings of membranous nephropathy. A series of medical treatments, including steroid therapy, thyroid hormone and human albumin replacement therapy, were administered. However, acute renal failure accompanied by hypotension, was not sufficiently prevented. After 9 sessions of plasmapheresis therapy, the severe proteinuria and low serum albumin levels were improved. Even after resting hypotension was normalized, neither renal function nor thyroid function were fully recovered. After discharge, renal function gradually returned to normal, and the blood pressure developed into a hypertensive state concomitant with the normalization of thyroid function. This report is a rare case of autoimmune thyroid disease complicated with minimal change nephrotic syndrome. In most cases of nephritic syndrome, acute renal failure (ARF) has been reported to coexist with hypertension. Although pseudohypothyroidism is well-known in nephrotic pathophysiology, complications of actual hypothyroidism are uncommon. It is suggested that the development of hypotension and ARF could be enhanced not only by hypoproteinemia, but also by severe hypothyroidism.

  1. Nephrotic syndrome due to minimal change disease secondary to spider bite: clinico-pathological case of a non-described complication of latrodectism.

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    Méndez, Gonzalo P; Enos, Daniel; Moreira, José Luis; Alvaredo, Fátima; Oddó, David

    2017-04-01

    The patient was an 18-year-old man who developed nephrotic syndrome after a 'wheat spider' bite (Latrodectus mactans). Due to this atypical manifestation of latrodectism, a renal biopsy was performed showing minimal change disease. The nephrotic syndrome subsided after 1 week without specific treatment. This self-limited evolution suggests that the mechanism of podocyte damage was temporary and potentially mediated by a secondary mechanism of hypersensitivity or direct effect of the α-latrotoxin. The patient did not show signs of relapse in subsequent checkup. This is the first reported case of nephrotic syndrome due to a minimal change lesion secondary to latrodectism.

  2. Nephrotic syndrome due to minimal change disease secondary to spider bite: clinico-pathological case of a non-described complication of latrodectism

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    Enos, Daniel; Moreira, José Luis; Alvaredo, Fátima; Oddó, David

    2017-01-01

    Abstract The patient was an 18-year-old man who developed nephrotic syndrome after a ‘wheat spider’ bite (Latrodectus mactans). Due to this atypical manifestation of latrodectism, a renal biopsy was performed showing minimal change disease. The nephrotic syndrome subsided after 1 week without specific treatment. This self-limited evolution suggests that the mechanism of podocyte damage was temporary and potentially mediated by a secondary mechanism of hypersensitivity or direct effect of the α-latrotoxin. The patient did not show signs of relapse in subsequent checkup. This is the first reported case of nephrotic syndrome due to a minimal change lesion secondary to latrodectism. PMID:28396739

  3. [Case of goodpasture syndrome associated with minimal change nephrotic syndrome (MCNS) in a patient with rheumatoid arthritis (RA)].

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    Takeda, Yoko; Abe, Aya; Toki, Takeshi; Komaba, Hirotaka; Abe, Takaya; Umezu, Michio; Joh, Kensuke; Fukagawa, Masafumi

    2009-01-01

    In early June 2004, a 50-year-old female was admitted to the hospital for slight fever, general fatigue, hemoptysis, dyspnea, and renal dysfunction (serum creatinine[Cr] : 6.05 mg/dL). She had been treated with prednisolone (PSL : 10-20 mg/day) for RA. She was diagnosed with Goodpasture syndrome based on a high titer of anti-glomerular basement membrane antibody (87 EU), and pulmonary hemorrhage. The renal and pulmonary impairments were markedly improved by the pulse therapy, plasma exchange and temporary hemodialysis. However, the Cr level remained at 2.0 mg/dL, indicating nephrotic syndrome. Light microscopy with Periodic acid-Shiff(PAS) staining demonstrated global sclerosis in three of ten glomeruli. Five glomeruli showed the formation of cellular, and fibrocellular crescents, and the formation of fibrous crescents. Tubular damage and interstitial fibrosis were severe. Immunofluorescence microscopy disclosed major depositions of IgG in a linear pattern along the glomerular basement membrane(GBM). Electron microscopy revealed foot process effacement (>50%)and no electron-dense deposits. Therefore, we diagnosed Goodpasture syndrome associated with minimal change nephrotic syndrome (MCNS). Some reports have dealt with the association of RA and Goodpasture syndrome with D-penicillamine, and of RA and antineutrophil cytoplasmic antibodies (ANCA)-related vasculitis with pulmonary hemorrhage, but none has dealt with cases complicated with RA and Goodpasture syndrome associated with MCNS. Accordingly, whether or not there is a causal relationship between RA and Goodpasture syndrome remains obscure, but since the number of reported cases is small, experience with more cases is necessary to clarify this matter.

  4. Is there an association of hepatitis B virus infection with minimal change disease of nephrotic syndrome? A clinical observational report.

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    Zhou, Tian-Biao; Jiang, Zong-Pei

    2015-04-01

    The rate of hepatitis B virus (HBV) infection is high in the Chinese population, and the implications of HBV infection are widely recognized, and membranous nephropathy is the most common renal lesion to be associated with HBV infection. Minimal change disease (MCD) is one of the most important histopathological characteristics in patients with nephrotic syndrome. There is no any study to report that HBV infection is associated with the etiology of MCD. Herein, we report four MCD patients with HBV infection and speculate that there is an association of HBV infection with the pathological type of MCD. In this study, we also reported the treatment schedule for these four MCD patients, and found that the anti-virus alone and combination of anti-virus with immunosuppressive agent could obtain a benefit for MCD patients with HBV infection. However, a well-designed study should be performed to confirm this association.

  5. Nephrotic syndrome; is rituximab the light at the end of the tunnel in the treatment of adult steroid-dependent minimal change disease and focal segmental glomerulosclerosis?

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    Kronbichler, Andreas; Mayer, Gert

    2014-01-01

    Implication for health policy/practice/research/medical education: Reports on patients with steroid-dependent nephrotic syndrome and underlying minimal change disease or focal segmental glomerulosclerosis have shown promising results. There is a strong need for more trials conducted in a prospective, controlled manner to clearly recommend rituximab therapy in this indication on a regular basis.

  6. [Ultrastructure of glomerular podocyts in the incipient phase of minimal change nephrotic syndrome with thin basement membrane disease].

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    Ogawa, Ryo; Miyoshi, Ken-ichi; Nagao, Tomoaki; Jotoku, Masanori; Irita, Jun; Okura, Takafumi; Higaki, Jitsuo

    2012-01-01

    An 80-year-old woman was referred to the Division of Nephrology at Ehime University Hospital because of leg edema in December 2010. She had been treated with 300 mg of tocopherol for scleroderma since 2007 and treated with 9 mg of prednisolone (PSL) for autoimmune hearing loss since 2010. Due to the occurrence of mild hematuria (5-9/HPF), proteinuria (0.9 g/day) and an increased serum creatinine level (1.31 mg/dL), a renal biopsy was performed. Light microscopy (LM) showed minor abnormality in the glomeruli, and immunohistology showed the absence of deposits of immunoglobulins and complements. Electron microscopy (EM) showed a thin glomerular basement membrane with a limited level of podocyte abnormalities. Due to the findings of intimal thickening of interlobular arteries and subcapsular accumulation of global sclerosis on LM, she was diagnosed with nephrosclerosis and thin basement membrane disease. Four weeks later, her leg edema had increased considerably and urinary protein had increased to 12.4 g/day. The second biopsy showed similar findings in LM and IF as the first biopsy, but EM revealed diffuse foot process effacement. She was diagnosed with minimal change nephrotic syndrome (MCNS) and treated with methylprednisolone pulse therapy followed by 40 mg of oral PSL. Her urinary protein had completely disappeared 6 weeks later. Complete remission with PSL treatment indicates that urinary protein at first renal biopsy was due to MCNS. Our case exhibited podocyte features in the incipient phase of human MCNS.

  7. DD genotype of ACE gene in boys: may it be a risk factor for minimal change nephrotic syndrome?

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    Alasehirli, Belgin; Balat, Ayşe; Büyükçelik, Mithat

    2012-01-01

    It has been shown that angiotensin-converting enzyme (ACE) gene insertion/deletion (I/D) polymorphism affects the circulating and cellular levels of ACE and may be a risk factor in several renal diseases. We analyzed the association of ACE gene I/D polymorphism with the clinical presentation of minimal change nephrotic syndrome (MCNS) in a Turkish child population. This study consisted of 97 children with MCNS and 144 healthy controls. Genotyping of ACE gene was performed using polymerase chain reaction (PCR). The distributions of ACE genotypes were II in 13%, ID in 49%, and DD in 38% in patient group, and 9%, 49%, and 42% in control group, respectively. The frequency of the D allele was 63% and that of the I allele was 37% in patients. There were no relevant differences in the allele frequencies and genotypes of ACE I/D polymorphism between patients and controls. However, DD genotype was higher in boys in children with MCNS (78.4%. vs. 50.0%, p = 0.004). The frequencies of DD genotype and D allele in boys were 7.25 and 2.56 times higher than II genotype and I allele in the patient group, respectively. We suggest that DD genotype in boys may be one of the risk factors for MCNS.

  8. Blood pressure in children with minimal change nephrotic syndrome during oedema and after steroid therapy: the influence of familial essential hypertension.

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    Kontchou, Laure Monthe; Liccioli, Giulia; Pela, Ivana

    2009-01-01

    To verify the variations of blood pressure in children with minimal change nephrotic syndrome and to correlate the blood pressure with familial history of essential hypertension. We measured blood pressure in 49 prepubertal nephrotic children, 17 females and 32 males, in the first week of oedema, and after 4 weeks of ISKDC (International Study of Kidney Disease in Children) standard steroid therapy. The children were divided into two groups: one with and the other without familial history of essential hypertension. Among all the patients, 65% showed systolic and/or diastolic blood pressure higher than the 90th percentile at the first assessment. Among the children with a familial history of essential hypertension, in the oedematous phase of the nephrotic syndrome, 88% showed blood pressure higher than the 90th percentile and no children showed blood pressure lower than the 75th percentile. After therapy, the percentage of children with blood pressure higher than the 90th percentile was 52%. In the group with a negative familial history, at the onset 53% showed blood pressure over the 90th percentile. After 4 weeks of therapy, the percentage of children with blood pressure higher than the 90th percentile was 34%. Our study reveals the influence of familial essential hypertension in the oedematous phase of the nephrotic syndrome in children. (c) 2009 S. Karger AG, Basel.

  9. Acute tubulointerstitial nephritis following intravenous immunoglobulin therapy in a male infant with minimal-change nephrotic syndrome.

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    Tanaka, H; Waga, S; Tateyama, T; Sugimoto, K; Kakizaki, Y; Yokoyama, M

    1999-10-01

    A boy aged 4 years with nephrotic syndrome (NS) was referred to our hospital because of the third relapse of NS. Hypogammaglobulinemia associated with massive proteinuria was observed at the presentation. Residual urinary tract infection required intravenous piperacillin and immunoglobulin therapy (IVIG). Soon after IVIG, he complained of high fever with chills, bilateral knee joint pain, dry cough and chest discomfort. Although he did not develop renal insufficiency, a transient increase in the urinary beta2-microglobulin and decrease in the serum complement hemolytic activity were observed. These clinical manifestations spontaneously ceased. A percutaneous renal biopsy for his NS performed 19 days after the episode of allergic reaction revealed tubulointerstitial nephritis (TIN) with marked eosinophil infiltrates. Glomeruli showed minor glomerular abnormalities. Renal complications associated with IVIG treatment have been reported to date, however, acute TIN has rarely been seen.

  10. New insight into the pathogenesis of minimal change nephrotic syndrome: Role of the persistence of respiratory tract virus in immune disorders.

    Science.gov (United States)

    Zhang, Hui; Wang, Zheng; Dong, Liqun; Guo, Yannan; Wu, Jin; Zhai, Songhui

    2016-07-01

    The pathogenesis of minimal change nephrotic syndrome (MCNS) is a complex clinical problem which, unfortunately, has been in need of significant breakthroughs for decades. Improved understanding of the mechanisms is important to develop effective treatment strategies. To our knowledge, the pathogenesis of MCNS is multifactorial, involving both intrinsic and extrinsic factors, reasonable to be regarded as a "long chain" cascade reaction. Current studies implicating that the disease could probably be caused by immune disorders, however, have focused merely on the middle or terminal of this "long chain". It remains unclear what really triggers the immune disorders. It is noteworthy that the close association of respiratory tract infection with the occurrence, relapse and aggravation of nephrotic syndrome has been confirmed for over two decades. Derived from what we demonstrated in earlier studies, that the persistence of respiratory tract virus may contribute to the onset and development of MCNS, this review summarizes current evidence investigating the possible mechanisms of viral persistence, and discusses the role of viral persistence in the pathogenesis of MCNS. The key point is: whether the persistence of respiratory tract virus results in immune disorders. The available evidence under review also highlight the fact that the background of genetic susceptibility to the disease was found in many patients, which could be triggered by extrinsic factors, e.g. by the infection of respiratory tract virus.

  11. Adult and paediatric patients with minimal change nephrotic syndrome show no major alterations in glomerular expression of sulphated heparan sulphate domains.

    Science.gov (United States)

    Wijnhoven, Tessa J M; Geelen, Joyce M; Bakker, Marinka; Lensen, Joost F M; Rops, Angelique L W M M; Kramer, Andrea B; Navis, Gerjan; van den Hoven, Mabel J W; van der Vlag, Johan; Berden, Jo H M; Wetzels, Jack F M; van den Heuvel, Lambert P W J; Monnens, Leo A H; van Kuppevelt, Toin H

    2007-10-01

    Minimal change nephrotic syndrome (MCNS) is the most frequent form of nephrotic syndrome in childhood. In the glomerular basement membrane (GBM) of adult patients with MCNS, a reduced expression of a specific heparan sulphate (HS) domain has been reported. In children with MCNS, urinary activity of the HS-degrading enzyme heparanase was increased. It is, therefore, possible that a decreased GBM HS expression is associated with the pathogenesis of proteinuria in patients with MCNS. In this study, HS in glomeruli of five adult and six paediatric patients with MCNS were analysed by immunofluorescence staining using four different antibodies, each defining a specific sulphated HS domain. The pediatric patients were subdivided into three groups depending on the presence or absence of podocyte foot process effacement, the level of proteinuria and prednisone administration at the time of the biopsy. In addition, kidneys of rats with adriamycin nephropathy (ADRN), a model for MCNS, were included in the study. Expression of sulphated HS domains was not aberrant in adult or paediatric patients compared with control subjects. Children with and without proteinuria had the same HS content. In contrast, rats with ADRN showed a decreased glomerular expression of sulphated HS domains. These results suggest that in patients with MCNS proteinuria is not associated with major changes in glomerular expression of sulphated HS domains.

  12. A novel approach to investigation of the pathogenesis of active minimal-change nephrotic syndrome using subtracted cDNA library screening.

    Science.gov (United States)

    Sahali, Djillali; Pawlak, André; Valanciuté, Asta; Grimbert, Philippe; Lang, Philippe; Remy, Philippe; Bensman, Albert; Guellaën, Georges

    2002-05-01

    Clinical and experimental observations suggest that minimal-change nephrotic syndrome (MCNS) results from T cell dysfunction, via unknown mechanisms. For the identification of genes that are potentially involved in MCNS, a subtractive cDNA library was constructed from cDNA from T cell-enriched peripheral blood mononuclear cells obtained from the same patient during relapse versus remission ("relapse minus remission"). This library was screened by differential hybridization with forward ("relapse minus remission") and reverse ("remission minus relapse") subtractive cDNAs probes, as well as unsubtracted probes from relapse and remission, and irrelevant nephrotic syndrome (membranous nephropathy). A total of 84 transcripts were isolated, of which 12 matched proteins of unknown function and 30 were unknown clones. Among the 42 known transcripts, at least 18 are closely involved in the T cell receptor-mediated complex signaling cascade, including genes encoding components of the T cell receptor and proteins associated with the cytoskeletal scaffold, as well as transcription factors. In particular, it was demonstrated that the expression levels of Fyb/Slap, L-plastin, and grancalcin were increased during relapse, suggesting that the integration of proximal signaling after T cell engagement involves the preferential recruitment of these cytoskeleton-associated proteins in MCNS. Because very low levels of interleukin-12 receptor beta2 mRNA were detected in relapse samples, the interleukin-12 signaling pathway might be defective, suggesting that, in MCNS, T cell activation evolves toward a T helper 2 phenotype. Therefore, the combination of subtractive cloning and differential screening constitutes an efficient approach to the identification of genes that are likely to be involved in the pathophysiologic processes of MCNS.

  13. Nephrotic Syndrome Associated with Thymoma

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    Sheng-Yen Hsiao

    2014-12-01

    Full Text Available Thymoma is associated with a wide variety of paraneoplastic syndromes such as myasthenia gravis, pure red cell aplasia, and hypogammaglobulinemia. Paraneoplastic glomerulonephritis is a rare clinical presentation of malignancy. This condition often goes undetected as it has no specific clinical symptoms and signs. Approximately 2% of thymoma patients have been reported to have paraneoplastic glomerulonephritis, and the nephrotic syndrome has been shown to be a clinical manifestation of the disorder. We report two cases diagnosed to have thymoma and nephrotic syndrome. Renal biopsy showed that one case had focal segmental glomerulosclerosis, whereas the other had minimal change disease. In case 1, the nephrotic syndrome was diagnosed before thymoma was detected, while in case 2, the symptomatic nephrotic syndrome occurred after thymoma treatment. Because parathymic nephropathy often remains undiagnosed and interferes with treatment, the possibility of the nephrotic syndrome should always be considered throughout the course of thymoma management, particularly in patients who also present with anasarca or hypoalbuminemia. A multidisciplinary approach is needed. Besides, it is to be noted that the nephrotic syndrome may be the initial presentation of thymoma.

  14. Complications of nephrotic syndrome

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    Se Jin Park

    2011-08-01

    Full Text Available Nephrotic syndrome (NS is one of the most common glomerular diseases that affect children. Renal histology reveals the presence of minimal change nephrotic syndrome (MCNS in more than 80% of these patients. Most patients with MCNS have favorable outcomes without complications. However, a few of these children have lesions of focal segmental glomerulosclerosis, suffer from severe and prolonged proteinuria, and are at high risk for complications. Complications of NS are divided into two categories: disease-associated and drug-related complications. Disease-associated complications include infections (e.g., peritonitis, sepsis, cellulitis, and chicken pox, thromboembolism (e.g., venous thromboembolism and pulmonary embolism, hypovolemic crisis (e.g., abdominal pain, tachycardia, and hypotension, cardiovascular problems (e.g., hyperlipidemia, acute renal failure, anemia, and others (e.g., hypothyroidism, hypocalcemia, bone disease, and intussusception. The main pathomechanism of disease-associated complications originates from the large loss of plasma proteins in the urine of nephrotic children. The majority of children with MCNS who respond to treatment with corticosteroids or cytotoxic agents have smaller and milder complications than those with steroid-resistant NS. Corticosteroids, alkylating agents, cyclosporin A, and mycophenolate mofetil have often been used to treat NS, and these drugs have treatment-related complications. Early detection and appropriate treatment of these complications will improve outcomes for patients with NS.

  15. Minimal change disease

    Science.gov (United States)

    ... get worse You develop new symptoms, including side effects from the medicines used to treat the disorder Alternative Names Minimal change nephrotic syndrome; Nil disease; Lipoid nephrosis; Idiopathic nephrotic syndrome of childhood Images ...

  16. Minimal-change disease as a paraneoplastic syndrome in a patient with ovarian carcinoma.

    Science.gov (United States)

    González-Fontal, Guido R; Restrepo, Juan G; Henao-Martínez, Andrés F

    2011-12-01

    Minimal-change disease (MCD) is an exceptional paraneoplastic presentation. We are describing the case of an ovarian paraneoplastic nephrotic syndrome. The kidney biopsy was consistent with MCD. Steroids and immunosuppressive therapy were given with no change in the nephrotic-range proteinuria. A complete resolution of the nephrotic syndrome was soon observed with improvement of her clinical condition after five cycles of chemotherapy with paclitaxel and carboplatin and tumor-debulking surgery. Ovarian carcinoma paraneoplastic nephrotic syndrome secondary to MCD is an extremely rare event, which is important to recognize since it is responsive to the standard chemotherapy.

  17. Nephrotic Syndrome in Adults

    Science.gov (United States)

    ... Kidney Disease Nephrotic Syndrome in Adults Related Topics Section Navigation Kidney Disease Acquired Cystic Kidney Disease Amyloidosis & ... for a Child with Kidney Disease Ectopic Kidney Medullary Sponge Kidney Kidney Dysplasia Kidney Failure Choosing a ...

  18. 微小病变性肾病综合征激素抵抗的多因素分析%Multiple factors analysis of steroid resistance of minimal change nephrotic syndrome

    Institute of Scientific and Technical Information of China (English)

    王明军; 张金荣; 徐婉梅; 黎伟; 张影华; 张元元; 何建静; 廖蕴华

    2015-01-01

    Aim:To evaluate risk factors of steroid-resistant minimal change nephrotic syndrome. Methods:retrospectively analysed 68 patients who were confirmed as minimal-change nephritic syndrome by renal biopsies,and divided as group steroid-resistant group(n =30)and non steroid-resistant group(n=38).A univariate analysis was performed firstly.Then the significant ones were performed a multivari-ate analysis by using binary Logistic regression model.Lastly,the sensitivity,the specificity,and the best threshold value of the indexes,which as indicating the steroid-resistant minimal change disease,was testi-fied by drawing a receiver operating characteristic curve(ROC).Results:(1)combined infection(P =0.014),red blood cell count(P =0.005 ),total cholesterol (P =0.015 ),24 h urinary protein (P = 0.000),serum albumin(P =0.011),high urine uric acid(P =0.045),C4 decrease(P =0.028)were all the risk factors of steroid-resistant minimal change disease(P 3.5 /HP,24 h u-rinary protein >5.57 g/d total cholesterol >9.49 mmol /L were the best threshold value indicating ster-oid-resistant minimal change disease.Conclusion:(1)infection,hematuria,24 h urinary protein quanti-tative is independent risk factors of Steroid-resistant minimal change nephrotic syndrome.(2)when Oc-cur hematuria,24 h urinary proteind≥5.57 g/d,total cholesterol≥9.49 mmol /L,,Should be alert to the occurrence of Steroid-resistant minimal change nephrotic syndrome.%目的:探讨微小病变性肾病综合征激素抵抗的相关危险因素.方法:回顾性调查68例经肾组织活检确诊为微小病变性肾小球肾炎患者临床资料,实验室检查结果及随访情况,将患者分为激素抵抗组(30例)及非抵抗组(38例),采用 SPSS17.0软件,进行单因素分析,对有意义的单因素采用二分类 Logistic 回归进行多因素分析,绘制受试者工作特性曲线(ROC)评价检验指标在预测微小病变性肾病综合征激素抵抗时的敏感度,特异

  19. Nephrotic syndrome in Namibian children.

    Science.gov (United States)

    van Buuren, A J; Bates, W D; Muller, N

    1999-10-01

    Patterns of nephrotic syndrome vary between regions and countries, and influence approaches to management. In the mid-1970s the University of Stellenbosch became involved in providing tertiary care to Namibia, including a paediatric nephrology service. The aim of this study was to document the clinical, pathological and outcome features of nephrotic syndrome in Namibian children. Seventy black Namibian children with nephrotic syndrome were managed from 1975 to 1988. Sixty-eight renal specimens (67 biopsies and 1 autopsy specimen) were evaluated. Twenty-nine of the 70 children (41.4%) were hepatitis B virus (HBV) carriers, of whom 25 (86.2%) were male. Of the 29, 26 had predominantly membranous glomerulonephritis (MGN), 1 mesangiocapillary glomerulonephritis (MCGN), and 1 focal segmental glomerulosclerosis (FSGS); 1 child in advanced renal failure was not biopsied. Five children (7.4%) showed minimal change disease (MCD), 11 (16.2%) FSGS and 15 (22.1%) diffuse mesangial proliferative glomerulonephritis (DMP). The remaining 10 children showed diffuse glomerulosclerosis (6), MCGN (3) and endocapillary proliferative GN (1). Four of the 5 children with MCD went into remission on immunosuppressive treatment. Of the 15 with DMP, 4 improved spontaneously and only 1 of those treated did not improve. Only 2 of those with FSGS improved on treatment. The children with HBV-associated MGN and MCGN were offered symptomatic rather than specific treatment. Thirteen children presented with degrees of chronic renal failure. Eight are known to have died, 3 of relentless nephrotic syndrome and 4 (of whom 3 were HBV carriers) of end-stage renal failure. One child died of penicillin anaphylaxis. The pattern of nephrotic syndrome in black Namibian children differed greatly from the non-African pattern elsewhere in that MCD was uncommon and HBV-associated GN was the most common single group. The most frequent pattern of HBV-associated GN was MGN with some mesangiocapillary features showing

  20. Hypercoagulability and nephrotic syndrome.

    Science.gov (United States)

    Gigante, Antonietta; Barbano, Biagio; Sardo, Liborio; Martina, Paola; Gasperini, Maria L; Labbadia, Raffaella; Liberatori, Marta; Amoroso, Antonio; Cianci, Rosario

    2014-05-01

    Patients with nephrotic syndrome are at increased risk for thromboembolic events such as deep venous and arterial thrombosis, renal vein thrombosis and pulmonary embolism. This thrombophilic phenomenon has been attributed to a "hypercoagulable" state in which an imbalance between naturally occurring pro-coagulant/pro-thrombotic factors and anti-coagulant/antithrombotic factors promotes in situ thrombosis in deep veins or arteries. Management of thromboembolic events may be divided in prophylactic and therapeutic strategies. Hypoalbuminemia is the most significant independent predictor factor of thrombotic risk, especially for values thrombosis. Reviewing the recent literature, we suggest the best therapeutic management of anticoagulation for patients with nephrotic syndrome, focusing on prophylactic strategies.

  1. Cerebral venous thrombosis and secondary polycythemia in a case of nephrotic syndrome

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    Shankar Prasad Nagaraju

    2016-01-01

    Full Text Available Cerebral venous thrombosis (CVT and polycythemia are considered as rare and life threatening complications of nephrotic syndrome. We report an unusual combination of both these complications in a case of nephrotic syndrome due to minimal change disease that was treated successfully. There was prompt and complete remission of nephrotic syndrome with steroid therapy, concurrent with complete resolution of polycythemia and CVT.

  2. Predictors of Relapse in Adult-Onset Nephrotic Minimal Change Disease.

    Science.gov (United States)

    Lee, Hajeong; Yoo, Kyung Don; Oh, Yun Kyu; Kim, Dong Ki; Oh, Kook-Hwan; Joo, Kwon Wook; Kim, Yon Su; Ahn, Curie; Han, Jin Suk; Lim, Chun Soo

    2016-03-01

    Minimal change disease (MCD) is a well-known benign primary glomerulonephritis because of its distinct rare tendency to progress to end-stage renal disease. However, factors associated with relapse in adults are not well known. We aimed to identify predictors of relapse in adult-onset MCD patients.A retrospective cohort of 195 patients with adult-onset primary MCD with nephritic syndrome and disease onset between 1979 and 2013 was followed up for >12 months. The number of relapses was counted and predictors of relapse were analyzed.A total of 195 patients were included. Median age at diagnosis was 38 years (IQR, 23-53 years) and 113 (57.9%) were men. During 81 months (IQR, 44-153 months) of follow-up, 92% of patients achieved remission after initial treatment. However, only 60 (32.8%) did not experience a relapse and 11 patients failed to remit. Among the remaining 124 patients, 65 experienced a relapse once or twice and 59 experienced a relapse more than twice. Younger onset age, increased severity of nephrotic features such as lower serum albumin levels and higher cholesterol level were associated with relapse. Interestingly, the grade of mesangial proliferation was lower in patients who experienced a relapse. Initial combined treatment with corticosteroids (CS) and cyclophosphamide reduced the number of relapses. In addition, patients with shorter treatment duration tended to experience relapse more often. Multivariate analysis showed that younger onset age, combined mesangial proliferation, initial treatment regimen, and treatment duration were independent risk factors for relapse. Progression to end-stage renal disease was developed in only a patient.In conclusion, more than two-thirds of adult-onset nephrotic MCD patients experienced relapse, although their renal progression was rare. Younger onset age, CS without cyclophosphamide treatment, and shorter treatment duration were independent risk factors for relapse in adult-onset MCD patients.

  3. Congenital nephrotic syndrome

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    Claudia Fanni

    2014-06-01

    Full Text Available CNS (Congenital nephrotic syndrome is a disorder characterized by the presence of a nephrotic syndrome in the first three months of life. Different pathologies can cause this syndrome. In general, we can distinguish primary forms (sporadic and hereditary and secondary forms (acquired and associated with other syndromes. The most common form is the Finnish CNS (CNF, congenital nephrotic syndrome of the Finnish type, a hereditary form whose name derives from the fact that the highest incidence is described in that country (1.2:10,000. The pathogenesis, the clinical picture, the diagnostic criteria, the therapy and the outcome are described in details.  Proceedings of the International Course on Perinatal Pathology (part of the 10th International Workshop on Neonatology · October 22nd-25th, 2014 · Cagliari (Italy · October 25th, 2014 · The role of the clinical pathological dialogue in problem solving Guest Editors: Gavino Faa, Vassilios Fanos, Peter Van Eyken

  4. Radiation nephritis causing nephrotic syndrome

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    Jennette, J.C.; Ordonez, N.G.

    1983-12-01

    Clinical symptoms of acute radiation nephritis with nephrotic syndrome developed in a fifty-six-year-old woman after abdominal radiation therapy for an astrocytoma of the spinal cord. The diagnosis of radiation nephritis was confirmed by renal biopsy. To our knowledge, this is the first documented case of radiation nephritis associated with nephrotic syndrome.

  5. Cerebral Sinovenous Thrombosis in a Child with Idiopathic Nephrotic Syndrome

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    L. Ghedira Besbes

    2011-01-01

    Full Text Available Nephrotic syndrome (NS is a renal disorder characterized by heavy proteinuria, hypoalbuninemia, edema and hypercholesterolemia. Nephrotic syndrome in children is known to be associated with an hypercoagulable state and thromboembolic complications. However cerebral sinovenous thrombosis (CSVT is very rare. Here we report a seven-year-old child with steroid-dependent idopathic nephrotic syndrome resulting from a minimal change disease, developed multiple cerebral sinovenous thrombosis, presenting with headache, left sixth nerve palsy, and papilledema. The diagnosis of CSVT was established by cranial computed tomography, magnetic resonance imaging, and magnetic resonance angiography. He gradually recovered after anticoagulant therapy. CSVT is very rare in nephrotic children. The diagnosis of CSVT should be considered in any patient with nephrotic syndrome who develops neurologic symptoms. This report highlights the importance of suspecting and recognizing this potentially life threatening complication and initiating early treatment.

  6. Mesothelioma of the testis and nephrotic syndrome: a case report

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    Bacchetta Justine

    2009-06-01

    Full Text Available Abstract Introduction Paraneoplastic glomerulopathies are rare manifestations of neoplastic disease to be distinguished from iatrogenic renal damage. Solid tumors are preferentially associated with membranous nephropathy, whereas Hodgkin's lymphomas are associated with minimal change disease. Case presentation We report a 63-year-old Caucasian male diagnosed with a mesothelioma of the tunica vaginalis testis who, secondary to this, also presented with a nephrotic syndrome due to minimal change disease. In the present case, the paraneoplastic etiology of the nephrotic syndrome can be discussed on four unusual elements: minimal change lesions were found; the glomerulopathy was very sensitive to corticosteroids; the nephrotic syndrome occurred 11 months after the diagnosis of the primary malignancy, but concomitantly with the recurrence; and the nephrotic syndrome did not decrease with tumor control and did not recur when the mesothelioma escaped treatment. No other etiologies could nevertheless explain this phenomenon. Conclusion Paraneoplastic nephrotic syndrome is often associated with membranous nephropathy in patients with solid tumors, especially in patients with lung and gastrointestinal tract neoplasia. The management of these patients is associated with a symptomatic treatment such as sodium and water restriction, diuretics and ACE inhibitors and a prophylaxis of specific complications of nephrotic syndrome including thromboembolism, infections and lipid abnormalities. Treatment of neoplasia must be undertaken rapidly, treatments must be regularly analyzed and drugs binding to albumin may be used with precaution.

  7. Bardet-Biedl syndrome presenting with steroid sensitive nephrotic syndrome

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    K K Singh

    2015-01-01

    Full Text Available Bardet-Biedl syndrome (BBS is a rare autosomal recessive disorder characterized by postaxial polydactyly, retinitis pigmentosa, central obesity, mental retardation, hypogonadism, and renal involvement. Renal involvement in various forms has been seen in BBS. Cases with nephrotic range proteinuria not responding to steroid have been described in this syndrome. Here we report a case of BBS who presented with nephrotic range proteinuria. The biopsy findings were suggestive of minimal change disease. The child responded well to steroid therapy and remains in remission.

  8. Primary Sjögren's syndrome with minimal change disease--a case report.

    Science.gov (United States)

    Yang, Mei-Li; Kuo, Mei-Chuan; Ou, Tsan-Teng; Chen, Hung-Chun

    2011-05-01

    Glomerular involvement in patients with primary Sjögren's syndrome (pSS) has rarely been reported. Among them, membranoproliferative glomerulonephritis and membranous nephropathy are the more common types. We report a middle-aged female presenting concurrently with nephrotic syndrome and microscopic hematuria, and her pSS was diagnosed by positive anti-Ro (SSA)/anti-La (SSB) autoantibodies, dry mouth, severely diffuse impaired function of both bilateral parotid and submandibular glands, and a positive Schirmer test. Renal pathology revealed minimal change disease and thin basement membrane nephropathy. The patient's nephrotic syndrome resolved after treatment with corticosteroids. To our knowledge, this is the first report of minimal change disease in a patient with pSS.

  9. Primary Sjögren’s syndrome with minimal change disease—A case report

    Directory of Open Access Journals (Sweden)

    Mei-Li Yang

    2011-05-01

    Full Text Available Glomerular involvement in patients with primary Sjögren’s syndrome (pSS has rarely been reported. Among them, membranoproliferative glomerulonephritis and membranous nephropathy are the more common types. We report a middle-aged female presenting concurrently with nephrotic syndrome and microscopic hematuria, and her pSS was diagnosed by positive anti-Ro (SSA/anti-La (SSB autoantibodies, dry mouth, severely diffuse impaired function of both bilateral parotid and submandibular glands, and a positive Schirmer test. Renal pathology revealed minimal change disease and thin basement membrane nephropathy. The patient’s nephrotic syndrome resolved after treatment with corticosteroids. To our knowledge, this is the first report of minimal change disease in a patient with pSS.

  10. The nephrotic syndrome and its complications.

    Science.gov (United States)

    Cameron, J S

    1987-09-01

    Modern views of the pathogenesis and natural history of nephrotic syndrome have changed substantially since the early studies by Cotugno and Bright. Contrary to beliefs held 20 years ago, we do not possess a unique satisfying explanation for the induction, maintenance, and resolution of nephrotic edema, and many concepts firmly established as "classic" are now being revised or reconsidered. These include the relationship between urinary protein losses and hypoalbuminemia, which is complicated by several factors such as daily protein intake, albumin catabolism, and the possible role of albumin loss at extrarenal sites. The influence of lowered plasma albumin on the decrease in plasma volume is also quite complex, due to technical difficulties in measuring plasma volume and turnover of radio-labeled albumin. The most contentious areas are how sodium and water retention are initiated and maintained and the relationship between hypoalbuminemia, plasma oncotic pressure, and edema. While aldosterone excretion and plasma concentrations are elevated in nephrotic patients, data on the renin-angiotensin system are controversial and the renal handling of sodium is related to a host of factors including glomerular filtration rate, altered proximal tubular reabsorption, and the role of vasodilators or vasoconstrictors. The complications of nephrotic syndrome are protean and relatively common. Among those are acute renal failure, thrombosis, infections, and hyperlipidemia. Since the introduction of percutaneous biopsy, the spectrum of lesions underlying nephrotic syndrome has widened considerably, the most common being minimal change, especially in children. There are very few prognostic indicators by which response to treatment may be predicted and these include persistent microscopic hematuria.

  11. Pulmonary carcinoid tumor associated with nephrotic syndrome.

    Science.gov (United States)

    DePace, N L; Elquezabal, A; Hardenburg, H C

    1980-04-01

    A patient with carcinoid tumor of the lung associated with nephrotic syndrome was treated. Excision of the tumor resulted in remission of marked proteinuria, hypoalbuminemia, and edema. A review of the literature disclosed many neoplasms associated with the nephrotic syndrome; however, no association of the nephrotic syndrome and a carcinoid tumor of the lung has previously been reported, to our knowledge.

  12. Mycophenolate mofetil versus cyclosporine for remission maintenance in nephrotic syndrome

    NARCIS (Netherlands)

    E.M. Dorresteijn (Eiske); J.E. Kist-Van Holthe (Joana); E.N. Levtchenko (Elena); J. Nauta (Jeroen); W.C.J. Hop (Wim); A.J. van der Heijden (Bert)

    2008-01-01

    textabstractWe performed a multi-centre randomized controlled trial to compare the efficacy of mycophenolate mofetil (MMF) to that of cyclosporine A (CsA) in treating children with frequently relapsing nephrotic syndrome and biopsy-proven minimal change disease. Of the 31 randomized initially select

  13. Thrombosis in nephrotic syndrome.

    Science.gov (United States)

    Barbano, Biagio; Gigante, Antonietta; Amoroso, Antonio; Cianci, Rosario

    2013-07-01

    Nephrotic syndrome (NS) is characterized by heavy proteinuria, edema, hypoalbuminemia, and hyperlipidemia and the most frequent causes are glomerular diseases. An uncommon presentation is iatrogenic NS, an adverse effect of some drugs administration. In the clinical course of NS, a typical feature is dysregulated coagulation state, promoted by the breakdown of permselectivity barrier of the glomerular capillary wall, resulting in the leakage of high-molecular-mass proteins, at least the size of albumin. This hypercoagulable condition is supported by several factors, such as abnormalities in platelet activation and an imbalance between anticoagulation/antithrombosis and procoagulant/prothrombotic mechanisms. Thus, NS and the risk of developing thromboses are strictly related. Thrombotic events affect the venous system rather than arterial vessels with different features and frequencies. Deep venous system of the lower extremities and renal veins are the most frequent source of pulmonary embolism, the most dangerous NS complication. Prophylactic anticoagulation and thrombosis treatment are not clearly established because large randomized trials and guidelines are lacking. The management of NS and the decision of when and how to anticoagulate the patient represent a teamwork challenge for physicians.

  14. Permeability factors in idiopathic nephrotic syndrome: historical perspectives and lessons for the future

    NARCIS (Netherlands)

    Maas, R.J.H.; Deegens, J.K.J.; Wetzels, J.F.M.

    2014-01-01

    The term idiopathic nephrotic syndrome (iNS) traditionally covers minimal change disease and primary focal segmental glomerulosclerosis (FSGS), now thought to be separate disease entities. Clinical and experimental evidence suggest that circulating permeability factors are involved in their pathogen

  15. Familial steroid-sensitive idiopathic nephrotic syndrome: seven cases from three families in China

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    Yonghui Xia

    2013-05-01

    Full Text Available OBJECTIVES: Familial steroid-sensitive idiopathic nephrotic syndrome is rare, and only approximately 3% of patients have affected siblings. METHODS: Herein, we report seven cases of patients with steroid-sensitive idiopathic nephrotic syndrome from three Chinese families. Mutational screening of the Nphs2 gene was performed in all the patients. RESULTS: All seven of the familial steroid-sensitive idiopathic nephrotic syndrome cases in our sample exhibited minimal change disease, and one case also presented with mesangial proliferative glomerulonephritis, according to the renal pathology. No significant was associations were found between Nphs2 gene mutations and the onset of proteinuria and nephrotic syndrome in these familial cases. CONCLUSIONS: The presence of minimal change disease is important, but it is not an unusual finding in patients with familial steroid-sensitive idiopathic nephrotic syndrome, which appears to be clinically benign and genetically distinct from other types of nephrosis.

  16. Rare case of nephrotic syndrome: Schimke syndrome

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    Anna Kelly Krislane de Vasconcelos Pedrosa

    Full Text Available Abstract Schimke syndrome corresponds to dysplasia of bone and immunity, associated with progressive renal disease secondary to nephrotic syndrome cortico-resistant, with possible other abnormalities such as hypothyroidism and blond marrow aplasia. It is a rare genetic disorder, with few reports in the literature. The most frequent renal involvement is nephrotic syndrome with focal segmental glomerulosclerosis and progressive renal failure. The objective of this study was to report a case of Schimke syndrome, diagnostic investigation and management of the case.

  17. Nephrotic syndrome due to immunoglobulin M mesangial glomerulonephritis preceding juvenile idiopathic arthritis.

    Science.gov (United States)

    Voyer, Luis E; Alvarado, Caupolican; Cuttica, Rubén J; Balestracci, Alejandro; Zardini, Marta; Lago, Néstor

    2013-05-21

    The association between nephrotic syndrome and juvenile idiopathic arthritis have rarely been described in pediatric patients. We report a child with steroid-responsive nephrotic syndrome, with frequent relapses, who presented with a new relapse of nephrotic syndrome associated with arthritis and uveitis at 21 months in remission after treatment with chlorambucil. Juvenile idiopathic arthritis was diagnosed and kidney biopsy examination showed mesangial glomerulonephritis with immunoglobulin M deposits. To our knowledge, only 2 cases of nephrotic syndrome preceding juvenile idiopathic arthritis have been reported, one without histopathology assessment and the other with minimal change disease. Although mesangial glomerulonephritis with nephrotic syndrome and juvenile idiopathic arthritis could have been coincidental, the immune pathogenic mechanism accepted for both diseases suggests they could be related.

  18. Extrarenal complications of the nephrotic syndrome.

    Science.gov (United States)

    Harris, R C; Ismail, N

    1994-04-01

    The systemic complications of nephrotic syndrome are responsible for much of the morbidity and mortality seen with this condition. This review discusses the causes for the hypoalbuminemia and the associated metabolic abnormalities of the nephrotic syndrome. No unifying hypothesis exists for the induction, maintenance, and resolution of nephrotic edema. In view of the wide spectrum of renal diseases leading to the nephrotic syndrome, more than a single mechanism may be responsible for the renal salt retention in these diverse conditions. Although hypoalbuminemia may be important, especially when plasma oncotic pressure is very low (serum albumin nephrotic kidney appears to be a major factor in pathogenesis of the edema. However, the decreased serum albumin and/or oncotic pressure seen with nephrotic syndrome is a major contributing factor to the development of the hyperlipidemia of nephrotic syndrome. Patients with unremitting nephrotic syndrome should be considered for combined dietary and lipid-lowering drug therapy. Urinary losses of binding proteins lead to the observed abnormalities in the endocrine system and in trace metals, and urinary losses of coagulation factors contribute to the hypercoagulable state. At present, selective renal venography is recommended when the suspicion of renal vein thrombosis is justified by clinical presentation. The impact on renal function caused by treating asymptomatic chronic renal vein thrombosis is undetermined, but anticoagulation for chronic renal vein thrombosis is associated with relatively few complications.

  19. Histopathological types in adult nephrotic syndrome

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    Md. Ghulam Yusuf

    2016-01-01

    Full Text Available In Bangladesh, there are very few studies about biopsy proven adult Nephrotic syndrome (NS with histological types and their clinical findings. To determine the histological types of glomerulonephritis (GN in adult NS and correlate them with the clinical presentations and biochemical parameters, we studied 100 biopsies in 87 patients who underwent ultrasonography- guided renal biopsy in Rangpur Medical College and Hospital from July 2010 to June 2012. The mean age of the patients was 32.8 ± 13.2 years; male was preponderance (72.4% and most of the patients (67.8% came from rural areas. Membranoproliferative GN (MPGN was the most common underlying cause that was found in 32 (36.8% patients followed by mesangial prolife- rative GN in 27 (31% patients, membranous GN in 16 (18.4% cases, minimal change disease in four (4.6% patients, diffuse proliferative GN in four (4.6% patients, focal segmental GN, and focal proliferative GN in two (2.4% patients each. High proteinuria level was found in minimal change disease, which was 7.59 ± 0.24 g/24 h (mean ± standard deviation. The most common symptoms were oliguria (92% and edema (86.2% followed by hematuria (dark urine (72.4% and hypertension (35.6%. MPGN was the most common histological type of adult NS in Rangpur.

  20. METHYLPREDNISOLONE PULSE THERAPY IN MANAGEMENT OF NON RESPONDER NEPHROTIC SYNDROME

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    A. Madani

    1999-08-01

    Full Text Available Some patients with the diagnosis of childhood nephrotic syndrome are unresponsive to conventional treatment regimens. Recent studies of more aggressive therapies have provided strong evidence of the benefit of high dose methylprednisolonc (MP protocol with alternate - day prednisone alone or with alternate - day prednisone plus an alkylating agent (I in these patients."nFrom May 1996 to May 1997 we have treated 14 patients with non-responder nephrotic syndrome with mcthyprcdnisolone protocol. Eight patients had histologic diagnosis of focal segmental glomerulosclerosis, 3 diffuse mesangial proliferation and 3 has minimal change disease. C'ylosporin was added in two patients to methylprcdnisotonc at the beginning of the second course of therapy. Tfie patients were observed for an average of 8 months (range 4-12 months. In the last follow up there were no patients in remission and all remained nephrotic. Seven patients had persistent massive proteinuria with normal creatinine clearance (CrCI. Two had decreased CrCl. Five progressed to end-stage renal disease. Tlicsc observations suggest that "Puke" methy{prednisolone is not effective in patients with non respondcr nephrotic syndrome.

  1. An unusual case of nephrotic syndrome

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    M Sahay

    2016-01-01

    Full Text Available Nephrotic syndrome can be rarely due to inherited disorders of enzymes. One such variety is lecithin cholesterol acyltransferase deficiency. It leads to accumulation of unesterified cholesterol in the eye and other organs. We report a case of nephrotic syndrome with cloudy cornea and hypocholesterolemia with foam cells and lipid deposits on renal biopsy. Awareness about this rare disease may help in the early institution of specific measures to prevent progression to end-stage renal disease.

  2. Nephrotic Syndrome in Children: From Bench to Treatment

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    J.-C. Davin

    2011-01-01

    Full Text Available Idiopathic nephrotic syndrome (INS is the most frequent form of NS in children. INS is defined by the association of the clinical features of NS with renal biopsy findings of minimal changes, focal segmental glomerulosclerosis (FSGS, or mesangial proliferation (MP on light microscopy and effacement of foot processes on electron microscopy. Actually the podocyte has become the favourite candidate for constituting the main part of the glomerular filtration barrier. Most cases are steroid sensitive (SSINS. Fifty percents of the latter recur frequently and necessitate a prevention of relapses by nonsteroid drugs. On the contrary to SSINS, steroid resistant nephrotic syndrome (SRINS leads often to end-stage renal failure. Thirty to forty percents of the latter are associated with mutations of genes coding for podocyte proteins. The rest is due to one or several different circulating factors. New strategies are in development to antagonize the effect of the latter.

  3. Nephrotic syndrome complicated with portal, splenic, and superior mesenteric vein thrombosis.

    Science.gov (United States)

    Park, Bong Soo; Park, Sihyung; Jin, Kyubok; Choi, Gibok; Park, Kang Min; Jo, Kyeong Min; Kim, Yang Wook

    2014-09-01

    Thromboembolism is a major complication of nephrotic syndrome. Renal vein thrombosis and deep vein thrombosis are relatively common, especially in membranous nephropathy. However, the incidence of portal vein and superior mesenteric vein (SMV) thrombosis in patients with nephrotic syndrome is very rare. To date, several cases of portal vein thrombosis treated by anticoagulation therapy, not by thrombolytic therapy, have been reported as a complication of nephrotic syndrome. Here, we report a case of portal, splenic, and SMV thrombosis in a patient with a relapsed steroid dependent minimal change disease who was treated successfully with anticoagulation and thrombolytic therapy using urokinase. Radiologic findings and his clinical conditions gradually improved. Six months later, a complete remission of the nephrotic syndrome was observed and the follow-up computed tomography scan showed the disappearance of all portal vein, splenic vein, and SMV thrombi.

  4. Nephrotic syndrome complicated with portal, splenic, and superior mesenteric vein thrombosis

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    Bong Soo Park

    2014-09-01

    Full Text Available Thromboembolism is a major complication of nephrotic syndrome. Renal vein thrombosis and deep vein thrombosis are relatively common, especially in membranous nephropathy. However, the incidence of portal vein and superior mesenteric vein (SMV thrombosis in patients with nephrotic syndrome is very rare. To date, several cases of portal vein thrombosis treated by anticoagulation therapy, not by thrombolytic therapy, have been reported as a complication of nephrotic syndrome. Here, we report a case of portal, splenic, and SMV thrombosis in a patient with a relapsed steroid dependent minimal change disease who was treated successfully with anticoagulation and thrombolytic therapy using urokinase. Radiologic findings and his clinical conditions gradually improved. Six months later, a complete remission of the nephrotic syndrome was observed and the follow-up computed tomography scan showed the disappearance of all portal vein, splenic vein, and SMV thrombi.

  5. Rare case of nephrotic syndrome: Schimke syndrome.

    Science.gov (United States)

    Pedrosa, Anna Kelly Krislane de Vasconcelos; Torres, Luiz Fernando Oliveira; Silva, Ana Corina Brainer Amorim da; Dantas, Adrianna Barros Leal; Zuntini, Káthia Liliane da Cunha Ribeiro; Aguiar, Lia Cordeiro Bastos

    2016-01-01

    Schimke syndrome corresponds to dysplasia of bone and immunity, associated with progressive renal disease secondary to nephrotic syndrome cortico-resistant, with possible other abnormalities such as hypothyroidism and blond marrow aplasia. It is a rare genetic disorder, with few reports in the literature. The most frequent renal involvement is nephrotic syndrome with focal segmental glomerulosclerosis and progressive renal failure. The objective of this study was to report a case of Schimke syndrome, diagnostic investigation and management of the case. Resumo A síndrome Schimke corresponde à displasia imuno-óssea, associada à doença renal progressiva secundária à síndrome nefrótica córtico-resistente, podendo haver outras anormalidades como hipotireoidismo e aplasia de medula óssea. Trata-se de uma patologia genética rara, com poucos relatos na literatura. O acometimento renal mais frequente é uma síndrome nefrótica por glomeruloesclerose segmentar e focal e falência renal progressiva. O objetivo deste estudo foi relatar um caso de síndrome de Schimke, investigação diagnóstica e condução do caso.

  6. Nephrotic Syndrome and Acute Renal Failure Apparently Induced by Sunitinib

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    Ying-Shou Chen

    2009-10-01

    Full Text Available We report a case of nephrotic syndrome and acute renal failure apparently induced by sunitinib. A 67-year-old man with a history of metastatic renal cell carcinoma presented with progressive kidney dysfunction with proteinuria, general edema, and body weight gain of 21 kg after undergoing 3 weeks of sunitinib therapy. The patient had taken no other over-the-counter medications, and all other possible causes of nephrotic syndrome were excluded. The Naranjo Adverse Drug Reaction Probability Scale score for this event was 6, indicating a high probability that the observed presentations were associated with use of the drug. However, despite the discontinuation of sunitinib, his condition deteriorated, and hemodialysis was initiated for respiratory distress. A renal biopsy was performed, which revealed ischemic acute tubular necrosis with minimal change nephropathy. In conclusion, nephrologists and oncologists should be aware that nephrotic syndrome with ischemic acute tubular necrosis is a possible adverse effect of sunitinib. For early diagnosis of this condition and to avoid renal damage, we recommend differential diagnosis of serum creatinine and proteinuria in patients undergoing sunitinib therapy.

  7. Management of idiopathic nephrotic syndrome in childhood

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    Peco-Antić Amira

    2004-01-01

    Full Text Available The management of idiopathic nephrotic syndrome (INS in children includes immunosuppressive and symptomatic treatment. The response to corticosteroid therapy is the best prognostic marker of the disease. The majority of children with INS (about 85% are steroid-sensitive as they normalize proteinuria within 4 weeks of daily, oral prednisone administration. The most of steroid-sensitive patients (94% has minimal change of nephrotic syndrome, while the majority (80.5%-94.4% of those who are steroid-resistant has focal segmental glomerulosderosis or mesangioproliferative glomerulonephritis. Initial therapy of INS consists of 60 mg/m2/day prednisone daily for 4 weeks followed by 40 mg/m2 on alternate days for 4 weeks, thereafter decreasing alternate day therapy every month by 25% over the next 4 months. Thus, the overall duration of the initial cortico-steroids course is 6 months that may be significantly protective against the future development of frequent relapses. Approximately 30% of patients experience only one attack and are cured after the first course of therapy; 10-20% have only 3 or 4 steroid-responsive episodes before permanent cure; the remaining 40-50% of patients are frequent relapsers, or steroid-dependent. Standard relapse therapy consists of 60 mg/m2/ day prednisone until urine is protein free for at least 3 days, followed by 40 mg/m2 on alternate days for 4 weeks. The treatment of frequent-relapses and steroid-dependent INS includes several different regimens: maintenance (6 months alternate steroid therapy just above steroid threshold (0.1-0.5 mg/kg/ 48h, levamisole, alkylating agents (cyclophosphamide or chlorambucil or cyclosporine. The worse prognosis is expected in steroid-resistant patients who are the most difficult to treat. Renal biopsy should be performed in them. At present, there is no consensus on therapeutic regimen for steroid-resistant patients. The following immunosuppressive drugs have been used with varying

  8. Antithrombin III and the nephrotic syndrome.

    Science.gov (United States)

    Jørgensen, K A; Stoffersen, E

    1979-05-01

    Plasma and urinary antithrombin III (AT-III) was measured in 15 cases of nephrotic syndrome. Plasma AT-III correlated well with serum albumin, but poorly with proteinuria, whereas urinary AT-III correlated well to proteinuria. The plasma AT-III level had a mean similar to 25 healthy controls, but the range was significantly wider. A case with nephrotic syndrome and left renal vein thrombosis is reported. The urinary output of AT-III rose and the plasma level fell with the activity of the disease. Although AT-III and albumin have similar molecule weight, their renal clearance was found to be different. It is suggested that urinary loss of AT-III plays a role in the hypercoagulable state sometimes found in the nephrotic syndrome.

  9. Thromboembolic complications in the nephrotic syndrome: pathophysiology and clinical management.

    Science.gov (United States)

    Singhal, Rajni; Brimble, K Scott

    2006-01-01

    Patients with the nephrotic syndrome are at increased risk of developing venous and arterial thromboembolism, the most common of which is renal vein thrombosis. There are several unanswered or controversial issues relating to the nephrotic syndrome and thromboembolism, which include the mechanism of thromboembolism, and optimal diagnostic and anticoagulant management strategies. This review will discuss several of these issues: the epidemiology and clinical spectrum of thromboembolic disease occurring in patients with the nephrotic syndrome; the pathophysiology of the hypercoagulable state associated with the nephrotic syndrome; the diagnosis of renal vein thrombosis in the nephrotic syndrome; and the evidence for prophylactic and therapeutic anticoagulation strategies in such patients.

  10. Glucocorticoid pharmacogenetics in pediatric idiopathic nephrotic syndrome.

    Science.gov (United States)

    Cuzzoni, Eva; De Iudicibus, Sara; Franca, Raffaella; Stocco, Gabriele; Lucafò, Marianna; Pelin, Marco; Favretto, Diego; Pasini, Andrea; Montini, Giovanni; Decorti, Giuliana

    2015-01-01

    Idiopathic nephrotic syndrome represents the most common type of primary glomerular disease in children: glucocorticoids (GCs) are the first-line therapy, even if considerable interindividual differences in their efficacy and side effects have been reported. Immunosuppressive and anti-inflammatory effects of these drugs are mainly due to the GC-mediated transcription regulation of pro- and anti-inflammatory genes. This mechanism of action is the result of a complex multistep pathway that involves the glucocorticoid receptor and several other proteins, encoded by polymorphic genes. Aim of this review is to highlight the current knowledge on genetic variants that could affect GC response, particularly focusing on children with idiopathic nephrotic syndrome.

  11. Medullary nephrocalcinosis, distal renal tubular acidosis and polycythaemia in a patient with nephrotic syndrome

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    Karunarathne Suneth

    2012-07-01

    Full Text Available Abstract Background Medullary nephrocalcinosis and distal renal tubular acidosis are closely associated and each can lead to the other. These clinical entities are rare in patients with nephrotic syndrome and polycythaemia is an unusual finding in such patients. We describe the presence of medullary nephrocalcinosis, distal renal tubular acidosis and polycythaemia in a patient with nephrotic syndrome due to minimal change disease. Proposed mechanisms of polycythaemia in patients with nephrotic syndrome and distal renal tubular acidosis include, increased erythropoietin production and secretion of interleukin 8 which in turn stimulate erythropoiesis. Case presentation A 22 year old Sri Lankan Sinhala male with nephrotic syndrome due to minimal change disease was investigated for incidentally detected polycythaemia. Investigations revealed the presence of renal tubular acidosis type I and medullary nephrocalcinosis. Despite extensive investigation, a definite cause for polycythaemia was not found in this patient. Treatment with potassium and bicarbonate supplementation with potassium citrate led to correction of acidosis thereby avoiding the progression of nephrocalcinosis and harmful effects of chronic acidosis. Conclusion The constellation of clinical and biochemical findings in this patient is unique but the pathogenesis of erythrocytosis is not clearly explained. The proposed mechanisms for erythrocytosis in other patients with proteinuria include increased erythropoietin secretion due to renal hypoxia and increased secretion of interleukin 8 from the kidney. This case illustrates that there may exist hitherto unknown connections between tubular and glomerular dysfunction in patients with nephrotic syndrome.

  12. A PROSPECTIVE STUDY OF NEPHROTIC SYNDROME IN CHILDREN

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    Vijayalakshmi

    2015-05-01

    Full Text Available AIM OF THE STUDY: To study the incidence, age and sex related demographics in children with Nephrotic Syndrome (NS and to find the aetiology in atypical cases of NS by renal biopsy. MATERIALS AND METHOD S: The present study was a single centre, descriptive, prospective and observational cross sectional study. All patients who were admitted in the department of paediatrics with Nephrotic syndrome during the study period of 2 years from July 2010 to June 2012 were included. The demographics and renal biopsy results were analys ed further. RESULTS : Total number of cases of NS was 44 against total Paediatric admissions of 4827. Incidence of NS was 0.91% (44/4827 among all paediatric admissions. Male to female ratio was 2.14:1. Majority of NS cases were 3 - 5 years old (36.36%, n=44 , followed by 5 - 7 years (29.55%, n=44, 7 - 9 years (18.18%, n=44 and 1 - 3 years (15.91%, n=44. Total number of renal biopsies done was 18, out of which 14 (77.78%, n=18 had minimal change disease (MCNS and 3 (16.67%, n=18 had Focal Segmental Glomerulos clerosis (FSGS and 1 (5.55%, n=18 had anti Glomerular Basement Membrane disease. CONCLUSION: The incidence of Nephrotic syndrome in children was 0.91% (44/4827 among total paediatric admissions, had male preponderance with ratio of males to females of 2 .14:1, most commonly affecting children of 3 - 5 years of age and the most common aetiology being Minimal Change Disease (MCD.

  13. Genetic testing in nephrotic syndrome--challenges and opportunities.

    Science.gov (United States)

    Gbadegesin, Rasheed A; Winn, Michelle P; Smoyer, William E

    2013-03-01

    Monogenic nephrotic syndrome (nephrotic syndrome caused by a single gene defect) is responsible for only a small percentage of cases of nephrotic syndrome, but information from studies of the unique cohort of patients with this form of the disease has dramatically improved our understanding of the disease pathogenesis. The use of genetic testing in the management of children and adults with nephrotic syndrome poses unique challenges for clinicians in terms of who to test and how to use the information obtained from testing in the clinical setting. In our view, not enough data exist at present to justify the routine genetic testing of all patients with nephrotic syndrome. Testing is warranted, however, in patients with congenital nephrotic syndrome (onset at 0-3 months), infantile nephrotic syndrome (onset at 3-12 months), a family history of nephrotic syndrome, and those in whom nephrotic syndrome is associated with other congenital malformations. The family and/or the patient should be given complete and unbiased information on the potential benefits and risks associated with therapy, including the reported outcomes of treatment in patients with similar mutations. Based on the data available in the literature so far, intensive immunosuppressive treatment is probably not indicated in monogenic nephrotic syndrome if complete or partial remission has not been achieved within 6 weeks of starting treatment. We advocate that family members of individuals with genetic forms of nephrotic syndrome undergo routine genetic testing prior to living-related kidney transplantation. Prospective, multicentre studies are needed to more completely determine the burden of disease caused by monogenic nephrotic syndrome, and randomized controlled trials are needed to clarify the presence or absence of clinical responses of monogenic nephrotic syndrome to available therapies.

  14. 吗替麦考酚酯联合低剂量激素治疗HBsAg阳性的成人微小病变性肾病综合征%Mycophenolate mofetil combined with low dose prednisone in the treatment for adults with minimal change nephrotic syndrome and concomitant HBsAg positive

    Institute of Scientific and Technical Information of China (English)

    李夏玉; 田炯; 陈江华; 李恒; 何强; 林维勤; 倪勤; 贺学林; 吴建永

    2009-01-01

    Objective To assess the safety and efficacy of mycophenolate mofetil (MMF) combined with low dose corticosteroid in the treatment of adults with minimal change nephrotic syndrome and concomitant HBsAg positive (MCNS-HBsAg). Methods Thirty adults with MCNS-HBsAg were enrolled in this prospective study and were assigned to two groups. The MMF group (n=14) received low dose of prednisone combined with MMF (MMF 1.0 to 2.0 g/d patients of Pred group versus 35.7% patients of MMF group. 43.8% patients of Pred group versus 21.4% patients of MMF group received lamivudine therapy. Elevation of alanine aminotransferase(ALT) ocurred in 50% patients of Pred group and 28.6% patients of MMF group. The complete remission (CR) rate after 24 weeks treatment was 11/14 in Pred group versus 10/12 in MMF group. 6/11 patients of the Pred group and 4/10 patients of the MMF group who achieved CR experienced relapses during follow-up. Conclusions Use of MMF combined with low dose prednisone is as effective as conventional prednisone regimen in treating adults with MCNS-HBsAg. The MMF protocol seems to be superior in HBV reactivation to conventional prednisone protocol.%目的 探讨吗替麦考酚酯(MMF)联合低剂量糖皮质激素方案治疗HBsAg阳性的成人微小病变性.肾病综合征的疗效及安全性.方法 前瞻性地选择HBsAg阳性、HBeAg阴性及血清HBV-DNA<1000拷贝/ml的成人微小病变性肾病综合征患者30例,分成激素组(16例)及MMF组(14例).激素组接受常规激素治疗方案(泼尼松片,1 mg·kg-1·d-1);MMF组接受低剂量激素(泼尼松片,0.5 mg·kg-1·d-1)联合MMF 1.0~2.0 g/d.结果 激素组和MMF组乙肝病毒激活发生比例分别为62.5%及35.7%,其中接受拉米呋定治疗分别为43.8%及21.4%;谷丙转氨酶升高发生比例分别为50.0%及28.6%.激素组及MMF组的完全缓解比例分别为11/14和10/12,两组复发比例分别为6/11和4/10.结论 与常规激素治疗方案比较,MMF联合低剂量糖皮质激素

  15. Late de novo minimal change disease in a renal allograft

    OpenAIRE

    Madhan Krishan; Temple-Camp Cynric

    2009-01-01

    Among the causes of the nephrotic syndrome in renal allografts, minimal change disease is a rarity with only few cases described in the medical literature. Most cases described have occurred early in the post-transplant course. There is no established treatment for the condition but prognosis is favorable. We describe a case of minimal change disease that developed 8 years after a successful transplantation of a renal allograft in a middle-aged woman. The nephrotic syndrome was accompanied by...

  16. Nephrotic syndrome and Guillan-Barré Syndrome: a rare association in child.

    Science.gov (United States)

    Bouyahia, Olfa; Khelifi, Ibtissem; Gharsallah, Lamia; Harzallah, Kais; Mrad, Sonia Mazigh; Ghargah, Tahar; Boukthir, Samir; Azza, Sammoud El Gharbi

    2010-01-01

    Only few cases of nephrotic syndrome associated with Guillain-Barre Syndrome (GBS) have been reported in the adult and pediatric literature. A 3-year-old boy was initially admitted to our hospital following five days of progressive weakness of his extremities, fatigue, right leg pain and numbness. There was no past history of renal or neurological disease. Cerebro-spinal fluid studies showed a protein level of 92 mg/dL and a white cell count of 1 per high-power field. The diagnosis of GBS was verified with a nerve conduction velocity test as well as. The GBS symptoms improved gradually on intravenous immunoglobulin. Three weeks later, he developed severe proteinuria and edema; laboratory investigation showed nephrotic syndrome which responded to steroid therapy. Renal biopsy showed minimal change glomerulonephritis. He remained free of proteinuria during his 20 months of follow-up.

  17. Nephrotic syndrome and Guillan-barre syndrome: A rare association in child

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    Bouyahia Olfa

    2010-01-01

    Full Text Available Only few cases of nephrotic syndrome associated with Guillain-Barre Syndrome (GBS have been reported in the adult and pediatric literature. A 3-year-old boy was initially admitted to our hospital following five days of progressive weakness of his extremities, fatigue, right leg pain and numbness. There was no past history of renal or neurological disease. Cerebro-spinal fluid studies showed a protein level of 92 mg/dL and a white cell count of 1 per high-power field. The diagnosis of GBS was verified with a nerve conduction velocity test as well as. The GBS symptoms improved gradually on intravenous immunoglobulin. Three weeks later, he deve-loped severe proteinuria and edema; laboratory investigation showed nephrotic syndrome which responded to steroid therapy. Renal biopsy showed minimal change glomerulonephritis. He re-mained free of proteinuria during his 20 months of follow-up.

  18. Two cases of nephrotic syndrome with different etiologies

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    Sanjay K Mandal

    2013-01-01

    Full Text Available There are various causes of secondary nephrotic syndrome. Finding an underlying etiology in a case of nephrotic syndrome or subnephrotic range proteinuria can markedly alter the therapeutic options and disease course. We describe two cases of secondary nephrotic syndrome. The first case was a 22-year-old male with pulmonary tuberculosis with nephrotic syndrome secondary to renal amyloidosis, whereas the second case was a 17-year-old male with chronic hepatitis B-associated nephrotic syndrome. It is important, especially in developing countries, to be aware that tuberculosis and infections like hepatitis B, C, etc. continue to be part of the differential diagnosis of secondary nephrotic syndrome in adolescents and young adults.

  19. Long-term Outcomes of Childhood Onset Nephrotic Syndrome

    OpenAIRE

    Hjorten, Rebecca; Anwar, Zohra; Reidy, Kimberly Jean

    2016-01-01

    There are limited studies on long-term outcomes of childhood onset nephrotic syndrome (NS). A majority of children with NS have steroid-sensitive nephrotic syndrome (SSNS). Steroid-resistant nephrotic syndrome (SRNS) is associated with a high risk of developing end-stage renal disease. Biomarkers and analysis of genetic mutations may provide new information for prognosis in SRNS. Frequently relapsing and steroid-dependent NS is associated with long-term complications, including dyslipidemia, ...

  20. Abdominal aortic thrombosis in a patient with nephrotic syndrome.

    Science.gov (United States)

    Nakamura, M; Ohnishi, T; Okamoto, S; Yamakado, T; Isaka, N; Nakano, T

    1998-01-01

    We report a patient who presented with severe nephrotic syndrome complicated with infrarenal aortic and right renal arterial thrombosis. The nephrotic syndrome frequently causes thromboembolic complications in veins, but arterial thrombosis is relatively rare, especially in the aorta. Various predisposing factors leading to thromboembolic complications are discussed. In this case, the thromboembolic complication may have some clinical association with the hypercoagulable state in nephrotic syndrome.

  1. Diuretics and its use in nephrotic syndrome

    OpenAIRE

    2012-01-01

    Nephrotic syndrome is a clinical condition characterized by an urinary massive lossof proteins, specially albumin, which leads to the development of hypoalbuminemiaand secondary edema. Other alterations, like dyslipidemia, accompany to thesemanifestations in a compensatory way to the loss of oncotic pressure, but thesemanifestations aren´t part of the definition. The treatment is based on the use ofcorticoids and cytotoxic drugs, however other measures for the control of edema, as theuse of d...

  2. Nephrotic Syndrome and The TCM Treatment

    Institute of Scientific and Technical Information of China (English)

    王巍; 王淑琴

    2004-01-01

    @@ Nephrotic syndrome is a symptom complex characterized by severe albuminuria (+++ to ++++ in qualitative test and > 3.5 g/L in quantitative test),hypoproteinemia (< 3 g/L), edema and hyperlipemia(apparently increased plasma cholesterol and triglyceride and increased low density lipoprotein,LDL) due to abnormally increased permeability of glomerular capillary wall against plasma proteins.There may be lipoiduria with normal or abnormal level of high density lipoprotein (HDL).

  3. Profound nephrotic syndrome in a patient with ovarian teratoma

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    Abdallah Jeroudi

    2013-01-01

    Full Text Available The nephrotic syndrome (NS has been associated with a variety of malignancies in a number of reports in the literature, but has been reported in only nine cases associated with ovarian neoplasms. Membranous nephropathy is the most common glomerular pathology causing the NS in patients with solid tumors. There has been only one report of an ovarian neoplasm associated with minimal change disease (MCD. We describe the case of a 36-year-old woman who presented with the NS secondary to biopsy-proven MCD, likely secondary to mature ovarian teratoma. Treatment by tumor removal and prednisone led to remission of the NS. To the best of our knowledge, this is the first report of an ovarian teratoma and the second report of an ovarian neoplasm associated with MCD.

  4. Plasma Histamine And Serotonin Levels In Children With Nephrotic Syndrome And Acute Poststreptococcal Glomerulonephritis

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    Nagwa Mohamed and Talaat El sayed

    2005-12-01

    Full Text Available Plasma histamine and serotonin concentrations were measured using fluorimeteric assay in 40 children with renal diseases. Minimal change nephrotic syndrome (15 focal segmental glomerulosclerosis(10 and acute poststreptococcal glomerulonephritis(15 to determine the relation between plasma levels of histamine and serotonin and these various types of renal diseases in children. Plasma histamine level was significantly increased in group of children with acute poststreptococcal glomerulonephritis. Plasma serotonin levels were significantly increased in all 3 groups of patient, when compared with those of controls. Raised plasma histamine in acute poststreptococcal glomerulonephritis group may be evidence of the acute immunological inflammation and defective renal excretion due to mild renal impairment in these children. Raised plasma serotonin in all 3 groups of patients may be due to diminished uptake and release of serotonin from platelets in children with minimal change nephrotic syndrome and focal segmental glomerulosclerosis and due to defective renal execretion in children with acute poststreptococcal glomerulo-nephritis.

  5. CLINICAL, BIOCHEMICAL AND HISTOPATHOLOGICAL PROFILE OF ADULT NEPHROTIC SYNDROME PATIENTS IN A TERTIARY CARE HOSPITAL

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    Krishnamoorthy

    2015-09-01

    Full Text Available BACKGROUND: The nephrotic syndrome is recognized as an independent entity of renal disease for over half a century . 1 Causes of nephrotic syndrome varies with age, time period, geographical location and race. In children, minimal change nephrotic syndrome is the commonest 2 ; however, membranous nephropathy is most frequent in adults . 3 As it commonly affects the younger age group and is associated with high morbidity and mortality, there is a need to understand and diagnose the disease at an early stage. Hence, this study has been done to identify the clinical presentation, biochemical parameters and histopathology associated with nephrotic syndrome in adults and its subtypes. OBJECTIVE: To study the clinical, biochemical and histopathological profile of patients with Adult Nephrotic Syndrome admitted in our tertiary care hospital. METHODS: Prospective study of 100 patients with Adult Nephrotic Syndrome admitt ed in our tertiary care hospital were screened with facial puffiness and pedal edema. They were tested for urine proteinuria, urine protein creatinine ratio or 24 hour urine protein estimation. Later renal biopsy was done for all patients to stratify the subtypes. RESULTS: In this study, males were predominantly affected. Most common presenting complaints were facial puffiness and pedal edema. Systolic BP was increased in 96% of patients and diastolic BP was elevated in 50% of patients. Serum LDL and TGL were elevated in nephrotic syndrome. In young patients less than 40 years Focal Segmental Glomerulosclerosis (FSGS is the commonest type, then Membrano Proliferative Glomerulo Nephritis (MPGN and Minimal Change Disease (MCD. In individuals more than 40 years, membranous nephropathy was predominantly seen followed by FSGS. CONCLUSION: There is a changing trend in primary nephrotic syndrome and FSGS was found to be the commonest subtype. Male preponderance was noticed and also FSGS was found to be more common in younger adults. Most

  6. Insulin-like growth factor-I and insulin-like growth factor-binding proteins in the nephrotic syndrome.

    Science.gov (United States)

    Feld, S M; Hirschberg, R

    1996-06-01

    Similar to findings in the nephrotic syndrome in humans, rats with the doxorubicin-induced nephrotic syndrome (which resembles minimal change disease) have reduced serum levels of insulin-like growth factor-I (IGF-I). This is mainly caused by glomerular ultrafiltration of IGF-I-containing binding protein complexes, primarily of a molecular weight of approximately 50 kilodaltons, and urinary losses of the peptide. Despite urinary excretion of IGF-binding protein (IGFBP)-2, serum levels are increased more than twofold in the nephrotic syndrome compared with controls, because of increased synthesis of this binding protein by the liver. In contrast, the liver synthesis of IGFBP-3, the predominant binding protein in normal serum, is unchanged in the nephrotic syndrome. However, binding and serum levels of IGFBP-3 are reduced in nephrotic rat serum, apparently due to proteolytic degradation of IGFBP-3. The glomerular ultrafiltration of IGF-I, which leads to biologically significant IGF-I concentrations of about 1.35 nM in proximal tubule fluid, may have metabolic consequences, such as increased tubular phosphate absorption. Hypothetically, tubule fluid IGF-I may also contribute to progressive tubulointerstitial fibrosis which is sometimes present in protractive nephrotic glomerulopathies. The profound changes in the IGF-I/IGFBP system in the nephrotic syndrome may also contribute to systemic metabolic abnormalities and growth failure.

  7. Treatment of anemia of nephrotic syndrome with recombinant erythropoietin

    NARCIS (Netherlands)

    Gansevoort, RT; Vaziri, ND; deJong, PE

    Nephrotic syndrome has been recently shown to cause erythropoietin (EPO) deficiency in humans and experimental models. However, efficacy and safety of recombinant EPO (rEPO) in the treatment of the associated anemia has not been previously investigated. We report a patient with nephrotic syndrome

  8. Treatment of anemia of nephrotic syndrome with recombinant erythropoietin

    NARCIS (Netherlands)

    Gansevoort, RT; Vaziri, ND; deJong, PE

    1996-01-01

    Nephrotic syndrome has been recently shown to cause erythropoietin (EPO) deficiency in humans and experimental models. However, efficacy and safety of recombinant EPO (rEPO) in the treatment of the associated anemia has not been previously investigated. We report a patient with nephrotic syndrome an

  9. Clarithromycin-induced acute interstitial nephritis and minimal change disease.

    Science.gov (United States)

    Russell, Wendy; Smith, William

    2009-10-01

    Drug associated acute interstitial nephritis and minimal change disease has been well documented but the simultaneous presentation of both is rare and has not been reported with clarithromycin. We describe a case of simultaneous acute tubulointerstitial nephritis and minimal change disease induced by clarithromycin. The patient had acute kidney injury, nephrotic syndrome, eosinophilic pneumonitis and a maculopapular skin rash. The role of steroid therapy in acute interstitial nephritis is controversial but is accepted as beneficial in minimal change nephrotic syndrome. Steroid therapy in our patient resulted in complete clinical resolution.

  10. Restless Legs Syndrome in Pediatric Patients With Nephrotic Syndrome

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    Victoria Cheung BA

    2015-05-01

    Full Text Available Background. Restless legs syndrome (RLS is a sleep disorder characterized by an urge to move or the presence of unpleasant sensations in the extremities. The prevalence of RLS is higher in children and adults with chronic kidney disease and in adults with glomerular disease. Objective. To determine the prevalence of RLS in children with nephrotic syndrome. Methods. We studied 50 children with nephrotic syndrome and 22 controls. The following surveys were administered: Pediatric Emory RLS questionnaire, Pediatric Daytime Sleepiness Scale, and Pediatric Sleep Questionnaire. Results. Children with nephrotic syndrome were 9.0 ± 4.4 years old, 27 were male, and 27 were in remission. The prevalence of RLS was similar in the nephrotic syndrome cases and controls, whether or not indeterminate cases were considered positive: 14.0% versus 13.6% including indeterminate cases, and 8.0% versus 9.1% excluding indeterminate cases. Conclusion. RLS is not more common in children with glomerular disease compared to healthy controls.

  11. [Thrombotic complications in the nephrotic syndrome].

    Science.gov (United States)

    Keusch, G

    1989-08-01

    Thromboembolic episodes are one of the most serious complications in patients with nephrotic syndrome, with an overall incidence of 25%. The most frequent site of thrombosis is the renal vein, with a reported incidence varying from 2-42%. Arterial thromboses are much less common than venous thromboses, with an overall incidence of 3%. Clinical course of renal vein thrombosis may be acute or chronic. Renal venography is the method of choice in its diagnosis. Duplex scanning, computed tomography and magnetic resonance imaging may be as accurate as venography. Once the diagnosis of renal vein thrombosis is established, anticoagulation therapy should be started. Thrombectomy or thrombolytic therapy seem to have little to offer over oral anticoagulation. The increased incidence of thrombotic complications in nephrotic syndrome may be due to a hypercoagulable state distinguished by an increase in coagulation factors (V, VIII, X and fibrinogen); a decrease in the levels of coagulation inhibitors (antithrombin III, protein S); an increase in alpha 2-antiplasmin activity; and exaggerated platelet adhesiveness and aggregation. This pre-thrombotic state may be aggravated by additional rheological factors (immobilization, diuretic therapy etc.). Serum albumin has been found to be an appropriate parameter to assess the risk of thrombosis development in these patients. A serum albumin level below 20 g/l carries a high risk of thromboembolic complications. Prophylactic anticoagulation therapy is therefore indicated in patients with serum albumin below 20 g/l.

  12. Calcium and Vitamin D Metabolism in Pediatric Nephrotic Syndrome; An Update on the Existing Literature

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    Mohammad Esmaeeili

    2015-03-01

    Full Text Available  Minimal Change Disease (MCD is the leading cause of childhood Nephrotic Syndrome (NS. Therefore in pediatrics nephrotic syndrome, most children beyond the first year of life will be treated with corticosteroids without an initial biopsy. Children with NS often display a number of calcium homeostasis disturbances causing abnormal bone histology, including hypocalcemia, reduced serum vitamin D metabolites, impaired intestinal absorption of calcium, and elevated levels of immunoreactive parathyroid hormone (iPTH. These are mainly attributed to the loss of a variety of plasma proteins and minerals in the urine as well as steroid therapy. Early diagnosis and management of these abnormalities, could prevent the growth retardation and renal osteodystrophy that affects children with nephrotic syndrome. Here we reviewed the literature for changes of calcium and vitamin D metabolism in nephrotic syndrome and its consequences on bones, also the effect of corticosteroid and possible preventive strategies that could be done to avoid long term outcomes in children. Although the exact biochemical basis for Changes in levels of calcium and vitamin D metabolites in patients with NS remains speculative; Because of the potential adverse effects of these changes among growing children, widespread screening for vitamin D deficiency or routine vitamin D supplementation should be considered.

  13. Chryseobacterium gleum pneumonia in an infant with nephrotic syndrome

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    Baha Abdalhamid

    2016-01-01

    Conclusion: To the best of our knowledge, this is the first case of pneumonia caused by Chryseobacterium gleum in an infant with nephrotic syndrome. It is also the first report of C. gleum causing respiratory tract infection in Saudi Arabia.

  14. New insights into lipid metabolism in the nephrotic syndrome

    NARCIS (Netherlands)

    Kaysen, GA; de Sain-van der Velden, MGM

    1999-01-01

    Hyperlipidemia in the nephrotic syndrome results from increased synthesis and decreased catabolism of lipoproteins. The contribution of each to establishing blood lipid levels is unknown. Increased triglyceride rich lipoprotein concentration, very low density lipoprotein (VLDL) and intermediate dens

  15. Secondary Syphilis With Hepatitis and Nephrotic Syndrome: A Rare Concurrence.

    Science.gov (United States)

    Makker, Jasbir; Bajantri, Bharat; Nayudu, Suresh Kumar

    2016-07-01

    Syphilis, a chronic multisystem disease, is caused by a spirochete, Treponema pallidum. Clinical presentation may expand to several stages including primary, secondary and latent syphilis, which may present as early or late syphilis. Nephrotic syndrome and acute hepatitis are well-known complications of secondary syphilis. To the best of our knowledge, secondary syphilis with coexisting renal and hepatic complications has rarely been reported. Here we present a rare case of concurrent nephrotic syndrome and acute hepatitis in a patient with secondary syphilis.

  16. Bone Metabolism of Children with Idiopathic Nephrotic Syndrome

    OpenAIRE

    渡辺, 繁子; Watanabe, Shigeko

    1986-01-01

    Children with nephrotic syndrome are frequently at risk for osteodystrophy due to secondary hyperparathyroidism. Severe proteinuria is accompanied by loss of 25-OH-Vit. D3, which causes hyperparathyroidism following hypocalcemia. On the other hand, administred glucocorticoid suppresses protein synthesis in osteoblasts to cause osteoporosis, and disturbs absorption of calcium in the intestine, which also leads to hyperparathyroidism. In this study 66 children with nephrotic syndrome were exami...

  17. Glomerular Glucocorticoid Receptors Expression and Clinicopathological Types of Childhood Nephrotic Syndrome.

    Science.gov (United States)

    Gamal, Yasser; Badawy, Ahlam; Swelam, Salwa; Tawfeek, Mostafa S K; Gad, Eman Fathalla

    2017-02-01

    Glucocorticoids are primary therapy of idiopathic nephrotic syndrome (INS). However, not all children respond to steroid therapy. We assessed glomerular glucocorticoid receptor expression in fifty-one children with INS and its relation to response to steroid therapy and to histopathological type. Clinical, laboratory and glomerular expression of glucocorticoid receptors were compared between groups with different steroid response. Glomerular glucocorticoid expression was slightly higher in controls than in minimal change early responders, which in turn was significantly higher than in minimal change late responders. There was significantly lower glomerular glucocorticoid receptor expression in steroid-resistance compared to early responders, late responders and controls. Glomerular glucocorticoid expression was significantly higher in all minimal change disease (MCD) compared to focal segmental glomerulosclerosis. In INS, response to glucocorticoid is dependent on glomerular expression of receptors and peripheral expression. Evaluation of glomerular glucocorticoid receptor expression at time of diagnosis of NS can predict response to steroid therapy.

  18. D-Pencillamine induced nephrotic syndrome in 11-year old girl with wilson disease: A case report

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    Dr. Anil Kumar*

    2013-12-01

    Full Text Available D-pencillamine and zinc remains the first line of treatment for Wilson’s disease in India. Membranous glomerulopathy is most commonly associated with nephrotic syndrome secondary to d penicillamine but isolated cases of minimal change lesions are rarely reported.We report a pediatric patient with Wilson’s disease who developed nephrotic syndrome 9 months after starting D-pencillamine. After stopping D-pencillamine and with only zinc for maintanence, her proteinuria resolved within a week’s time with full dose of steroids for nephrotic syndrome.Wilson disease itself may have tubular dysfunction but glomerulopathy is rare Isolated minimal change disease can occur in a 11 – year old patient yet it is statistically more likely to occur in a much younger age group.The most likely cause of nephrotic syndrome in this child is due to the late complication of D-penicillamine. It also re – emphasizes the importance of early monitoring for proteinuria and the need to shift to an alternative agent if side effects develop

  19. Pathophysiology, Evaluation, and Management of Edema in Childhood Nephrotic Syndrome

    Science.gov (United States)

    Ellis, Demetrius

    2016-01-01

    Generalized edema is a major presenting clinical feature of children with nephrotic syndrome (NS) exemplified by such primary conditions as minimal change disease (MCD). In these children with classical NS and marked proteinuria and hypoalbuminemia, the ensuing tendency to hypovolemia triggers compensatory physiological mechanisms, which enhance renal sodium (Na+) and water retention; this is known as the “underfill hypothesis.” Edema can also occur in secondary forms of NS and several other glomerulonephritides, in which the degree of proteinuria and hypoalbuminemia, are variable. In contrast to MCD, in these latter conditions, the predominant mechanism of edema formation is “primary” or “pathophysiological,” Na+ and water retention; this is known as the “overfill hypothesis.” A major clinical challenge in children with these disorders is to distinguish the predominant mechanism of edema formation, identify other potential contributing factors, and prevent the deleterious effects of diuretic regimens in those with unsuspected reduced effective circulatory volume (i.e., underfill). This article reviews the Starling forces that become altered in NS so as to tip the balance of fluid movement in favor of edema formation. An understanding of these pathomechanisms then serves to formulate a more rational approach to prevention, evaluation, and management of such edema. PMID:26793696

  20. Late de novo minimal change disease in a renal allograft

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    Madhan Krishan

    2009-01-01

    Full Text Available Among the causes of the nephrotic syndrome in renal allografts, minimal change disease is a rarity with only few cases described in the medical literature. Most cases described have occurred early in the post-transplant course. There is no established treatment for the condition but prognosis is favorable. We describe a case of minimal change disease that developed 8 years after a successful transplantation of a renal allograft in a middle-aged woman. The nephrotic syndrome was accompanied by deterioration of allograft function. Treatment with mycophenolate mofetil was successful in inducing remission and stabilizing allograft function.

  1. Late de novo minimal change disease in a renal allograft.

    Science.gov (United States)

    Madhan, Krishan K; Temple-Camp, Cynric R E

    2009-03-01

    Among the causes of the nephrotic syndrome in renal allografts, minimal change disease is a rarity with only few cases described in the medical literature. Most cases described have occurred early in the post-transplant course. There is no established treatment for the condition but prognosis is favorable. We describe a case of minimal change disease that developed 8 years after a successful transplantation of a renal allograft in a middle-aged woman. The nephrotic syndrome was accompanied by deterioration of allograft function. Treatment with mycophenolate mofetil was successful in inducing remission and stabilizing allograft function.

  2. Splenic hypofunction in the nephrotic syndrome of childhood

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    McVicar, M.I.; Chandra, M.; Margouleff, D.; Zanzi, I.

    1986-05-01

    The reticuloendothelial system, including the spleen, subserves important immunologic functions. Loss of splenic function results in an increased incidence of severe bacterial infections and is accompanied by thrombocytosis. Several nephrotic children were noted to have remarkably high platelet counts and predisposition to bacterial infection with encapsulated organisms. We, therefore, investigated the splenic function of nine children with primary nephrotic syndrome and measured the phagocytic function of the spleen by sequestration of Technetium-99-labelled heat-treated autologous RBC, administered intravenously. Four children had decreased splenic function. Repeat studies performed in two of these children after remission of the nephrotic syndrome gave normal results. There were six episodes of bacterial infection (3 peritonitis, 1 septic arthritis, 1 cellulitis, and 1 Escherichia coli urinary tract infection) among the four patients with decreased splenic function. There were no episodes of bacterial infection among the five nephrotic children with normal splenic function. Nephrotic patients with decreased splenic function had significantly increased platelet counts (921,000 +/- 196,000; mean +/- SEM) compared to those with normal function (435,000 +/- 46,000; P less than 0.001). Our findings suggest the possibility that some nephrotic children may have decreased splenic function in association with increased susceptibility to bacterial infections.

  3. A case of minimal change disease in a Fabry patient.

    Science.gov (United States)

    Zarate, Yuri A; Patterson, Larry; Yin, Hong; Hopkin, Robert J

    2010-03-01

    Fabry disease is an X-linked lysosomal storage disorder caused by mutations of the GLA gene and deficiency in alpha-galactosidase A activity. Glycosphingolipids accumulation causes renal injury that manifests early during childhood as tubular dysfunction and later in adulthood as proteinuria and renal insufficiency. Nephrotic syndrome as the first evidence of Fabry-related kidney damage is rare. We report the case of a teenager with known Fabry disease and normal renal function who developed acute nephrotic syndrome. He was found to have typical glycosphingolipids accumulation with no other findings suggestive of alternative causes of nephrotic syndrome on kidney biopsy. After treatment with enzyme replacement therapy and oral steroids, he went into complete remission from nephrotic syndrome, a response that is atypical for Fabry disease patients who develop heavy proteinuria as a result of longstanding disease and chronic renal injury. The nephrotic syndrome in this patient appears to have developed secondary to minimal change disease. We recommend considering immunotherapy in addition to enzyme replacement therapy in those patients with confirmed Fabry disease and acute nephrotic syndrome with clinical and microscopic findings suggestive of minimal change disease.

  4. Long-term Outcomes of Childhood Onset Nephrotic Syndrome.

    Science.gov (United States)

    Hjorten, Rebecca; Anwar, Zohra; Reidy, Kimberly Jean

    2016-01-01

    There are limited studies on long-term outcomes of childhood onset nephrotic syndrome (NS). A majority of children with NS have steroid-sensitive nephrotic syndrome (SSNS). Steroid-resistant nephrotic syndrome (SRNS) is associated with a high risk of developing end-stage renal disease. Biomarkers and analysis of genetic mutations may provide new information for prognosis in SRNS. Frequently relapsing and steroid-dependent NS is associated with long-term complications, including dyslipidemia, cataracts, osteoporosis and fractures, obesity, impaired growth, and infertility. Long-term complications of SSNS are likely to be under-recognized. There remain many gaps in our knowledge of long-term outcomes of childhood NS, and further study is indicated.

  5. A RETROSPECTIVE STUDY ON CLINICAL PRESENTATION OF STEROID SENSITIVE NEPHROTIC SYNDROME

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    Sosamma M. M

    2016-09-01

    Full Text Available BACKGROUND Nephrotic syndrome is a disease affecting the renal system. Most paediatricians will invariably encounter children with nephrotic syndrome in their clinic. The disease is characterised by the presence of oedema, persistent heavy proteinuria, hypoproteinaemia and hypercholesterolaemia. The disease is influenced by factors like age, geography, race and also has certain genetic influence related to HLA (DR7, B12, B8. In children, minimal change nephrotic syndrome is the most common variant of primary nephrotic syndrome. It accounts to more than eighty per cent of the cases seen children under seven years whereas it has a chance of fifty per cent in the age group of seven to sixteen years. Males are affected two times higher compared to females. The parents usually bring the child to the hospital with signs of oedema. Usually, the child recovers with treatment, but in some cases, there can be relapse. MATERIALS AND METHODS  The study was conducted in the Department of Paediatrics, Travancore Medical College, Kollam.  The study was done from January 2015 to January 2016.  Sixty cases were identified and were chosen for the study. INCLUSION CRITERIA 1. Steroid sensitive cases of nephrotic syndrome. 2. Age less than twelve years. 3. Admitted cases. EXCLUSION CRITERIA 1. Steroid-resistant and steroid-dependent cases. 2. Age more than twelve years. 3. Outpatient cases. RESULTS Out of the sixty cases studied, forty one cases belonged to male sex and nineteen cases belonged to female sex. Based on the age group, maximum number of cases belonged to age group four to eight years, which amounted to thirty four cases followed by age group eight to twelve years, which amounted to eighteen cases. Age group zero to four years had the least number of cases, which amounted to eight in number. Based on clinical signs and symptoms, fifty five cases presented with oedema either periorbital, scrotal or pedal oedema. Ten cases presented with fever

  6. Sustained complete remission of steroid- and cyclophosphamide-resistant minimal-change disease with a single course of rituximab therapy

    National Research Council Canada - National Science Library

    Janardan, Jyotsna; Ooi, Khai; Menahem, Solomon

    2014-01-01

    We report a case of steroid- and cyclophosphamide-resistant nephrotic syndrome secondary to minimal-change disease occurring in an otherwise healthy 19-year-old female, responding rapidly to two doses...

  7. Mammary-type myofibroblastoma with the nephrotic syndrome.

    Science.gov (United States)

    Colbert, Gates B; Vankawala, Preksha; Kuperman, Michael B; Mennel, Robert G

    2016-07-01

    We describe a 23-year-old white man who presented with anasarca and a new periumbilical mass. He had preserved kidney function and laboratory findings consistent with nephrotic syndrome, including 9.7 g/day albuminuria. Serum serologies were positive for anti-SSa and anti-SSb and low complements but were negative for antinuclear antibody. Pathologic findings of the abdominal mass showed a mammary-type myofibroblastoma. A kidney biopsy revealed a diffuse proliferative and membranous immune-mediated glomerulonephritis with 10% interstitial fibrosis. This is a novel case of mammary-type myofibroblastoma associated with nephrotic syndrome mimicking a proliferative lupus pattern.

  8. Serum immunoglobulin E and interleukin-13 levels in children with idiopathic nephrotic syndrome.

    Science.gov (United States)

    Mishra, Om P; Teli, Akkatai S; Singh, Usha; Abhinay, Abhishek; Prasad, Rajniti

    2014-12-01

    Serum IgE and IL-13 levels were estimated in 40 idiopathic nephrotic syndrome and 16 controls. There were 15 first episode nephrotic syndrome (FENS), 15 infrequent relapsing nephrotic syndrome (IRNS) and 10 patients belonged to frequent relapsing nephrotic syndrome (FRNS). Serum IgE and IL-13 levels were significantly increased in active nephrotic syndrome and its sub-groups as compared to controls and remission (p Il-13 was significantly higher in FRNS in comparison with FENS (p = 0.041). Both IgE and IL-13 levels were comparable in nephrotic patients with and without bronchial asthma. Serum IL-13 had significant positive correlation with IgE (r = 0.605, p IL-13 are found in nephrotic syndrome and could have a role in the pathogenesis of disease.

  9. Expression of galectin-3 in nephrotic syndrome glomerulopaties in children.

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    Dariusz Samulak

    2009-12-01

    Full Text Available BACKGROUND
    Galectins are a family of ancient animal carbohydrate binding proteins; the name is from their description as beta-galactoside-specific lectins. They have been strongly implicated in inflammation and cancer. Studies of the association of galectins with various aspects of kidney disease in humans are still at an early stage. In line with the above, the aim of the present report was to analyse the immunohistochemical expression of galectin-3 (the only chimera galectin currently identified in renal biopsy specimens of children with idiopathic nephrotic syndrome (INS.

    PATIENTS AND METHODS
    Eighteen children with minimal change disease (MCD, 30 with diffuse mesangial proliferation (DMP and 11 with focal segmental glomerulosclerosis (FSGS treated between 2003 and 2006 in the Department of Paediatric Cardiology and Nephrology, Poznan University of Medical Sciences. An indirect immunohistochemical protocol using a polyclonal rabbit antibody against human galectin-3 was employed.

    RESULTS
    In the control, MCD and DMP children who responded to steroid therapy anti-galectin-3 reactivity was present both in renal cortex and medulla. It was the strongest within cortical collecting ducts and subjectively less expressed in distal tubules. The total number of galectin-3 positive cortical and medullary segments of collecting ducts was significantly higher in the subjects who did not respond to steroid therapy These patients revealed also immunohistochemical reactivity of galectin-3 within nuclei of individual glomerular mesangial cells (p<0,001.

    CONCLUSIONS
    A suggested galectin-3 authority in mature human glomeruli during proteinuric glomerulopathies may indicate, on the one hand, its anti-inflammatory effect, but on the other can prognosticate a further glomerular reconstruction leading to FSGS. Taken together, both glomerular and

  10. Clarithromycin-induced acute interstitial nephritis and minimal change disease

    OpenAIRE

    Russell, Wendy; Smith, William

    2009-01-01

    Drug associated acute interstitial nephritis and minimal change disease has been well documented but the simultaneous presentation of both is rare and has not been reported with clarithromycin. We describe a case of simultaneous acute tubulointerstitial nephritis and minimal change disease induced by clarithromycin. The patient had acute kidney injury, nephrotic syndrome, eosinophilic pneumonitis and a maculopapular skin rash. The role of steroid therapy in acute interstitial nephritis is con...

  11. [Nephrotic syndrome. What is new since the 1988 study?].

    Science.gov (United States)

    Seves, M G; Brito, M J; Lamy, S; Luiz, P V; Bastos, G; Faleiro, M; Batista, J; De Sousa, J F

    1998-07-01

    The authors make a retrospective review of 53 new cases of Nephrotic Syndrome followed up in the Nephrology Unit from November 1988 to March 1994, bearing in mind the evaluation of casual changes of the disease standard regarding a previous study of 1988. Epidemiological, clinical, therapeutical and evolutional aspects were studied. Forty-four cases of primary Nephrotic Syndrome (83%) were identified, 61.4% of which behaved as cortico-sensitive, 25% as cortico-dependent, and 13.6% as cortico-resistant; 8 cases (15%) of Nephrotic Syndrome secondary to infection, Systemic Lupus Erythematosus and Amyloidosis, and 1 case of congenital Nephrotic Syndrome (2%). The theory that the high number of cortico-dependent is, probably, related with a higher severity in the relapse diagnosis and/or changes in the children's standard of living is admissible. It was also observed that at present there is a lower number of hospital discharges, related to more careful attitudes adopted regarding the evolution of the disease.

  12. Steroid Resistant Nephrotic Syndrome-Genetic Consideration.

    Science.gov (United States)

    Tasic, Velibor; Gucev, Zoran; Polenakovic, Momir

    2015-01-01

    Nephrotic syndrome is defined as the association of massive proteinuria, hypoalbuminaemia, edema, and hyperlipidemia. It is separated to steroid-sensitive or steroid-resistant (SRNS) forms in respect to the response to intensive steroid therapy. SRNS usually progresses to end-stage renal failure. According to the North American Pediatric Renal Trials and Collaborative Studies SRNS constitutes the second most frequent cause of ESRD in the first two decades of life. Unfortunately, there is no curative treatment for majority of patients. Majority of the SRNS patients have the histologic picture of focal segmental glomerulosclerosis. Interestingly, the risk of recurrence in the kidney graft in patients with hereditary SRNS is lower than in those who do not have genetic background. The etiology and pathogenesis of SRSN has remained enigma for decades. The discovery of 39 dominant or recessive SRNS genes enabled better understanding of the function of the glomerular podocytes and slit membrane. Hildebrandt's group has shown that 85% of the SRNS cases with onset by 3 months of age and 66% with onset by 1 year of age can be explained by recessive mutations in one of four genes only (NPHS1, NPHS2, LAMB2, or WT1). The same group used modern diagnostic techniques such as the next generation sequencing and tested a large international cohort of SRNS patients (n = 1783 families). The diagnostic panel included 21 genes with a recessive mode of inheritance and 6 genes with a dominant mode of inheritance. Single-gene cause was detected in 29.5% (526 of 1783) of the families with SRNS that manifested before 25 years of age. The identification of causative single-gene mutations may have important therapeutic consequences in some cases. This is very important for patients who carry mutations in a gene of coenzyme Q10 biosynthesis (COQ2, COQ6, ADCK4, or PDSS2). In these patients the treatment with coenzyme Q10 may be indicated. Also, patients with recessive mutations in PLCE1 may

  13. RENIN ANGIOTENSIN SYSTEM GENE POLYMORPHISMS IN CHILDREN WITH NEPHROTIC SYNDROM

    Directory of Open Access Journals (Sweden)

    Zh.P. Sharnova

    2006-01-01

    Full Text Available To investigate the role of the reninangiotensin system genes polymorphisms in develop and progression of nephrotic syndrom (NS in children we determined the genotypes of angiotensin converting enzyme (ACE, angiotensinogen (AGT and angiotensin ii receptor (ATII-R of 1 type in 80 russian children with ns including and 15 children with chronic renal failure (CRF. Genotype frequencies did not differ between patients with ns and controls (n = 165. The distribution of ace, AGT and ATII-R 1 type genotypes was similar among ns sub groups, such as focal segmental glomerulosclerosis (FSGS (n = 18, steroid-sensitive nephrotic syndrome (n = 32, nephrotic syndrome with hypertension and hemoturia (n = 22 and with control group. When ns subjects with CRF (n = 15 were compared with control, the prevalence of ace DD genotype was significantly higher (47% VS 21%; χ2 = 4,44; p < 0,05. Our results indicate that the DD genotype ace may be a factor of risk for the dеvеlopment of progressive renal impairment in the children with nephrotic syndrome. The analysis of treatment's effect with inhibitor of ace in groups patients with steroid resistant NS (SRNS demonstrated decreasing of renoprotective effect of this drugs in patients with id and dd genotypes com? Pared with ii genotype: the degree of blood pressure, proteinuria and the rate of glomerular filtration decrease was significantly lower (55,46 ± 9,25 VS 92,74 ± 25; р < 0,05 in these patients.Key words: nephrotic syndrom, chronic renal failure, polymorphism of genes, renin-angiotensin system.

  14. Hypovolemia and hypovolemic shock in children with nephrotic syndrome.

    Science.gov (United States)

    Wang, S J; Tsau, Y K; Lu, F L; Chen, C H

    2000-01-01

    Hypovolemic shock is not an uncommon presentation in nephrotic syndrome, yet it is seldom mentioned in the literature. This study was performed to investigate the prevalence of hypovolemia and hypovolemic shock in the acute nephrotic stage, and the association of hemoconcentration and abdominal pain with hypovolemic status. Two hundred and twenty-five patients with a total of 328 admissions to the pediatric ward of our hospital during 1983 to 1996 were retrospectively reviewed for hypovolemic episodes. Clinical presentation and laboratory data including hemoglobin, serum sodium, albumin, cholesterol, and triglyceride levels were investigated. Thirteen patients with 19 episodes (5.8%) of hypovolemic shock were found, and had more severe hemoconcentration (hemoglobin 19.6 +/- 2.2 g/dL) and hyponatremia (127.3 +/- 7.2 mEq/L). Another 33 patients with 41 symptomatic hypovolemic episodes without hypotension (12.5%) were found, and their hemoglobin levels were higher compared to patients without hypovolemic symptoms. Among 61 episodes of abdominal pain and hemoconcentration, 58 were responsive to albumin infusion. This suggested the presence of hypovolemia. Twenty patients had abdominal pain without hemoconcentration, and 18 of them had primary peritonitis. Hypovolemia was found in patients at the acute nephrotic stage, and was usually associated with hemoconcentration and abdominal pain. A combined examination of hemoglobin and serum sodium is the best indicator of hypovolemic status. Both primary peritonitis and hypovolemic episodes should be taken into consideration when managing abdominal pain in children with nephrotic syndrome.

  15. Circulating angiopoietin-like 4 links proteinuria with hypertriglyceridemia in nephrotic syndrome

    Science.gov (United States)

    Clement, Lionel C; Macé, Camille; Avila-Casado, Carmen; Joles, Jaap A; Kersten, Sander; Chugh, Sumant S

    2014-01-01

    The molecular link between proteinuria and hyperlipidemia in nephrotic syndrome is not known. We show in the present study that plasma angiopoietin-like 4 (Angptl4) links proteinuria with hypertriglyceridemia through two negative feedback loops. In previous studies in a rat model that mimics human minimal change disease, we observed localized secretion by podocytes of hyposialylated Angptl4, a pro-proteinuric form of the protein. But in this study we noted high serum levels of Angptl4 (presumably normosialylated based on a neutral isoelectric point) in other glomerular diseases as well. Circulating Angptl4 was secreted by extrarenal organs in response to an elevated plasma ratio of free fatty acids (FFAs) to albumin when proteinuria reached nephrotic range. In a systemic feedback loop, these circulating pools of Angptl4 reduced proteinuria by interacting with glomerular endothelial αvβ5 integrin. Blocking the Angptl4–β5 integrin interaction or global knockout of Angptl4 or β5 integrin delayed recovery from peak proteinuria in animal models. But at the same time, in a local feedback loop, the elevated extrarenal pools of Angptl4 reduced tissue FFA uptake in skeletal muscle, heart and adipose tissue, subsequently resulting in hypertriglyceridemia, by inhibiting lipoprotein lipase (LPL)-mediated hydrolysis of plasma triglycerides to FFAs. Injecting recombinant human ANGPTL4 modified at a key LPL interacting site into nephrotic Buffalo Mna and Zucker Diabetic Fatty rats reduced proteinuria through the systemic loop but, by bypassing the local loop, without increasing plasma triglyceride levels. These data show that increases in circulating Angptl4 in response to nephrotic-range proteinuria reduces the degree of this pathology, but at the cost of inducing hypertriglyceridemia, while also suggesting a possible therapy to treat these linked pathologies. PMID:24317117

  16. Levamisole for steroid-dependent nephrotic syndrome of childhood

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    Hadadi M

    2008-06-01

    Full Text Available Background: Childhood nephrotic syndrome is frequently characterized by a relapsing course. Due to their adverse effects, the use of corticosteroids for the management of frequently relapsing nephrotic syndrome is limited. Levamisole, a steroid sparing agent, has been found to have low toxicity. This study was conducted to evaluate the efficacy of levamisole in steroid-sensitive nephrotic syndrome (SDNS.  Methods: In this retrospective study from January 1988 to September 2006, we included data from 305 pediatric SDNS patients at the Children's Medical Center clinics in Tehran, Iran. Nephrotic syndrome was diagnosed using classic criteria. None of the patients had any signs or symptoms of secondary causes of nephrotic syndrome. All had received prednisolone 60 mg/m2/day. After remission, prednisolone administration was reduced to every other day and the steroid was tapered over the next three months. With every recurrence, prednisolone was prescribed with the same dosage, but after remission it was continued at a lower dosage for another six months or longer if there was risk of recurrence. Levamisole was administered to all patients at a dose of 2 mg/kg every other day.          Results: Patients ranged in age from 1 to 20 years (mean±SD: 4.84 ±3.1 and 70.8% were male. At the last follow up, 84 (27.5% were in remission, while 220 (72.1% patients had relapsed or needed a low dose of steroid. Levamisole was effective in reducing the prednisolone dosage and long-term remission in 68 (22.3% and 90 (29.5% cases, respectively. A comparison of before vs. after levamisole treatment revealed a had significant decrease in the number of relapses (2.05±0.88 vs. 1.1±1.23; P<0.0001 and the prednisolone dosage (0.74±0.39 vs. 0.32±0.38 mg/kg/day; P<0.0001. Only one patient developed levamisole-induced neutropenia. Conclusions: In childhood steroid-dependent nephrotic syndrome, levamisole is an efficacious, safe initial therapy in

  17. Treatment of nephrotic syndrome associated with idiopathic rapidly progressive glomerulonephritis and cyclosporin A.

    Science.gov (United States)

    Maduell, F; Sánchez-Alcaraz, A; Sigüenza, F; Caridad, A; Sangrador, G

    1993-02-01

    Recent reports suggest that cyclosporin A is beneficial in inducing remission of idiopathic nephrotic syndrome. Nephrotic syndrome is seen in 10-30% of patients with rapidly progressive glomerulonephritis. We report a case of a 69-year-old man with nephrotic syndrome, associated with idiopathic rapidly progressive glomerulonephritis, who was treated initially with corticosteroid and cyclophosphamide. Three months later he developed thrombophlebitis and leucopenia and cyclophosphamide was suspended. Relapse of nephrotic syndrome associated with rapidly progressive glomerulonephritis developed and therapy with cyclosporin A was used with a good response.

  18. Molecular and Genetic Basis of Inherited Nephrotic Syndrome

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    Maddalena Gigante

    2011-01-01

    Full Text Available Nephrotic syndrome is an heterogeneous disease characterized by increased permeability of the glomerular filtration barrier for macromolecules. Podocytes, the visceral epithelial cells of glomerulus, play critical role in ultrafiltration of plasma and are involved in a wide number of inherited and acquired glomerular diseases. The identification of mutations in nephrin and other podocyte genes as causes of genetic forms of nephrotic syndrome has revealed new important aspects of the pathogenesis of proteinuric kidney diseases and expanded our knowledge of the glomerular biology. Moreover, a novel concept of a highly dynamic slit diaphragm proteins is emerging. The most significant discoveries in our understanding of the structure and function of the glomerular filtration barrier are reviewed in this paper.

  19. Chryseobacterium gleum pneumonia in an infant with nephrotic syndrome.

    Science.gov (United States)

    Abdalhamid, Baha; Elhadi, Nasreldin; Alsamman, Khaldoon; Aljindan, Reem

    2016-01-01

    Chryseobacterium gleum is commonly distributed in the environment. It can cause a wide variety of infections in immunocompromised patients in hospital setting. A 6 month old infant with nephrotic syndrome was admitted to the emergency room for an acute onset of fever, difficulty breathing, cyanosis, and low oral intake. Cultures of endotracheal tube specimens were positive for Chryseobacterium gleum which was confirmed by ribosomal sequencing. The organism was susceptible to trimethoprim-sulfamethoxazole, minocycline, and levofloxacin. The patient clinically improved on levofloxacin treatment. To the best of our knowledge, this is the first case of pneumonia caused by Chryseobacterium gleum in an infant with nephrotic syndrome. It is also the first report of C. gleum causing respiratory tract infection in Saudi Arabia.

  20. [Thrombosis and disorders of hemostasis in nephrotic syndrome].

    Science.gov (United States)

    Kanfer, A

    1992-01-01

    Thromboses and disorders of hemostasis in nephrotic syndrome. Thromboembolic complications are common in nephrotic syndrome (NS). This article reviews the factors of thrombogenesis in NS, including: 1) a hypercoagulable state with platelet hyperaggregability, hyperfibrinogenemia and elevated factor VIII, decrease in plasma levels of coagulation inhibitors antithrombin III and free protein S, reduced fibrinolytic activity; 2) excessive intravascular thrombin formation marked by increased plasma levels of fibrinopeptide A. The intensity of hemostasis disorders coincides with that of NS. Most disorders are related to hypoalbuminemia and proteinuria. In agreement with experimental data, the role of intraglomerular activation of coagulation during active phases of glomerulopathies has to be considered. This could explain the predominance of renal vein thrombosis in several glomerulopathies with NS. Several coagulation disorders in SN have implications for therapy.

  1. [Treatment of renal vein thrombosis associated with nephrotic syndrome].

    Science.gov (United States)

    Funami, M; Takaba, T; Tanaka, H; Murakami, A; Kadokura, M; Hori, G; Ishii, J

    1988-06-01

    Renal vein thrombosis is a rare entity in which true incidence is unknown. The disease occurs most frequently in patients with nephrotic syndrome, but it also can occur in the presence of other hypercoagulable state. Two cases of renal vein thrombosis with nephrotic syndrome which were treated by thrombectomy are reported here. One patient was successfully treated by renal vein and inferior vena cava thrombectomy before developing severe pulmonary embolism. The other was treated by renal vein thrombectomy by which fatal shock was able to be prevented. In those cases, immediate operation was indicated, primarily to prevent additional, possibly fatal, pulmonary embolism and also to improve perfusion of the kidney. In the hope of salvaging the kidney, thrombectomy may be the treatment of choice for acute renal vein thrombosis, complication of pulmonary embolism and inferior vena cava thrombosis, right renal vein thrombosis without collateral flow and acute renal vein thrombosis with shock.

  2. Genetics of childhood steroid-sensitive nephrotic syndrome.

    Science.gov (United States)

    Karp, Alana M; Gbadegesin, Rasheed A

    2016-07-29

    The pathogenesis of childhood-onset nephrotic syndrome (NS), disparity in incidence of NS among races, and variable responses to therapies in children with NS have defied explanation to date. In the last 20 years over 50 genetic causes of steroid-resistant nephrotic syndrome (SRNS) have been identified, and at least two disease loci for two pathologic variants of SRNS (focal segmental glomerulosclerosis and membranous nephropathy) have been defined. However, the genetic causes and risk loci for steroid-sensitive nephrotic syndrome (SSNS) remain elusive, partly because SSNS is relatively rare and also because cases of SSNS vary widely in phenotypic expression over time. A recent study of a well-defined modest cohort of children with SSNS identified variants in HLA-DQA1 as a risk factor for SSNS. Here we review what is currently known about the genetics of SSNS and also discuss how recent careful phenotypic and genomic studies reinforce the role of adaptive immunity in the molecular mechanisms of SSNS.

  3. 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.

  4. 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

    2010-01-01

    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. PMID:20110379

  5. Minimal change disease: a CD80 podocytopathy?

    Science.gov (United States)

    Ishimoto, Takuji; Shimada, Michiko; Araya, Carlos E; Huskey, Janna; Garin, Eduardo H; Johnson, Richard J

    2011-07-01

    Minimal change disease is the most common nephrotic syndrome in children. Although the etiology of minimal change disease remains to be elucidated, it has been postulated that it is the result of a circulating T-cell factor that causes podocyte cytoskeleton disorganization leading to increased glomerular capillary permeability and/or changes in glomerular basement membrane heparan sulfate glycosaminoglycans resulting in proteinuria. Minimal change disease has been associated with allergies and Hodgkin disease. Consistent with these associations, a role for interleukin-13 with minimal change disease has been proposed. Furthermore, studies evaluating podocytes also have evolved. Recently, increased expression of CD80 (also termed B7-1) on podocytes was identified as a mechanism for proteinuria. CD80 is inhibited by binding to CTLA-4, which is expressed on regulatory T cells. Recently, we showed that urinary CD80 is increased in minimal change disease patients and limited studies have suggested that it is not commonly present in the urine of patients with other glomerular diseases. Interleukin-13 or microbial products via Toll-like receptors could be factors that induce CD80 expression on podocytes. CTLA-4 appears to regulate CD80 expression in podocytes, and to be altered in minimal change disease patients. These findings lead us to suggest that proteinuria in minimal change disease is caused by persistent CD80 expression in podocytes, possibly initiated by stimulation of these cells by antigens or cytokines.

  6. Distribution of pathological finding in the children with nephrotic syndrome from Guangxi

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    Tian-Biao Zhou

    2014-01-01

    Full Text Available To identify the variations in pediatric renal biopsy pathology and clinicopathological features in Guangxi, China, in the past ten years, we studied retrospectively the kidney biopsies performed to evaluate the primary nephrotic syndrome (PNS in 218 children at two main medical centers in Guangxi from January 1999 to January 2009. The major pathological finding was mesangial proliferative glomerulonephritis (48.2%, focal segmental glomerulosclerosis (16.5%, immunoglobulin A nephropathy (13.3% and minimal change disease (11.0%. Patients with different pathological types yielded different response rates to glucocorticoids (P <0.001. There were statistical significant differences between prognosis for the different pathological types (P <0.05. The pathological characteristics of PNS in children were diverse and significant for guiding the grade of glucocorticoid response and predicting the prognosis of the PNS disease.

  7. Correlation of fractional excretion of magnesium with steroid responsiveness in children with nephrotic syndrome

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    Jubaida Rumana

    2014-01-01

    Full Text Available Steroid-resistant nephrotic syndrome (SRNS patients are candidates for other alter-native drug regimes, and the non-responsiveness to steroid is more common among glomerulo-nephritides other than minimal change disease. Without performing biopsy and proper renal histology, progression of the disease cannot be assessed. Fractional excretion of magnesium (FE Mg has been found to correlate directly with various renal histologies. The aim of this study is to evaluate the relationship of FE Mg in children with the histological pattern in SRNS. In this prospective observational study, 40 children of nephrotic syndrome, both with the first episode as well as relapse, aged 1-12 years were included in the study. Of them, 20 were steroid-responsive cases and 20 were steroid-resistant cases. FE Mg was determined in all the patients and renal histology was performed in the steroid-resistant cases. A correlation was found between FE Mg and renal histology. Data were analyzed in SPSS program version-16. Comparison of two groups was performed by the Fisher exact test and unpaired t test. P-value less than 0.05 were considered to be significant. The results of histo-pathology showed that the mean difference in FE Mg was significant (P <0.001, as FE Mg was 7.0 ± 2.3% in mesangiocapillary glomerulonephritis, 6.9 ± 1.3% in focal segmental glomerulosclerosis, 4.7 ± 0.6% in immunoglobulin M nephropathy, 4.5 ± 1.2% in focal segmental proliferative glomerulo-nephritis, 4.4 ± 1.6% in minimal change disease, 4.2 ± 0.4% in diffuse mesangial proliferative glome-rulonephritis and 3.8 ± 1.3% in mesangial proliferative glomerulonephritis. There was a statistically significant difference between FE Mg in steroid-resistant nephrotic syndrome (4.9 ± 1.9 and steroid-responsive syndrome (1.2 ± 0.3. FE Mg is a simple, minimally invasive screening marker for SRNS, and is an early predictor of clinical outcome. It can be considered as an initial investigation where biopsy

  8. Correlation of fractional excretion of magnesium with steroid responsiveness in children with nephrotic syndrome.

    Science.gov (United States)

    Rumana, Jubaida; Hanif, Mohammed; Muinuddin, Golam; Maruf-Ul-Quader, Mohammed

    2014-07-01

    Steroid-resistant nephrotic syndrome (SRNS) patients are candidates for other alter-native drug regimes, and the non-responsiveness to steroid is more common among glomerulo-nephritides other than minimal change disease. Without performing biopsy and proper renal histology, progression of the disease cannot be assessed. Fractional excretion of magnesium (FE Mg) has been found to correlate directly with various renal histologies. The aim of this study is to evaluate the relationship of FE Mg in children with the histological pattern in SRNS. In this prospective observational study, 40 children of nephrotic syndrome, both with the first episode as well as relapse, aged 1-12 years were included in the study. Of them, 20 were steroid-responsive cases and 20 were steroid-resistant cases. FE Mg was determined in all the patients and renal histology was performed in the steroid-resistant cases. A correlation was found between FE Mg and renal histology. Data were analyzed in SPSS program version-16. Comparison of two groups was performed by the Fisher exact test and unpaired t test. P-value less than 0.05 were considered to be significant. The results of histo-pathology showed that the mean difference in FE Mg was significant (P <0.001), as FE Mg was 7.0 ± 2.3% in mesangiocapillary glomerulonephritis, 6.9 ± 1.3% in focal segmental glomerulosclerosis, 4.7 ± 0.6% in immunoglobulin M nephropathy, 4.5 ± 1.2% in focal segmental proliferative glomerulo-nephritis, 4.4 ± 1.6% in minimal change disease, 4.2 ± 0.4% in diffuse mesangial proliferative glome-rulonephritis and 3.8 ± 1.3% in mesangial proliferative glomerulonephritis. There was a statistically significant difference between FE Mg in steroid-resistant nephrotic syndrome (4.9 ± 1.9) and steroid-responsive syndrome (1.2 ± 0.3). FE Mg is a simple, minimally invasive screening marker for SRNS, and is an early predictor of clinical outcome. It can be considered as an initial investigation where biopsy cannot be

  9. Budd-Chiari syndrome during nephrotic relapse in a patient with resistance to activated protein C clotting inhibitor.

    Science.gov (United States)

    Gambaro, G; Patrassi, G; Pittarello, F; Nardellotto, A; Checchetto, S; D'Angelo, A

    1998-10-01

    It has long been known that patients with nephrotic syndrome have a hypercoagulable state, which explains the association between nephrotic syndrome, renal vein thrombosis, and thromboembolism. However, the Budd-Chiari syndrome has never been reported in nephrotic patients. This is the first report of such an association that, most likely, depended on a primary resistance to activated protein C.

  10. Minimal change disease onset observed after bevacizumab administration.

    Science.gov (United States)

    Hanna, Ramy M; Lopez, Eduardo; Wilson, James; Barathan, Shrinath; Cohen, Arthur H

    2016-04-01

    This is a report of a patient with minimal change disease (MCD) onset after bevacizumab administration. A 72-year-old man with inoperable Grade 3 astrocytoma was treated with a combination of temozolomide and the vascular endothelial growth factor monoclonal antibody bevacizumab. After two biweekly treatments, he developed nephrotic syndrome. Despite cessation of bevacizumab, his renal function deteriorated and a renal biopsy disclosed MCD. Thereafter, he was started on high-dose oral prednisone and renal function immediately improved. Within weeks, the nephrotic syndrome resolved. Although rare, biologic agents can cause various glomerulopathies that can have important therapeutic implications. MCD should be considered in patients who develop nephrotic syndrome while exposed to antiangiogenic agents.

  11. Minimal change disease: A case report of an unusual relationship.

    Science.gov (United States)

    Edrees, Fahad; Black, Robert M; Leb, Laszlo; Rennke, Helmut

    2016-01-01

    Kidney injury associated with lymphoproliferative disorders is rare, and the exact pathogenetic mechanisms behind it are still poorly understood. Glomerular involvement presenting as a nephrotic syndrome has been reported, usually secondary to membranoproliferative glomerulonephritis. We report a case of a 63-year-old male who presented with bilateral leg swelling due to nephrotic syndrome and acute kidney injury. A kidney biopsy showed minimal change disease with light chain deposition; however, no circulating light chains were present. This prompted a bone marrow biopsy, which showed chronic lymphocytic leukemia (CLL) with deposition of the same kappa monoclonal light chains. Three cycles of rituximab and methylprednisolone resulted in remission of both CLL and nephrotic syndrome, without recurrence during a three-year follow-up.

  12. Rituximab in Minimal Change Disease

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    Nima Madanchi

    2017-03-01

    Full Text Available Treatment with rituximab, a monoclonal antibody against the B-lymphocyte surface protein CD20, leads to the depletion of B cells. Recently, rituximab was reported to effectively prevent relapses of glucocorticoid-dependent or frequently relapsing minimal change disease (MCD. MCD is thought to be T-cell mediated; how rituximab controls MCD is not understood. In this review, we summarize key clinical studies demonstrating the efficacy of rituximab in idiopathic nephrotic syndrome, mainly MCD. We then discuss immunological features of this disease and potential mechanisms of action of rituximab in its treatment based on what is known about the therapeutic action of rituximab in other immune-mediated disorders. We believe that studies aimed at understanding the mechanisms of action of rituximab in MCD will provide a novel approach to resolve the elusive immune pathophysiology of MCD.

  13. Acute lymphoblastic leukaemia after treatment of nephrotic syndrome with immunosuppressive drugs.

    Science.gov (United States)

    Kuis, W; de Kraker, J; Kuijten, R H; Donckerwolcke, R A; Voûte, P A

    1976-06-01

    The authors present a 4-year-old girl with nephrotic syndrome who developed actue lymphoblastic leukaemia 5 months after the start of a combined treatment of alternate day prednisone and daily cyclophosphamide during 3 months. The nephrotic syndrome was due to focal segmental glomerulosclerosis. The occurrence of leukaemia might be related to the preceeding treatment with cyclophosphamide.

  14. Serum D-dimer concentrations in nephrotic syndrome track with albuminuria, not estimated glomerular filtration rate.

    LENUS (Irish Health Repository)

    Sexton, D J

    2012-01-01

    The nephrotic syndrome is associated with an increased risk of venous and arterial thrombosis. There are little published data on the distribution, interpretation or determinants of serum D-dimer levels in patients with the nephrotic syndrome. We aimed to describe this relationship.

  15. Meningitis and intracranial bleed in a child with steroid-resistant nephrotic syndrome

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    Kanika Kapoor

    2015-01-01

    Full Text Available Meningitis and associated intracranial bleeding have been rarely reported in patients with steroid-resistant nephrotic syndrome. We present such a case with raised intracranial tension in a 13-year-old child and discuss the management issues. Prompt recognition and appropriate treatment of these complications can be life saving in a child with nephrotic syndrome.

  16. Meningitis and intracranial bleed in a child with steroid-resistant nephrotic syndrome.

    Science.gov (United States)

    Kapoor, Kanika; Saha, Abhijeet; Thakkar, Dhwanee; Dubey, N K; Vani, Kavita

    2015-11-01

    Meningitis and associated intracranial bleeding have been rarely reported in patients with steroid-resistant nephrotic syndrome. We present such a case with raised intracranial tension in a 13-year-old child and discuss the management issues. Prompt recognition and appropriate treatment of these complications can be life saving in a child with nephrotic syndrome.

  17. Copper and zinc metabolism in aminonucleoside-induced nephrotic syndrome.

    Science.gov (United States)

    Pedraza-Chaverrí, J; Torres-Rodríguez, G A; Cruz, C; Mainero, A; Tapia, E; Ibarra-Rubio, M E; Silencio, J L

    1994-01-01

    Copper (Cu) and zinc (Zn) were measured in urine, serum and tissues from rats with nephrotic syndrome (NS) induced with a single subcutaneous dose of puromycin aminonucleoside (PAN; 15 mg/100 g BW). Control animals were pair-fed. Urine was collected daily, and the rats were sacrificed on day 10. PAN-nephrotic rats had proteinuria (days 3-10), high urinary Cu (days 1, 2, 4-10) and Zn (days 3-10) excretion. On day 10, nephrotic rats had: (a) albuminuria, hypoalbuminemia, hypoproteinemia, high urine and low serum levels of ceruloplasmin; (b) low Cu and Zn serum levels; (c) high clearance and fractional excretion of Cu and Zn, and (d) low kidney and liver Cu content and essentially normal tissue Zn levels. The alterations in Cu metabolism were more intense than those in Zn metabolism. Urine Cu and Zn showed a positive correlation with urine total protein on days 3-10 which suggests that high urinary excretion of Cu and Zn may be due to the excretion of its carrier proteins. In conclusion, these rats did not show a typical Zn deficiency but a clear decrease in Cu in the liver and kidney.

  18. Thromboembolic complications in nephrotic syndrome. Coagulation abnormalities, renal vein thrombosis, and other conditions.

    Science.gov (United States)

    Llach, F

    1984-11-01

    In patients with nephrotic syndrome, the presence of a hypercoagulable state is thought to give rise to a high incidence of thromboembolic phenomena. Renal vein thrombosis is a common complication in nephrotic patients, mainly in those with membranous nephropathy, and many other types of thromboembolic complications also occur. The mortality rate in nephrotic patients with thromboembolic complications may be significantly increased, with pulmonary emboli likely being the most common cause of death.

  19. Minimal-change disease secondary to etanercept.

    Science.gov (United States)

    Koya, Mariko; Pichler, Raimund; Jefferson, J Ashley

    2012-10-01

    Etanercept is a soluble tumor necrosis factor alpha (TNFα) receptor which is widely used in the treatment of rheumatoid arthritis, psoriasis and other autoimmune inflammatory disorders. It is known for its relative lack of nephrotoxicity; however, there are reports on the development of nephrotic syndrome associated with the treatment with TNFα antagonists. Here, we describe a patient with psoriasis who developed biopsy-proven minimal-change disease (MCD) shortly after initiating etanercept. Our case is unique in that the MCD resolved after discontinuation of this medication, notably without the use of corticosteroids, strongly suggesting a drug-related phenomenon.

  20. Should we consider MMF therapy after rituximab for nephrotic syndrome?

    Science.gov (United States)

    Filler, Guido; Huang, Shih-Han Susan; Sharma, Ajay P

    2011-10-01

    The management of steroid-dependent nephrotic syndrome, especially in patients who have failed to respond to cytotoxic drugs, such as cyclophosphamide, remains challenging. Rituximab represents a new (off-label) therapeutic option. In a significant portion of patients, it has a short serum half-life following the recovery of CD20-positive cells. The addition of mycophenolate mofetil (MMF) as a maintenance therapy is also an attractive option, but one which requires testing in a prospective randomized clinical trial with therapeutic drug monitoring and mechanistic ancillary studies.

  1. Pulmonary embolism as the primary presenting feature of nephrotic syndrome

    Directory of Open Access Journals (Sweden)

    Pallavi Periwal

    2016-01-01

    Full Text Available A 36-year-old previously healthy male presented with subacute onset of shortness of breath and chest pain. He was diagnosed with bilateral extensive pulmonary embolism (PE. In the absence of any predisposing factors, an extensive workup for unprovoked thrombophilia was done. During the course of his illness, the patient developed anasarca and was diagnosed to be suffering from nephrotic syndrome (NS, secondary to membranous glomerulopathy. Although, thrombotic complications are commonly associated with NS, it is unusual for PE to be the primary presenting feature in these patients.

  2. Chronic graft versus host disease and nephrotic syndrome.

    Science.gov (United States)

    Barbouch, Samia; Gaied, Hanene; Abdelghani, Khaoula Ben; Goucha, Rim; Lakhal, Amel; Torjemen, Lamia; Hamida, Fethi Ben; Abderrahim, Ezzedine; Maiz, Hedi Ben; Adel, Khedher

    2014-09-01

    Disturbed kidney function is a common complication after bone marrow transplantation. Recently, attention has been given to immune-mediated glomerular damage related to graft versus host disease (GVHD). We describe a 19-year-old woman who developed membranous glomerulonephritis after bone marrow transplantation (BMT). Six months later, she developed soft palate, skin and liver lesions considered to be chronic GVHD. Fifteen months after undergoing BMT, this patient presented with nephrotic syndrome. A renal biopsy showed membranous glomerulonephritis associated with a focal segmental glomerulosclerosis. She was started on corticosteroid treatment with good outcome.

  3. Chronic graft versus host disease and nephrotic syndrome

    Directory of Open Access Journals (Sweden)

    Samia Barbouch

    2014-01-01

    Full Text Available Disturbed kidney function is a common complication after bone marrow transplantation. Recently, attention has been given to immune-mediated glomerular damage related to graft versus host disease (GVHD. We describe a 19-year-old woman who developed membranous glomerulonephritis after bone marrow transplantation (BMT. Six months later, she developed soft palate, skin and liver lesions considered to be chronic GVHD. Fifteen months after undergoing BMT, this patient presented with nephrotic syndrome. A renal biopsy showed mem-branous glomerulonephritis associated with a focal segmental glomerulosclerosis. She was started on corticosteroid treatment with good outcome.

  4. Minimal change disease: a variant of lupus nephritis.

    Science.gov (United States)

    Moysés-Neto, Miguel; Costa, Roberto S; Rodrigues, Fernanda F; Vieira Neto, Osvaldo M; Reis, Marlene A; Louzada Júnior, Paulo; Romão, Elen A; Dantas, Márcio

    2011-02-01

    Some patients with systemic lupus erythematosus (SLE) present with nephrotic syndrome due to minimal change disease (MCD). Histopathological diagnosis of patients with SLE and nephrotic-range proteinuria has shown that these patients present with diffuse proliferative glomerulonephritis and membranous glomerulonephritis, World Health Organization (WHO) classes IV and V, respectively, more frequently than the other classes. In the present study, we reported a case of nephrotic syndrome and renal biopsy-proven MCD associated with SLE. A complete remission occurred after steroid treatment, which was followed by a relapse 15 months later with a concomitant reactivation of SLE. A second biopsy showed WHO class IIb lupus nephritis. Prednisone treatment was restarted, and the patient went into complete remission again. The association of MCD and SLE may not be a coincidence, and MCD should be considered as an associated SLE nephropathy.

  5. History of Nephrotic Syndrome and Evolution of its Treatment

    Directory of Open Access Journals (Sweden)

    Abhijeet ePal

    2016-05-01

    Full Text Available The recognition, evaluation, and early treatment of nephrotic syndrome in infants and children originates from physicians dating back to Hippocrates. It took nearly another thousand years before the condition was described for its massive edema requiring treatment with herbs and other remedies. A rich history of observations and interpretations followed over the course of centuries until the recognition of the combination of clinical findings of foamy urine, swelling of the body, and measurements of urinary protein and blood analyses showed the phenotypic characteristics of the syndrome that were eventually linked to the early anatomic descriptions from first kidney autopsies and then renal biopsy analyses. Coincident with these findings were a series of treatment modalities involving the use of natural compounds to a host of immunosuppressive agents that are applied today. With the advent of molecular and precision medicine, the field is poised to make major advances in our understanding and effective treatment of nephrotic syndrome and prevent its long-term sequelae.

  6. Defining nephrotic syndrome from an integrative genomics perspective.

    Science.gov (United States)

    Sampson, Matthew G; Hodgin, Jeffrey B; Kretzler, Matthias

    2015-01-01

    Nephrotic syndrome (NS) is a clinical condition with a high degree of morbidity and mortality, caused by failure of the glomerular filtration barrier, resulting in massive proteinuria. Our current diagnostic, prognostic and therapeutic decisions in NS are largely based upon clinical or histological patterns such as "focal segmental glomerulosclerosis" or "steroid sensitive". Yet these descriptive classifications lack the precision to explain the physiologic origins and clinical heterogeneity observed in this syndrome. A more precise definition of NS is required to identify mechanisms of disease and capture various clinical trajectories. An integrative genomics approach to NS applies bioinformatics and computational methods to comprehensive experimental, molecular and clinical data for holistic disease definition. A unique aspect is analysis of data together to discover NS-associated molecules, pathways, and networks. Integrating multidimensional datasets from the outset highlights how molecular lesions impact the entire individual. Data sets integrated range from genetic variation to gene expression, to histologic changes, to progression of chronic kidney disease (CKD). This review will introduce the tenets of integrative genomics and suggest how it can increase our understanding of NS from molecular and pathophysiological perspectives. A diverse group of genome-scale experiments are presented that have sought to define molecular signatures of NS. Finally, the Nephrotic Syndrome Study Network (NEPTUNE) will be introduced as an international, prospective cohort study of patients with NS that utilizes an integrated systems genomics approach from the outset. A major NEPTUNE goal is to achieve comprehensive disease definition from a genomics perspective and identify shared molecular drivers of disease.

  7. Elevation of plasma-soluble HLA-G in childhood nephrotic syndrome is associated with IgE.

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    Liu, Yanqing; Lai, Meimei; Lou, Yunyan; Han, Qiuyue; Yang, Qing; Chen, Minguang; Li, Jingbo; Wang, Huiyan; Yan, Weihua; Zheng, Xiaoqun

    2017-01-01

    Background Nephrotic syndrome is related to immune system dysfunction. Soluble human leukocyte antigen-G has been suggested to have an immunomodulatory role. Additionally, human leukocyte antigen-G expression may be influenced by the 14-base pair insertion/deletion polymorphism. However, this molecule has not been investigated in nephrotic syndrome. Methods Fifty-five children with nephrotic syndrome were enrolled: 24 primary nephrotic syndrome patients and 31 recurrent nephrotic syndrome patients. A group of 120 healthy subjects were included as reference controls. Additionally, 22 patients in nephrotic syndrome remission after treatments were also included. Both nephrotic syndrome patients and healthy subjects were genotyped for the 14-base pair insertion/deletion polymorphism. Plasma soluble human leukocyte antigen-G concentrations and serum immunoglobulin concentrations were determined. Results Nephrotic syndrome patients showed significantly higher levels of both soluble human leukocyte antigen-G and immunoglobulin E compared to normal controls. Nephrotic syndrome patients presented a higher frequency of the -14-base pair allele than did normal controls. Soluble human leukocyte antigen-G concentrations in remission patients were dramatically lower compared to in nephrotic syndrome patients. Moreover, soluble human leukocyte antigen-G and immunoglobulin E were moderately correlated in nephrotic syndrome patients. Conclusions The present study demonstrated that plasma soluble human leukocyte antigen-G concentrations were significantly elevated and that a relationship between serum total immunoglobulin E in nephrotic syndrome patients and the human leukocyte antigen-G -14-base pair allele may be a risk factor for nephrotic syndrome. These findings suggest that soluble human leukocyte antigen-G may be used as a monitoring marker for nephrotic syndrome patients' condition.

  8. Long-term treatment of childhood refractory and steroid dependent nephrotic syndrome with Cyclosporin A

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    Madani A

    2002-07-01

    Full Text Available Background: Cyclosporin A (CsA is now commonly used in the management of children with steroid-dependent and steroid resistant nephoitic syndrome. It has been reported to be effective in maintaining remission in 70-100 percent of patients with SDNS but somewhat SRNS 0-100 percent. The aim of this study was to evaluate the efficacy of long-term (CsA in children with refractory nephrotic syndrome (RNS and steroid dependent nephrotic syndrome (SDNS. Materials and Methods: The long-term effect of (CsA in 91 Iranian children aged 3 months to 11 years (54 with RNS and 37 with SDNS was assessed between 1984 and 1999. Eighty of 91 children received renal biopsy prior to introduction of (CsA, and the other 11 patients had not consent for kidney biopsy. If the patients did not show remission aftre receiving 3-6 months of (CsA, the medication was discontinued. Results: All patient were treated with (CsA in combination with low dose alternate day prednisolone. In children with RNS and SDNS, therapy with (CsA induced, remission in 25 of 54 (46.2 percent and 27 of 37 (73 percent respectively (P<0.02. Of the 32 patients with minimal change disease (MCD, 23 (72 percent responded to therapy, compared with 4 of 18 (22 percent with focal segmental glomerulosclerosis (FSGS (P<0.005. Twenty-four (48 percent of 50 who entered complete remission, had relapse 1-12 months after cessation of (CsA. The duration between the onset of nephrotic syndrome (NS and administration of (CsA and sexuality of patients had no effect in result of treatment. Side effects occurred in 25 patients (27.4 percent. No patients exhibited raised transaminases, 8 (8.7 percent of the children developed hirsutism, 7 (7.6 percent hypertension, 7 (7.6 percent gingival hyperplasia, (2.2 percent neurological toxicity and 1 (1 percent increase in serum creatinine. Conclusion: Our findings suggest that (CsA can be used to induce a complete remission in a significant proportion of patients with RNS and

  9. [Hodgkin disease revealed by a nephrotic syndrome: A case report].

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    Cheptou, M; Pichault, V; Campagni, R; Vodoff, M-V; Fischbach, M; Paillard, C

    2015-12-01

    Pediatric nephrotic syndrome (NS) is most often idiopathic or primary but in rare cases, it can be secondary to neoplasia. We report on a case of steroid-resistant NS revealing as a paraneoplastic syndrome of Hodgkin disease (HD) in a 12-year-old boy. The onset of the NS can be earlier, later, or simultaneous to the HD. Treatment of the lymphoma allows the disappearance of the NS. In the case we observed, the diagnosis of HD was delayed because HD presented with an isolated, hilar adenopathy in the absence of retroperitoneal or peripheral locations. In children aged 10 years or more presenting with NS, steroid-resistant or otherwise, a possible paraneoplastic origin such as Hodgkin lymphoma should always be taken into consideration and eventually eliminated.

  10. An Estimation of Steroid Responsiveness of Idiopathic Nephrotic Syndrome in Iranian Children

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    Niloofar Hajizadeh

    2010-06-01

    Full Text Available Objective:Idiopathic Nephrotic syndrome (INS is the most common form of nephrotic syndrome (NS in children with the potential of progression to end stage renal disease (ESRD. INS is steroid-responsive in most children, but not all patients respond to it. The aim of this study was to determine the rate of steroid responsiveness in children with INS that referred to Childrens Medical Center since 1995 to 2007. Methods:In as a cross sectional study, the medical records of all children with INS aged 1 to 15 years who were referred to our referral hospital was reviewed. All patients with onset of disease less than 1 year of age, spontaneous remission, secondary forms of NS associated with systemic diseases, and follow up duration of less than 12 months were excluded from the study. Patients were categorized into 6 groups: Group 1 needed biopsy prior to any treatment, group 2 non-relapsing NS, group 3 infrequently relapsing NS, Group 4 frequently relapsing NS, group 5& steroid dependent NS and group 6 steroid resistant NS. Findings:A total of 238 patients were enrolled in the study. Kidney biopsy was performed in 79 cases. Minimal change lesion (MCL was the most common (36.7% pathological diagnosis. Steroid responsiveness was found in 81.5% of all cases including: 96% of MCL (consisting of biopsy proven cases and presumed ones, 32% of focal and segmental glomerulosclerosis, 73% of diffuse mesangial proliferation and 58% of membranoproliferative glomerulonephritis& patients. During minimal follow up period of 12 months, there were 194 patients in remission, 32 patients with active NS, and 12 patients in ESRD. Conclusion:Our study results showed that 81.5% of all patients, 96.2% of MCL and 32% of FSGS patients initially responded to steroid therapy.

  11. Minimal-change renal disease and Graves’ disease: a case report and literature review

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    Hasnain, Wirasat; Stillman, Isaac E.; Bayliss, George P.

    2011-01-01

    Objective To describe a possible association between Graves' disease and nephrotic syndrome secondary to minimal change renal disease and to review the literature related to renal diseases in patients with Graves' disease. Methods The clinical, laboratory, and renal biopsy findings in a patient with Graves' disease and minimal change renal disease are discussed. In addition, the pertinent English-language literature published from 1966 to 2009, determined by means of a MEDLINE search, is revi...

  12. Minimal-change renal disease and Graves’ disease: a case report and literature review

    OpenAIRE

    Hasnain, Wirasat; Stillman, Isaac E.; Bayliss, George P.

    2011-01-01

    Objective: To describe a possible association between Graves' disease and nephrotic syndrome secondary to minimal change renal disease and to review the literature related to renal diseases in patients with Graves' disease. Methods: The clinical, laboratory, and renal biopsy findings in a patient with Graves' disease and minimal change renal disease are discussed. In addition, the pertinent English-language literature published from 1966 to 2009, determined by means of a MEDLINE search, is re...

  13. Skin grafting for necrotizing fasciitis in a child with nephrotic syndrome.

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    Bagri, Narendra; Saha, Abhijeet; Dubey, Nandkishore K; Rai, Ashish; Bhattacharya, Sameek

    2013-11-01

    Necrotizing fasciitis is a rare complication of nephrotic syndrome in children, with a high mortality rate. We report a case with successful outcome with judicious intravenous antibiotics and skin grafting of the bilateral lower thighs.

  14. Pneumococcal sepsis, peritonitis, and cellulitis at the first episode of nephrotic syndrome.

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    Naseri, Mitra

    2013-09-01

    Bacterial infections are common in patients with nephrotic syndrome, including peritonitis, sepsis, meningitis, urinary tract infection, and cellulitis. An 8-year-old boy presented with colicky abdominal pain, vomiting, swollen and painful erythematous lesions around the umbilicus and in anterior surface of left thigh (cellulitis), mild generalized edema, and ascites. The microorganism isolated from peritoneal fluid and blood cultures was Pneumococcus. Association of pneumococcal sepsis, peritonitis, and cellulitis has been rarely reported in nephrotic syndrome.

  15. Dysregulation of ENaC in Animal Models of Nephrotic Syndrome and Liver Cirrhosis

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    Kim,Soo Wan

    2006-01-01

    Nephrotic syndrome and liver cirrhosis are common clinical manifestations, and are associated with avid sodium retention leading to the development of edema and ascites. However, the mechanism for the sodium retention is still incompletely understood and the molecular basis remains undefined. We examined the changes of sodium (co)transporters and epithelial sodium channels (ENaCs) in the kidneys of experimental nephrotic syndrome and liver cirrhosis. The results demonstrated that puromycin- o...

  16. A rare association of Castleman′s disease and nephrotic syndrome

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    I Tazi

    2011-01-01

    Full Text Available Castleman′s Disease (CD is an uncommon and poorly understood disorder of lymph node hyperplasia of unknown etiology. This entity belongs to the atypical lymphoproliferative disorders, a heterogeneous group of diseases characterized by a hyperplastic reactive process involving the immune system. The association of the nephrotic syndrome and CD is extremely rare and their interrelation remains enigmatic. We report a case of CD of the hyaline-vascular type with unicentric localization complicated by nephrotic syndrome.

  17. Reciprocal Regulation of 11β-HSDs May Predict Steroid Sensitivity in Childhood Nephrotic Syndrome.

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    Sai, Shuji; Yamamoto, Masaki; Yamaguchi, Rie; Chapman, Karen E; Hongo, Teruaki

    2016-09-01

    Childhood nephrotic syndrome, in which steroid-dependence occurs concurrently with steroid-resistance, requires aggressive therapy to prevent relapse. Predictive biomarkers that can be used to stratify treatment are urgently needed. Here we report that reciprocal regulation of the glucocorticoid metabolizing enzymes, 11β-hydroxysteroid dehydrogenase types 1 and 2, is associated with steroid-responsiveness and disease remission in childhood nephrotic syndrome, potentially providing a marker to identify patients in which aggressive therapy is required.

  18. Toxic epidermal necrolysis associated with deflazacort therapy with nephrotic syndrome

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    Eun Chae Lee

    2014-12-01

    Full Text Available Toxic epidermal necrolysis (TEN is a drug-related fatal disease. Extensive necrosis of the epidermis can lead to serious complications. This report describes two cases of TEN, associated with deflazacort (DFZ, in two boys, aged 4 years and 14 years, with nephrotic syndrome (NS. The 14-year-old male teenager received DFZ following NS relapse. After 17 days, pruritic papules appeared on the lower extremities. Another case involved a 4-year-old boy receiving DFZ and enalapril. After a 41-day DFZ treatment period, erythematous papules appeared on the palms and soles. Within 3 days, both boys developed widespread skin lesions (>50% and were admitted to the intensive care unit for resuscitative and supportive treatment. The patients showed improvement after intravenous immunoglobulin-G therapy. Owing to the rapid, fatal course of TEN, clinicians need to be aware of the adverse effects of this drug when treating cases of NS.

  19. Mesangioproliferative glomerulonephritis: An important glomerulonephritis in nephrotic syndrome of young adult

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    Usha

    2008-07-01

    Full Text Available Mesangioproliferative glomerulonephritis (MesPGN consists 10% of the total renal biopsy of glomerulonephritis. Aim of the present study was to find out clinicopathological changes in MesPGN and differences between diffuse and focal variety. MesPGN was seen mostly in young adults with mean age of 28.63 years for males and 26.3 years for females. Male predominance was noted (M:F ratio - 1.4:1. About 70.83% patient presented with edema feet, followed by hypertension (29.19%, fever (16.66%, oliguria, nausea and vomiting (10.41%. Urine analysis in 50 patients revealed that 70% patients presented with nephrotic-range proteinuria, 36% patients with microscopic hematuria and 56% patients with leukocyturia. Statistically, no significant difference was found in clinical features of diffuse and focal MesPGN. Microscopic comparison between diffuse and focal variety showed that significant increase of focal glomerular basement membrane thickening, focal endothelial cell proliferation, focal smooth muscle hyperplasia, hyaline sclerosis and vasculitis was more common in diffuse variety. In focal variety, Capillary loop congestion, periglomerulitis, cloudy swelling and vacuolar degeneration in tubules were significantly more as compared to diffuse variety. Details of the clinical features, special laboratory tests and histological details revealed that diffuse variety had systemic diseases, which included Wegner′s granulomatosis, microscopic polyangitis, Henoch′s schonlein purpura, systemic lupus erythematosus (two cases and one case each of Kimura′s disease, pyelonephritis and tuberculosis. Only one case of focal MesPGN showed tuberculosis. Thus, our study concludes that MesPGN is an important cause of nephrotic syndrome among young adults. Secondly, search for some other diseases should be made and thirdly, if biopsy shows focal mesangial cell proliferations in minimal change glomerulonephritis (MCGN, it should be diagnosed as focal MesPGN rather than

  20. Treatment of steroid resistant nephrotic syndrome in children

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    Kari Jameela

    2010-01-01

    Full Text Available Achieving remission in children with Steroid-Resistant Nephrotic Syndrome (SRNS could be difficult. Many immunosuppressive drugs are used with variable success rates. We have studied the response of children with SRNS who presented to our pediatric′s renal unit between 2002 and 2007 to various modalities of therapy. We included patients with no response to pred-nisolone (60 mg/M 2 /day after four weeks of therapy; all the patients had renal biopsy and follow-up duration for at least one year. We excluded patients with congenital nephrotic syndrome, lupus, or sickle cell disease. There were 31 (23 girls and 8 boys with F: M= 2.9:1; the mean age at presentation was 4.2 ± 3.2 children who fulfilled the inclusion criteria. The mean duration of follow up was 3.1 ± 1.6 years. Twenty children (65% achieved partial (6 children or complete (14 children remission. There were 16 children treated with cyclophosphamide either oral or intra-venous, and only 4 of them (25% achieved remission. Seven children received oral chlorambucil, and only2 of them (28.5% achieved remission; none of the children experienced side effects. Fifteen children received cyclosporine, and only eight of them (53% achieved remission. Six children developed gum hypertrophy and one had renal impairment, which was reversible after discontinuing the drug. Mycophonelate mofetil (MMF was used as the last option in 5 children, and 2 of them achieved complete remission. One child developed a systemic cytomegalovirus (CMV infection which indicated discontinuing the drug. Fourteen (45% children needed more than one immunosuppressive therapy. Three children progressed to end stage renal failure and required dialysis. We conclude that SRNS in children is a difficult disease with significant morbidity. However, remission is achievable with cyclosporine and other immunosuppressive agents. Treatment should be individualized according to the underlying histopathology, and clinical and social

  1. Disruption of PTPRO Causes Childhood-Onset Nephrotic Syndrome

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    Ozaltin, Fatih; Ibsirlioglu, Tulin; Taskiran, Ekim Z.; Baydar, Dilek Ertoy; Kaymaz, Figen; Buyukcelik, Mithat; Kilic, Beltinge Demircioglu; Balat, Ayse; Iatropoulos, Paraskevas; Asan, Esin; Akarsu, Nurten A.; Schaefer, Franz; Yilmaz, Engin; Bakkaloglu, Ayşin

    2011-01-01

    Idiopathic nephrotic syndrome (INS) is a genetically heterogeneous group of disorders characterized by proteinuria, hypoalbuminemia, and edema. Because it typically results in end-stage kidney disease, the steroid-resistant subtype (SRNS) of INS is especially important when it occurs in children. The present study included 29 affected and 22 normal individuals from 17 SRNS families; genome-wide analysis was performed with Affymetrix 250K SNP arrays followed by homozygosity mapping. A large homozygous stretch on chromosomal region 12p12 was identified in one consanguineous family with two affected siblings. Direct sequencing of protein tyrosine phosphatase receptor type O (PTPRO; also known as glomerular epithelial protein-1 [GLEPP1]) showed homozygous c.2627+1G>T donor splice-site mutation. This mutation causes skipping of the evolutionarily conserved exon 16 (p.Glu854_Trp876del) at the RNA level. Immunohistochemistry with GLEPP1 antibody showed a similar staining pattern in the podocytes of the diseased and control kidney tissues. We used a highly polymorphic intragenic DNA marker—D12S1303—to search for homozygosity in 120 Turkish and 13 non-Turkish individuals in the PodoNet registry. This analysis yielded 17 candidate families, and a distinct homozygous c.2745+1G>A donor splice-site mutation in PTPRO was further identified via DNA sequencing in a second Turkish family. This mutation causes skipping of exon 19, and this introduces a premature stop codon at the very beginning of exon 20 (p.Asn888Lysfs∗3) and causes degradation of mRNA via nonsense-mediated decay. Immunohistochemical analysis showed complete absence of immunoreactive PTPRO. Ultrastructural alterations, such as diffuse foot process fusion and extensive microvillus transformation of podocytes, were observed via electron microscopy in both families. The present study introduces mutations in PTPRO as another cause of autosomal-recessive nephrotic syndrome. PMID:21722858

  2. High Steroid Sensitivity among Children with Nephrotic Syndrome in Southwestern Nigeria

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    Taiwo Augustina Ladapo

    2014-01-01

    Full Text Available Recent reports from both Caucasian and black populations suggest changes in steroid responsiveness of childhood nephrotic syndrome. This study was therefore undertaken to determine the features and steroid sensitivity pattern of a cohort of black children with nephrotic syndrome. Records of children managed for nephrotic syndrome from January 2008 to April 2013 were reviewed. Details including age, response to treatment, and renal histology were analysed. There were 108 children (median age: 5.9 years, peak: 1-2 years, 90.2% of whom had idiopathic nephrotic syndrome. Steroid sensitivity was 82.8% among children with idiopathic nephrotic syndrome but 75.9% overall. Median time to remission was 7 days. Median age was significantly lower in steroid sensitive compared with resistant patients. The predominant histologic finding in resistant cases was focal segmental glomerulosclerosis (53.3%. No cases of quartan malaria nephropathy or hepatitis B virus nephropathy were diagnosed. Overall mortality was 6.5%. In conclusion, unusually high steroid sensitivity is reported among a cohort of black children. This is likely attributable to the lower age structure of our cohort as well as possible changing epidemiology of some other childhood diseases. Surveillance of the epidemiology of childhood nephrotic syndrome and corresponding modifications in practice are therefore recommended.

  3. Children with Steroid-resistant Nephrotic Syndrome: a Single-Center Study

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    Rahime Renda

    2016-01-01

    Full Text Available Background and Aim: Steroid-resistant nephrotic syndrome (SRNS accounts for 10%-20% of all cases of idiopathic nephrotic syndrome. These patients are at risk of developing end-stage renal disease. The aim of this study was to determine the demographic characteristics, renal biopsy findings, response to immunosuppressive treatment, and prognosis in pediatric patients with SRNS.Materials and Methods: This retrospective study included 31 patients diagnosed as primary SRNS. Age at first episode, gender, parental consanguinity, and familial history of nephrotic syndrome were recorded. Demographic characteristics, renal biopsy findings, response to immunosuppressive treatment, and prognosis were analyzed, as were the number of and treatment of relapses, extra-renal manifestations, and complications of disease and treatment.Results: Mean age at first episode of nephrotic syndrome was 4,1±2,9 years. At the end of the first immunosuppressive treatment cycle, 14 (51.8% patients achieved complete remission, 4 (14.8% patients achieved partial remission, and 9 patients (33.3% did not achieve remission. Analysis of the final status of the patients showed that 16 patients (51.6% developed remission, 5 patients (16% continued to have nephrotic range proteinuria and 10 patients (32% developed chronic renal failure (CRF.Conclusion: The treatment of SRNS remains controversial. Early genetic testing can help the inevitable immunosuppressive treatments which may not be effective and have several side effects. Calcineurin inhibitors and mycophenolate mofetil are known to be effective immunosuppressive drugs for treating steroid resistant nephrotic syndrome .

  4. Successful treatment of dwarfism secondary to long-term steroid therapy in steroid-dependent nephrotic syndrome.

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    Sun, Linlin; Chen, Dongping; Zhao, Xuezhi; Xu, Chenggang; Mei, Changlin

    2010-01-01

    Prolonged steroid therapy is generally used for steroid-dependent nephrotic syndrome in pediatric patients. However, dwarfism secondary to a long-term regimen and its successful reverse is rarely reported. The underlying mechanism of dwarfism is still poorly understood, as both long-term steroid use and nephrotic syndrome may interact or independently interfere with the process of growth. Here, we present a 17-year-old patient with dwarfism and steroid-dependent nephrotic syndrome and the successful treatment by recombinant human growth factor and cyclosporine A with withdrawal of steroid. We also briefly review the current understanding and the management of dwarfism in pediatric patients with nephrotic syndrome.

  5. CD80, suPAR and nephrotic syndrome in a case of NPHS2 mutation.

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    Cara-Fuentes, Gabriel; Araya, Carlos; Wei, Changli; Rivard, Christopher; Ishimoto, Takuji; Reiser, Jochen; Johnson, Richard J; Garin, Eduardo H

    2013-01-01

    Podocin mutations are characterized by progression to end stage renal disease and histologic findings of Focal Segmental Glomerulosclerosis (FSGS). CD80 is a podocytes protein that may play a role in proteinuria, particularly in Minimal Change Disease whereas the soluble urokinase receptor (suPAR) is characteristically elevated in the serum of FSGS patients. In a patient with nephrotic syndrome and podocin mutation, urinary and serum CD80 as well as suPAR were measured using commercially available kits. Urinary CD80 molecular size was determined by western blot analysis. Glomerular staining for CD80 and podocin was performed. Patient displayed marked elevated CD80 and mildly increased suPAR urinary levels compared to controls. Serum CD80 level was within the range observed in normal controls. Serum suPAR level was elevated, albeit in the lower range reported for patients with primary FSGS. Immunofluorescence examination of kidney biopsy revealed glomerular CD80 expression. The combination of serum and urinary biomarkers can help differentiate various forms of FSGS. High urinary CD80 and elevated serum and urinary suPAR might represent a profile to differentiate this genetic form of FSGS from primary FSGS.

  6. Rituximab in adult minimal change disease and focal segmental glomerulosclerosis.

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    Kronbichler, Andreas; Bruchfeld, Annette

    2014-01-01

    Treatment of nephrotic syndrome due to minimal change disease and focal segmental glomerulosclerosis remains a challenge since steroid dependence, steroid resistance and a relapsing disease course exhibits a high cumulative steroid dosage. The necessity of using alternative steroid-sparing immunosuppressive agents with potential toxic side effects also restricts their long-term use. Rituximab, a monoclonal antibody targeting CD20, has been increasingly used in the therapy of difficult-to-treat nephrotic syndrome. A clinical response has been shown for patients with steroid-dependent or frequently relapsing nephrotic syndrome, whereas the benefit seems to be limited in steroid-resistant patients, especially those with underlying focal segmental glomerulosclerosis. No potentially life-threatening adverse events have been observed in the treatment of adult minimal change disease and focal segmental glomerulosclerosis following rituximab administration. Since most reports are retrospective and evidence of efficacy is derived from small case series, more prospective trials in a controlled, randomized manner are highly desirable to delineate the use of rituximab or other B cell-depleting agents in steroid-dependent, frequently relapsing or steroid-resistant patients.

  7. The Role of Proprotein Convertase Subtilisin/Kexin Type 9 in Nephrotic Syndrome-Associated Hypercholesterolemia.

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    Haas, Mary E; Levenson, Amy E; Sun, Xiaowei; Liao, Wan-Hui; Rutkowski, Joseph M; de Ferranti, Sarah D; Schumacher, Valerie A; Scherer, Philipp E; Salant, David J; Biddinger, Sudha B

    2016-07-05

    In nephrotic syndrome, damage to the podocytes of the kidney produces severe hypercholesterolemia for which novel treatments are urgently needed. PCSK9 (proprotein convertase subtilisin/kexin type 9) has emerged as an important regulator of plasma cholesterol levels and therapeutic target. Here, we tested the role of PCSK9 in mediating the hypercholesterolemia of nephrotic syndrome. PCSK9 and plasma lipids were studied in nephrotic syndrome patients before and after remission of disease, mice with genetic ablation of the podocyte (Podocyte Apoptosis Through Targeted Activation of Caspase-8, Pod-ATTAC mice) and mice treated with nephrotoxic serum (NTS), which triggers immune-mediated podocyte damage. In addition, mice with hepatic deletion of Pcsk9 were treated with NTS to determine the contribution of PCSK9 to the dyslipidemia of nephrotic syndrome. Patients with nephrotic syndrome showed a decrease in plasma cholesterol and plasma PCSK9 on remission of their disease (Phypercholesterolemia and a 7- to 24-fold induction in plasma PCSK9. The induction of plasma PCSK9 appeared to be attributable to increased secretion of PCSK9 from the hepatocyte coupled with decreased clearance. Interestingly, knockout of Pcsk9ameliorated the effects of NTS on plasma lipids. Thus, in the presence of NTS, mice lacking hepatic Pcsk9 showed a 40% to 50% decrease in plasma cholesterol and triglycerides. Moreover, the ability of NTS treatment to increase the percentage of low-density lipoprotein-associated cholesterol (from 9% in vehicle-treated Flox mice to 47% after NTS treatment), was lost in mice with hepatic deletion of Pcsk9 (5% in both the presence and absence of NTS). Podocyte damage triggers marked inductions in plasma PCSK9, and knockout of Pcsk9 ameliorates dyslipidemia in a mouse model of nephrotic syndrome. These data suggest that PCSK9 inhibitors may be beneficial in patients with nephrotic syndrome-associated hypercholesterolemia. © 2016 American Heart Association, Inc.

  8. Minimal change disease with acute renal failure: a case against the nephrosarca hypothesis.

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    Cameron, Mary Ann; Peri, Usha; Rogers, Thomas E; Moe, Orson W

    2004-10-01

    An unusual but well-documented presentation of minimal change disease is nephrotic proteinuria and acute renal failure. One pathophysiological mechanism proposed to explain this syndrome is nephrosarca, or severe oedema of the kidney. We describe a patient with minimal change disease who presented with heavy proteinuria and acute renal failure but had no evidence of renal interstitial oedema on biopsy. Aggressive fluid removal did not reverse the acute renal failure. Renal function slowly returned concomitant with resolution of the nephrotic syndrome following corticosteroid therapy. The time profile of the clinical events is not compatible with the nephrosarca hypothesis and suggests an alternative pathophysiological model for the diminished glomerular filtration rate seen in some cases of minimal change disease.

  9. Development of minimal-change glomerular disease and Hashimoto's thyroiditis during the treatment of sarcoidosis.

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    Ando, Fumiaki; Okado, Tomokazu; Sohara, Eisei; Rai, Tatemitsu; Uchida, Shinichi; Sasaki, Sei

    2013-11-01

    Minimal-change glomerular disease, sarcoidosis and autoimmune thyroid disease rarely occur in the same patient. We herein report a patient in which minimal-change glomerular disease and Hashimoto's thyroiditis developed during the treatment of sarcoidosis with steroids. A 66-year-old female was admitted to our hospital with symptoms of nephrotic syndrome. Nine months before admission, she was diagnosed as having ocular and pulmonary sarcoidosis, for which prednisolone at an initial dose of 40 mg/day was started. When the dose of prednisolone was tapered to 20 mg/day, she noticed swelling of the lower extremities. Examination confirmed the simultaneous occurrence of minimal-change glomerular disease and Hashimoto's thyroiditis, which were diagnosed based on kidney histology, ultrasonography of the thyroid gland and positive antithyroglobulin antibodies. We used intravenous methylprednisolone pulse therapy followed by 40 mg/day oral prednisolone. The patient achieved complete remission of nephrotic syndrome and steroids were tapered without relapse.

  10. Chronic graft-versus-host disease complicated by nephrotic syndrome.

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    Wang, Hsin-Hui; Yang, An-Hang; Yang, Ling-Yu; Hung, Giun-Yi; Chang, Jei-Wen; Wang, Chun-Kai; Lee, Tzong-Yann; Tang, Ren-Bin

    2011-09-01

    Chronic graft-versus-host disease (cGVHD) is one of the most frequent and serious complications of allogeneic hematopoietic stem cell transplantation (HSCT). Nephrotic syndrome (NS) is an uncommon and underrecognized manifestation of cGVHD. We report a patient who developed NS 18 months after allogeneic bone marrow transplantation. The onset of NS was accompanied by active manifestations of cGVHD, and immunosuppressants had not been tapered recently. Renal biopsy revealed membranous nephropathy. The patient failed to improve with three combined immunosuppressants (prednisolone, cyclosporine, and mycophenolate mofetil), but achieved partial remission after intravenous immunoglobulin (IVIG) infusion. Twenty-four months after the diagnosis of NS, the patient was still in hematological remission, with normal serum creatinine level, urinary protein loss of 0.7-1.9 g/day and mild oral mucositis. Our report suggests that NS can be a cGVHD-related immune disorder in HSCT patients. Monitoring of renal parameters, especially proteinuria, is important in cGVHD patients. Our case indicated that post-transplant NS, occurring without history of tapering or following immunosuppressant withdrawal, presents a more severe activity of cGVHD and a relatively severe clinical course. IVIG may modify and control the refractory GVHD-related NS, and can be one of the choices of treatment. Copyright © 2011. Published by Elsevier B.V.

  11. Myocardial Infarction in a Young Man With Nephrotic Syndrome.

    Science.gov (United States)

    Chu, Zhenliang; Zhu, Hang; Zhang, Bin; Jiang, Liqin

    2017-03-17

    A 26-year-old man diagnosed with nephrotic syndrome (NS) 2 years previously presented with chest pain. An electrocardiogram (ECG) performed at a local hospital showed ST-elevation in chest leads. Cardiac troponin-I was significantly positive. Echocardiography revealed mild regional wall-motion abnormalities in the heart apex. Seven days later, angiography (CAG) revealed a thrombus in the left anterior descending branch (LAD). Tirofiban was injected into the LAD for thromboclasis. ECG after CAG showed the ST-segment was much lower than before. The diagnosis after CAG was ST-segment elevation myocardial infarction (MI) and thrombogenesis in the LAD. He continued to receive antiplatelet and anticoagulation medication and atorvastatin after CAG, and was discharged 3 days later. MI is very rare in young males, but the incidence of MI is 8 times higher than normal in patients with NS. For young patients with MI, clinicians should pay more attention to the history of previous diseases with high risk of thromboembolism and they should actively promote prevention and the treatment of renal disease patients to reduce the incidence of complications of thromboembolism.

  12. Treatment approaches in children with steroid sensitive nephrotic syndrome

    Directory of Open Access Journals (Sweden)

    Süleyman Kalman

    2011-09-01

    Full Text Available Pediatric nephrotic syndrome (NS is a chronic glomerular disorder, and if untreated, is associated with increased risk of life-threatening infectious, thromboembolism, lipid abnormalities, and malnutrition. The aim of the management of NS in children is to induce and maintain complete remission with resolution of proteinuria and edema without encountering serious adverse effects of therapy. Over 90% of cases in children with idiopathic NS and a majority of them will respond to steroid therapy. However, a substantial number of patients relapse frequently and become steroid dependent. The occurrence of frequent relapses necessitates clear therapeutic strategies in order to maintain sustained remission and minimize steroid toxicity. Numerous therapeutic regimens have been proposed utilizing steroid sparing agents such as alkylating agents, principally, cyclophosphamide and chlorambucil, calcineurin inhibitors namely cyclosporin A and an immunomodulatory drug, levamisole, with variable success and associated side-effects. Recently, mycophenolate mofetil (MMF, tacrolimus, the anti-CD20 antibody, rituximab, have emerged as new therapeutic options for the management of steroid dependent NS in a few uncontrolled clinical trials. It is therefore important that the benefits and risks of these agents are weighed before considering their use in the treatment of patients with NS.

  13. [Nephrotic syndrome as the first manifestation of juvenile systemic scleroderma.

    Science.gov (United States)

    Couto, Saulo B; Sallum, Adriana M; Henriques, Luciana S; Malheiros, Denise M; Silva, Clovis A; Vaisbich, Maria H

    2014-10-22

    Renal involvement occurs in 1%-12% in juvenile systemic sclerosis (JSSc) patients, mainly with arterial hypertension, proteinuria and scleroderma renal crisis. We report herein a patient who presented nephrotic syndrome (NS) as the first manifestation of JSSc with focal segmental glomerulosclerosis (FSGS). A female patient presented steroid-sensitive NS at the age of 12 years. At 14 years, she had orbital and lower limbs edema, arterial hypertension, sclerodactyly and proximal skin sclerosis. Moderate capillary dilation and mild focal devascularization were observed in nailfold capillaroscopy, compatible with early stage of scleroderma (scleroderma pattern). Percutaneous renal biopsy guided by ultrasound revealed focal segmental glomerulosclerosis and direct immunofluorescence were negative. Therefore, she fullfilled the provisional classification criteria for JSSc. Patient was treated with oral 25-hydroxyvitamin D (800 IU/day), methotrexate (0.5mg/kg/week) and amlodipin (0.15 mg/kg). Prednisone (60 mg/m(2)/day) was administered for 4 consecutive weeks, followed by alternate-day (40mg/m(2)) for 2 consecutive months, with tapering for 4 months and then stopping this medication. Currently she is being treated with methotrexate 15 mg/week, without edema and proteinuria. In conclusion, we reported a rare case of NS with FSGS as the first manifestation of scleroderma. Therefore, renal biopsy is mandatory in JSSc patients with sustained proteinuria or NS.

  14. Chronic graft-versus-host disease complicated by nephrotic syndrome

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    Hsin-Hui Wang

    2011-09-01

    Full Text Available Chronic graft-versus-host disease (cGVHD is one of the most frequent and serious complications of allogeneic hematopoietic stem cell transplantation (HSCT. Nephrotic syndrome (NS is an uncommon and underrecognized manifestation of cGVHD. We report a patient who developed NS 18 months after allogeneic bone marrow transplantation. The onset of NS was accompanied by active manifestations of cGVHD, and immunosuppressants had not been tapered recently. Renal biopsy revealed membranous nephropathy. The patient failed to improve with three combined immunosuppressants (prednisolone, cyclosporine, and mycophenolate mofetil, but achieved partial remission after intravenous immunoglobulin (IVIG infusion. Twenty-four months after the diagnosis of NS, the patient was still in hematological remission, with normal serum creatinine level, urinary protein loss of 0.7–1.9 g/day and mild oral mucositis. Our report suggests that NS can be a cGVHD-related immune disorder in HSCT patients. Monitoring of renal parameters, especially proteinuria, is important in cGVHD patients. Our case indicated that post-transplant NS, occurring without history of tapering or following immunosuppressant withdrawal, presents a more severe activity of cGVHD and a relatively severe clinical course. IVIG may modify and control the refractory GVHD-related NS, and can be one of the choices of treatment.

  15. Sustained complete remission of steroid- and cyclophosphamide-resistant minimal-change disease with a single course of rituximab therapy.

    Science.gov (United States)

    Janardan, Jyotsna; Ooi, Khai; Menahem, Solomon

    2014-06-01

    We report a case of steroid- and cyclophosphamide-resistant nephrotic syndrome secondary to minimal-change disease occurring in an otherwise healthy 19-year-old female, responding rapidly to two doses of rituximab therapy. Complete disease remission has been sustained up to last follow-up (32 months) despite CD19 recovery. Literature review suggests emerging evidence that rituximab may have a role to play in recurrent and/or refractory minimal-change disease.

  16. Sustained complete remission of steroid- and cyclophosphamide-resistant minimal-change disease with a single course of rituximab therapy

    OpenAIRE

    Janardan, Jyotsna; Ooi, Khai; Menahem, Solomon

    2014-01-01

    We report a case of steroid- and cyclophosphamide-resistant nephrotic syndrome secondary to minimal-change disease occurring in an otherwise healthy 19-year-old female, responding rapidly to two doses of rituximab therapy. Complete disease remission has been sustained up to last follow-up (32 months) despite CD19 recovery. Literature review suggests emerging evidence that rituximab may have a role to play in recurrent and/or refractory minimal-change disease.

  17. Sustained complete remission of steroid- and cyclophosphamide-resistant minimal-change disease with a single course of rituximab therapy

    OpenAIRE

    Janardan, Jyotsna; Ooi, Khai; Menahem, Solomon

    2014-01-01

    We report a case of steroid- and cyclophosphamide-resistant nephrotic syndrome secondary to minimal-change disease occurring in an otherwise healthy 19-year-old female, responding rapidly to two doses of rituximab therapy. Complete disease remission has been sustained up to last follow-up (32 months) despite CD19 recovery. Literature review suggests emerging evidence that rituximab may have a role to play in recurrent and/or refractory minimal-change disease.

  18. Rituximab in minimal change nephropathy and focal segmental glomerulosclerosis: report of four cases and review of the literature.

    NARCIS (Netherlands)

    Peters, H.P.E.; Kar, NC van de; Wetzels, J.F.M.

    2008-01-01

    Minimal change nephropathy (MCNS) and focal segmental glomerulosclerosis (FSGS) are the main causes of the idiopathic nephrotic syndrome. MCNS usually responds to steroids and the long-term prognosis is generally good. However, some patients require prolonged treatment with immunosuppressive agents.

  19. Nephrotic syndrome and multiple tubular defects in children: an early sign of focal segmental glomerulosclerosis.

    Science.gov (United States)

    McVicar, M; Exeni, R; Susin, M

    1980-12-01

    The nephrotic syndrome is rarely associated with renal tubular defects, and the combination has been reported only in association with advanced renal insufficiency. We report here five children with nephrotic syndrome and multiple tubular defects which evolved when glomular filtration rate ranged between 56 and 90 ml/minute/1.73 m2. The tubular defects were first noted at 3, 4, 4, 7, and 22 months after the onset of the nephrotic syndrome, and renal glycosuria was the first sign in all five children. Glycosuria was intermittent in three patients, constant in two, and ceased with loss of kidney function. Four patients had hyperaminoaciduria and renal tubular acidosis (two of four tested had distal renal tubular acidosis). Three patients had decreased tubular reabsorption of phosphorus and defective maximum concentrating capacity. All five had focal segmental glomerulosclerosis proven by renal biopsy. Over a follow-up period of seven years, all of the children have developed advanced renal insufficiency, four of the five have required dialysis or transplantation within 21 to 72 months after onset, and one has stabilized renal function at 35 ml/minute/1.73 m2. The one patient receiving a kidney transplant has had recurrence of focal segmental glomerulosclerosis in the transplanted kidney and became nephrotic with three subsequent transplants. Our experience suggests that the nephrotic syndrome associated with tubular defects in children forms a subgroup of focal segmental glomerulosclerosis, with rapid progression to renal insufficiency and the potential for recurrence of the lesion in the transplanted kidney.

  20. Neuromyelitis optica accompanied by nephrotic syndrome and autoimmune-related pancytopenia.

    Science.gov (United States)

    ZhangBao, Jingzi; Zhou, Lei; Lu, Jiahong; Xi, Jianying; Zhao, Chongbo; Quan, Chao

    2016-05-01

    Neuromyelitis optica (NMO) associated with nephrotic syndrome and autoimmune-related pancytopenia has not been reported previously. We report herein a young woman who initially presented with bilateral blurring of vision and numbness in her hands. MRI disclosed multiple white matter lesions and a long cervical spinal cord lesion extending to the medulla oblongata. Serum aquaporin-4 antibody was positive and the patient was diagnosed with NMO. While in the hospital, she presented with hypoproteinemia and heavy proteinuria, meeting the diagnostic criteria of nephrotic syndrome. After high-dose methylprednisolone treatment, her vision improved significantly and urine protein quantity decreased. However, the patient subsequently developed severe pancytopenia with a positive Coombs' test. Thrombocytopenia finally led to uncontrollable gastrointestinal bleeding as the direct cause of the patient's death. This case illustrates the extremely rare condition of concurrence of NMO, nephrotic syndrome, and autoimmune pancytopenia in one patient, which suggests the involvement of organs beyond the central nervous system in NMO spectrum disorders.

  1. Assessment on Application of Chinese Herbal Medicine in the Process of Corticosterone Reduction in Nephrotic Syndrome

    Institute of Scientific and Technical Information of China (English)

    2001-01-01

    Objective: To evaluate the effect of Chinese herbal medicine (CHM) in the process of corticosterone (CS) reduction in nephrotic syndrome. Methods: One hundred and twenty patients of idiopathic nephrotic syndrome sensitive to prednisone treatment were divided into two groups randomly. The dosage of prednisone used was conventionally reduced in both groups but CHM herbal treatment was added to the treated group in the period of prednisone reduction. The effect of CHM was estimated by observing the recurrence rate of disease and side-effects of prednisone. Results: The recurrence rate of disease and the occurrence rate of side-effects of prednisone in the treated group were less than those in the control group significantly (P<0.01). Conclusion: Additional CHM in the process of CS reduction in treating CS sensitive nephrotic syndrome could markedly abate the recurrence rate of disease and the side-effect of CS reduction.

  2. Cyclosporine therapy monitored with abbreviated area under curve in nephrotic syndrome.

    Science.gov (United States)

    Rinaldi, Stefano; Sesto, Antonella; Barsotti, Paola; Faraggiana, Tullio; Sera, Francesco; Rizzoni, Gianfranco

    2005-01-01

    Cyclosporin A (CsA) is an effective therapy for children with long-lasting nephrotic syndrome (NS). Long-term treatment can result in chronic CsA nephropathy (CsAN) and there is controversy concerning its incidence and severity. Trough levels are commonly used to monitor the drug concentration. We report a retrospective clinical and histological analysis of 18 children (12 males, 6 females) with steroid-dependent nephrotic syndrome (15 patients) and partially steroid-sensitive nephrotic syndrome (3 patients) treated with CsA for a long-term period (mean 4.9 years, range 2.2-6.9). Before CsA treatment all patients had normal creatinine clearance. CsA was started at a dose of 5 mg/kg per day administered orally in two divided doses and adjusted to maintain the mean CsA blood concentration between 250 and 350 ng/ml obtained from abbreviated area under the curve (AUC). A renal biopsy was performed after a mean period of 3.9 years (range 2.2-6.2) from the start of CsA treatment. Tubular, interstitial, and arteriolar lesions were evaluated in order to assess CsAN. The mean CsA dose and the mean CsA blood concentration were 4.4 mg/kg per day (range 3.6-5.8) and 276.6 ng/ml (range 162-346), respectively. No child had a worsening creatinine clearance during CsA treatment and follow-up after CsA discontinuation. If compared with the year before the start of CsA treatment, NS relapses and prednisone (PDN) dose significantly decreased during CsA treatment, 4/year versus 0.8/year (P <0.0001) and 0.9 mg/kg per day versus 0.2 mg/kg per day (P <0.0001), respectively. Histological analysis showed 15 patients with minimal change disease and 3 with focal segmental glomerulosclerosis. Clear-cut lesions diagnostic of CsAN were never found and only mild lesions were observed in 5 children (suggestive of CsAN in 2 patients and consistent with CsAN in 3 patients). Long-term CsA treatment is confirmed to be effective in preventing NS relapses and reducing PDN dose. Renal function is not a

  3. Rituximab is an efficient and safe treatment in adults with steroid-dependent minimal change disease.

    Science.gov (United States)

    Munyentwali, Helene; Bouachi, Khedidja; Audard, Vincent; Remy, Philippe; Lang, Philippe; Mojaat, Rachid; Deschênes, Georges; Ronco, Pierre M; Plaisier, Emmanuelle M; Dahan, Karine Y

    2013-03-01

    Development of steroid dependency in patients with nephrotic syndrome may require a long-term multi-drug therapy at risk of drug toxicity and renal failure. Rituximab treatment reduces the steroid dosage and the need for immunosuppressive therapy in pediatric patients. Here we retrospectively analyze the efficacy and safety of rituximab in adult patients with steroid-dependent minimal change disease. To do this, we analyzed the outcome of all adult patients treated with rituximab for steroid-dependent minimal change nephrotic syndrome over a mean follow-up of 29.5 months (range 5.1-82 months). Seventeen patients with steroid-dependent or frequently relapsing minimal change nephrotic syndrome, unresponsive to several immunosuppressive medications, were treated with rituximab. Eleven patients had no relapses after rituximab infusion (mean follow-up 26.7 months, range 5.1-82 months) and nine of them were able to come off all other immunosuppressive drugs and steroids during follow-up. Six patients relapsed at least once after a mean time of 11.9 months (mean follow-up 34.5 months, range 16.9-50.1 months), but their immunosuppressive drug treatment could be stopped or markedly reduced during this time. No adverse events were recorded. Thus, rituximab is efficient and safe in adult patients suffering from severe steroid-dependent minimal change disease. Prospective randomized trials are needed to confirm this study.

  4. Varicella vaccination in children with nephrotic syndrome: a report of the Southwest Pediatric Nephrology Study Group.

    Science.gov (United States)

    Furth, Susan L; Arbus, Gerald S; Hogg, Ronald; Tarver, Joyce; Chan, Christina; Fivush, Barbara A

    2003-02-01

    To evaluate the safety and immunogenicity of varicella vaccine in children with nephrotic syndrome, including those taking low-dose, alternate-day prednisone. Prospective, open-label, multicenter clinical trial of varicella vaccine in a 2-dose regimen in US and Canadian children (12 months to or=5 gpELISA units) after 2 doses. At 2-year follow-up, all patients retained detectable antibody, and 91% (21 of 23) had levels >or=5 gpELISA units. There were no adverse events associated with vaccination. Varicella vaccine was generally well tolerated and highly immunogenic in children with nephrotic syndrome, including those on low-dose, alternate-day prednisone.

  5. Anakinra induces complete remission of nephrotic syndrome in a patient with familial Mediterranean fever and amyloidosis

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    Ángel M. Sevillano

    2016-01-01

    Full Text Available Renal amyloidosis is one of the most severe complications of familial Mediterranean fever (FMF. Colchicine has reduced the incidence of this complication, which now only appears in untreated, under-treated and resistant patients, but it is usually ineffective in patients with advanced amyloidosis. Here we report a patient with FMF and biopsy-proven amyloidosis who presented with nephrotic syndrome despite colchicine treatment. Anakinra (an interleukin-1β inhibitor was started and a dramatic complete remission of nephrotic syndrome was observed in the following months. Anakinra can be an effective treatment for FMF patients with severe secondary amyloidosis.

  6. Fulminant intracranial hypertension due to cryptococcal meningitis in a child with nephrotic syndrome

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    Leena Nagotkar

    2011-01-01

    Full Text Available Cryptococcus neoformans has a worldwide distribution. Meningoencephalitis is the most common manifestation of cryptococcosis. The outcome of a patient with cryptococcal infection depends on the immune status of the host. Patients with nephrotic syndrome are particularly susceptible to cryptococcal infection not only due to innate changes in their immune system but also because of the immunosuppressive agents used in the treatment. We report an 8-year-old boy with nephrotic syndrome, who developed cryptococcal meningitis and died of fulminant intracranial hypertension.

  7. Cardiac dysfunction in HgCl2-induced nephrotic syndrome.

    Science.gov (United States)

    Moreira-Rodrigues, Mónica; Henriques-Coelho, Tiago; Moura, Cláudia; Vasques-Nóvoa, Francisco; Sampaio-Maia, Benedita; Pestana, Manuel; Leite-Moreira, Adelino F

    2010-03-01

    The experimental model of HgCl(2) injection is characterized by a systemic autoimmune disease which leads to the development of nephrotic syndrome (NS). NS seems to be accompanied by cardiovascular alterations, since patients with NS present an increased incidence in cardiac disease. The aim of our work was to study the effects of HgCl(2)-induced NS on myocardial function and morphometry. Normotensive Brown-Norway rats were injected with HgCl(2) (1 mg/kg, HgCl(2) group; n = 6, subcutaneous) or the vehicle (control group; n = 6, subcutaneous) on days 0, 2, 4, 7, 9 and 11. The animals were placed in metabolic cages for evaluation of urinary excretion of noradrenaline, sodium, total proteins, albumin and creatinine. Fourteen and 21 days after the first HgCl(2) injection, left ventricle (LV) hemodynamics was evaluated through pressure micromanometers in basal and isovolumetric heartbeats. The heart and gastrocnemius muscle weights and tibial length were also examined. In an additional group of animals cardiac dimensions and ejection fraction were assessed by echocardiography and LV apoptosis and fibrosis were studied. HgCl(2)-injected rats presented proteinuria, albuminuria, hyperlipidemia, anemia, sodium retention and ascites at day 14. These alterations were accompanied by LV hemodynamic changes only in isovolumetric heartbeats. Similarly, on day 21, HgCl(2)-injected rats presented proteinuria, albuminuria, hyperlipidemia, anemia, but no sodium retention or ascites. These animals presented LV systolic and diastolic dysfunction in both basal and isovolumetric heartbeats, as well as cardiac atrophy, LV fibrosis and an increase in myocyte apoptosis. In conclusion, HgCl(2)-induced NS is accompanied by LV dysfunction and can be a promising model for studying the link between NS and cardiac disease.

  8. Atrial natriuretic factor in the acute nephritic and nephrotic syndromes.

    Science.gov (United States)

    Rodríguez-Iturbe, B; Colic, D; Parra, G; Gutkowska, J

    1990-09-01

    Because the role of systemic hormones in the pathophysiology of edema in acute renal disease remains incompletely understood, we compared the levels of atrial natriuretic factor (ANF) and plasma renin activity (PRA) in patients with acute glomerulonephritis (AGN), nephrotic syndrome (NS), and normal individuals during salt deprivation and salt loading. Sixteen patients with AGN (10 males) and nine patients with NS and hypoalbuminemia (7 males) were studied on admission, and after recovery (12 AGN patients) or remission (4 NS patients). Eighteen normal controls were each studied after five days on a low (20 mEq Na/day), regular (120 mEq Na/day) and high (300 mEq Na/day) dietary salt intake. Patients with AGN and NS had comparable edema (AGN 2.8 +/- 0.53 kg; NS 3.36 +/- 0.47 kg; SE) and urinary Na excretion (mean +/- SEM: AGN 0.97 +/- 0.11 mEq/hr; NS 1.06 +/- 0.16 mEq/hr), but AGN patients had five times higher ANF (AGN 27.2 +/- 4.06 fmol/ml; NS 5.51 +/- 1.02 fmol/ml; P less than 0.001) and six times lower PRA ng/liter.sec levels (AGN 0.187 +/- 0.047; NS 1.144 +/- 0.222; P less than 0.001) than NS patients. The degree of edema was correlated with ANF levels in AGN patients (P less than 0.001) but not in NS patients. There was a strong exponential negative correlation (r = -0.773, P less than 0.0001) between ANF and PRA, in which AGN patients and Na-restricted controls were located in the opposite ends of the volume sensing-response, and NS patients in the middle, alongside controls with regular Na intake.(ABSTRACT TRUNCATED AT 250 WORDS)

  9. Serum osteoprotegerin (OPG in children with primary nephrotic syndrome

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    Gamal B Mohamed

    2011-01-01

    Full Text Available A novel cytokine system secreted by osteoblast, osteoprotegerin (OPG and its ligand (OPGL regulates osteoclastogenesis. To determine the relation of the serum OPG levels in children with nephrotic syndrome (NS to the renal disease, we studied 30 patients with NS in comparison with 30 healthy children serving as controls. The study patients were divided into three equal groups: group 1 included newly diagnosed patients who were studied before and after a short course (one month of steroid therapy for the first time, group 2 included frequent relapsers (FR, and group 3 included infrequent relapsers (IFR. In addition to serum OPG (ELISA, osteocalcin (OC, parathormone (PTH, alkaline phosphatase (ALP, and 24- hour urinary Ca and proteins were measured. The NS patients revealed a significantly lower serum OPG and parameters of bone formation (ALP and OC and a significantly higher 24- hour urinary Ca than controls. A short course of glucocorticoids therapy for one month resulted in a significant decrease of serum OPG, ALP and OC levels and a significant increase of 24- hour urinary Ca, while serum PTH levels were not significantly affected by this the- rapy; the FR revealed a significantly lower serum level and a significantly higher 24- hour urinary Ca and serum PTH than the IFR. OPG had significant negative correlations with markers of disease activity and severity (ESR, serum cholesterol, 24- hour urinary protein and cumulative steroid dose, PTH and 24- hour urinary Ca. On the other hand, OPG had significant positive correlations with ALP, OC, and serum albumin. Low serum OPG, which is attributed to the renal disease and/or steroid therapy, may be an important factor contributing to bone resorption in NS. Studies of the protective effect of OPG administration against bone loss in NS are warranted.

  10. Nephroprotective effect of heparanase in experimental nephrotic syndrome.

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    Suheir Assady

    Full Text Available Heparanase, an endoglycosidase that cleaves heparan sulfate (HS, is involved in various biologic processes. Recently, an association between heparanase and glomerular injury was suggested. The present study examines the involvement of heparanase in the pathogenesis of Adriamycin-induced nephrotic syndrome (ADR-NS in a mouse model.BALB/c wild-type (wt mice and heparanase overexpressing transgenic mice (hpa-TG were tail-vein injected with either Adriamycin (ADR, 10 mg/kg or vehicle. Albuminuria was investigated at days 0, 7, and 14 thereafter. Mice were sacrificed at day 15, and kidneys were harvested for various analyses: structure and ultrastructure alterations, podocyte proteins expression, and heparanase enzymatic activity.ADR-injected wt mice developed severe albuminuria, while ADR-hpa-TG mice showed only a mild elevation in urinary albumin excretion. In parallel, light microscopy of stained cross sections of kidneys from ADR-injected wt mice, but not hpa-TG mice, showed mild to severe glomerular and tubular damage. Western blot and immunofluorescence analyses revealed significant reduction in nephrin and podocin protein expression in ADR-wt mice, but not in ADR-hpa-TG mice. These results were substantiated by electron-microscopy findings showing massive foot process effacement in injected ADR-wt mice, in contrast to largely preserved integrity of podocyte architecture in ADR-hpa-TG mice.Our results suggest that heparanase may play a nephroprotective role in ADR-NS, most likely independently of HS degradation. Moreover, hpa-TG mice comprise an invaluable in vivo platform to investigate the interplay between heparanase and glomerular injury.

  11. Statins in nephrotic syndrome: a new weapon against tissue injury.

    Science.gov (United States)

    Buemi, Michele; Nostro, Lorena; Crascì, Eleonora; Barillà, Antonio; Cosentini, Vincenzo; Aloisi, Carmela; Sofi, Tito; Campo, Susanna; Frisina, Nicola

    2005-11-01

    The nephrotic syndrome is characterized by metabolic disorders leading to an increase in circulating lipoproteins levels. Hypertriglyceridemia and hypercholesterolemia in this case may depend on a reduction in triglyceride-rich lipoproteins catabolism and on an increase in hepatic synthesis of Apo B-containing lipoproteins. These alterations are the starting point of a self-maintaining mechanism, which can accelerate the progression of chronic renal failure. Indeed, hyperlipidemia can affect renal function, increase proteinuria and speed glomerulosclerosis, thus determining a higher risk of progression to dialysis. 3-hydroxy-3-methylglutaryl-coenzyme A (HMG-CoA) reductase is the rate-limiting enzyme in cholesterol synthesis from mevalonate and its inhibitors, or statins, can therefore interfere with the above-mentioned consequences of hyperlipidemia. Statins are already well known for their effectiveness on primary cardiovascular prevention, which cannot be explained only through their hypolipemic effect. As far as kidney diseases are concerned, statin therapy has been shown to prevent creatinine clearance decline and to slow renal function loss, particularly in case of proteinuria, and its favorable effect may depend only partially on the attenuation of hyperlipidemia. Statins may therefore confer tissue protection through lipid-independent mechanisms, which can be triggered by other mediators, such as angiotensin receptor blockers. Possible pathways for the protective action of statins, other than any hypocholesterolemic effect, are: cellular apoptosis/proliferation balance, inflammatory cytokines production, and signal transduction regulation. Statins also play a role in the regulation of the inflammatory and immune response, coagulation process, bone turnover, neovascularization, vascular tone, and arterial pressure. In this study, we would like to provide scientific evidences for the pleiotropic effects of statins, which could be the starting point for the

  12. Nephrotic Syndrome and Idiopathic Membranous Nephropathy Associated with Autosomal-Dominant Polycystic Kidney Disease

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    Ramón Peces

    2011-01-01

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

  13. Nephrotic syndrome and idiopathic membranous nephropathy associated with autosomal-dominant polycystic kidney disease.

    Science.gov (United States)

    Peces, Ramón; Martínez-Ara, Jorge; Peces, Carlos; Picazo, Mariluz; Cuesta-López, Emilio; Vega, Cristina; Azorín, Sebastián; Selgas, Rafael

    2011-05-05

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

  14. The association of nephrotic syndrome and renal vein thrombosis: a clinicopathological analysis of eight pediatric patients.

    Science.gov (United States)

    Tinaztepe, K; Buyan, N; Tinaztepe, B; Akkök, N

    1989-01-01

    Cases with a pathological diagnosis of renal venous thrombosis (RVT) associated with nephrotic syndrome (NS) were studied retrospectively for clinicopathological evaluation. The material consisted of 21 RVT cases which were diagnosed in 2000 consecutive pediatric necropsies, with an overall incidence of about one percent. Eight of the 21 RVT cases were associated with nephrotic syndrome (34%), and this group formed 0.4 percent of the total necropsies in our pediatric center. The glomerulopathies of these nephrotic patients consisted of three cases of Finnish-type congenital NS (FCNS), three cases of renal amyloidosis secondary to familial Mediterranean fever, and two cases of membranoproliferative glomerulonephritis (MPGN). The presence of sepsis associated with disseminated intravascular coagulation, and the morphological age of the thrombi suggested that the RVT was secondary to sepsis in the FCNS cases. In the MPGN and secondary renal amyloidosis cases, the long duration of both the nephrotic state and the administration of diuretics along with glucocorticoid treatment and also the newly formed thrombi without infarction are strong evidences, although not definite, that the RVT developed as a complication of the glomerulopathy. Even though there were no definite clinical criteria for the diagnosis of most of the RVT cases, we would like to emphasize the importance of flank pain, the rapid deterioration of renal functions in a stable nephrotic patient, as well as the hypercoagulable state in the consideration of the development of RVT which indicate the need for appropriate radiological studies for confirmation of this condition during life.

  15. Long term outcomes in children with steroid resistant nephrotic syndrome treated with calcineurin inhibitors

    Directory of Open Access Journals (Sweden)

    Nathan T Beins

    2015-11-01

    Full Text Available Background: Steroid resistant nephrotic syndrome (SRNS is an important cause of chronic kidney disease (CKD in children that often progresses to end-stage renal disease (ESRD. Calcineurin inhibitors (CNIs have been shown to be effective in inducing short-term remission in some patients with SRNS. However, there are little data examining their long-term impact on ESRD progression rates.Methods: We performed a retrospective chart review of all patients treated for SRNS with CNIs at our institution from 1995-2013. Data collected including demographics, initial response to medical therapy, number of relapses, progression to ESRD, and treatment complications.Results: A total of 16 patients met inclusion criteria with a mean follow-up of 6.6 years (range 0.6-17.6 years. Histopathological diagnoses were focal segmental glomerulosclerosis (8, mesangial proliferative glomerulonephritis (4, IgM nephropathy (3, and minimal change disease (1. Three patients (18.8% were unresponsive to CNIs while the remaining 13 (81.2% achieved remission with CNI therapy. Six patients (37.5% progressed to ESRD during the study period, three of whom did so after initially responding to CNI therapy. Renal survival rates were 87%, 71%, and 57% at 2 years, 5 years, and 10 years respectively. Non-Caucasian ethnicity was associated with progression to ESRD. Finally, a higher number of acute kidney injury episodes was associated with a lower final estimated glomerular filtration rate.Discussion: Despite the majority of SRNS patients initially responding to CNI therapy, a significant percentage still progressed to ESRD despite achieving short-term remission. Recurrent episodes of AKI may be associated with progression of CKD in SRNS patients with.

  16. Familial Mediterranean fever, Inflammation and Nephrotic Syndrome: Fibrillary Glomerulopathy and the M680I Missense Mutation

    Directory of Open Access Journals (Sweden)

    Semerdjian Ronald J

    2003-08-01

    Full Text Available Abstract Background Familial Mediterranean fever (FMF is an autosomal recessive disease characterized by inflammatory serositis (fever, peritonitis, synovitis and pleuritis. The gene locus responsible for FMF was identified in 1992 and localized to the short arm of chromosome 16. In 1997, a specific FMF gene locus, MEFV, was discovered to encode for a protein, pyrin that mediates inflammation. To date, more than forty missense mutations are known to exist. The diversity of mutations identified has provided insight into the variability of clinical presentation and disease progression. Case Report We report an individual heterozygous for the M680I gene mutation with a clinical diagnosis of FMF using the Tel-Hashomer criteria. Subsequently, the patient developed nephrotic syndrome with biopsy-confirmed fibrillary glomerulonephritis (FGN. Further diagnostic studies were unremarkable with clinical workup negative for amyloidosis or other secondary causes of nephrotic syndrome. Discussion Individuals with FMF are at greater risk for developing nephrotic syndrome. The most serious etiology is amyloidosis (AA variant with renal involvement, ultimately progressing to end-stage renal disease. Other known renal diseases in the FMF population include IgA nephropathy, IgM nephropathy, Henoch-Schönlein purpura as well as polyarteritis nodosa. Conclusion To our knowledge, this is the first association between FMF and the M680I mutation later complicated by nephrotic syndrome and fibrillary glomerulonephritis.

  17. Nephrotic Syndrome Associated with Lung Cancer: A Rare Case of Malignancy Associated with AA Amyloidosis.

    Science.gov (United States)

    Gueutin, Victor; Langlois, Anne-Lyse; Shehwaro, Nathalie; Elharraqui, Ryme; Rouvier, Philippe; Izzedine, Hassane

    2013-01-01

    Nonhematologic malignancies are rarely reported to be associated with AA amyloidosis. Although the association between renal cell carcinoma and systemic AA amyloidosis has been established, the evidence linking pulmonary cancer to AA amyloidosis is scarce. Here, a case of biopsy-proven renal AA amyloidosis complicated with nephrotic syndrome associated with lung carcinoma is reported.

  18. Nephrotic Syndrome Associated with Lung Cancer: A Rare Case of Malignancy Associated with AA Amyloidosis

    Directory of Open Access Journals (Sweden)

    Victor Gueutin

    2013-01-01

    Full Text Available Nonhematologic malignancies are rarely reported to be associated with AA amyloidosis. Although the association between renal cell carcinoma and systemic AA amyloidosis has been established, the evidence linking pulmonary cancer to AA amyloidosis is scarce. Here, a case of biopsy-proven renal AA amyloidosis complicated with nephrotic syndrome associated with lung carcinoma is reported.

  19. [Ocular toxocariasis in a boy with idiopathic nephrotic syndrome - a case report].

    Science.gov (United States)

    Antonowicz, Agnieszka; Skrzypczyk, Piotr; Kępa, Beata; Pańczyk-Tomaszewska, Małgorzata

    2016-10-19

    Toxocariasis is a common zoonosis caused by infection with Toxocara canis or cati larvae. Ocular toxocariasis is one of the forms of infestation found in 1/1,000 - 1/10,000 children. Children with idiopathic nephrotic syndrome (INS) are at high risk of infections, also parasitic infestations, which can, in turn, cause relapses of the disease.

  20. Population pharmacokinetics of levamisole in children with steroid-sensitive nephrotic syndrome

    NARCIS (Netherlands)

    Kreeftmeijer-Vegter, A.R.; Dorlo, T.P.C.; Gruppen, M.P.; De Boer, A.; De Vries, P.J.

    2015-01-01

    Aim The aim was to investigate the population pharmacokinetics of levamisole in children with steroid-sensitive nephrotic syndrome. Methods Non-linear mixed effects modelling was performed on samples collected during a randomized controlled trial. Samples were collected from children who were receiv

  1. Maternal Environment Interacts with Modifier Genes to Influence Progression of Nephrotic Syndrome

    Science.gov (United States)

    Ratelade, Julien; Lavin, Tiphaine Aguirre; Muda, Andrea Onetti; Morisset, Ludivine; Mollet, Géraldine; Boyer, Olivia; Chen, Deborah S.; Henger, Anna; Kretzler, Matthias; Hubner, Norbert; Théry, Clotilde; Gubler, Marie-Claire; Montagutelli, Xavier; Antignac, Corinne; Esquivel, Ernie L.

    2008-01-01

    Mutations in the NPHS2 gene, which encodes podocin, are responsible for some cases of sporadic and familial autosomal recessive steroid-resistant nephrotic syndrome. Inter- and intrafamilial variability in the progression of renal disease among patients bearing NPHS2 mutations suggests a potential role for modifier genes. Using a mouse model in which the podocin gene is constitutively inactivated, we sought to identify genetic determinants of the development and progression of renal disease as a result of the nephrotic syndrome. We report that the evolution of renal disease as a result of nephrotic syndrome in Nphs2-null mice depends on genetic background. Furthermore, the maternal environment significantly interacts with genetic determinants to modify survival and progression of renal disease. Quantitative trait locus mapping suggested that these genetic determinants may be encoded for by genes on the distal end of chromosome 3, which are linked to proteinuria, and on the distal end of chromosome 7, which are linked to a composite trait of urea, creatinine, and potassium. These loci demonstrate epistatic interactions with other chromosomal regions, highlighting the complex genetics of renal disease progression. In summary, constitutive inactivation of podocin models the complex interactions between maternal and genetically determined factors on the progression of renal disease as a result of nephrotic syndrome in mice. PMID:18385421

  2. Cortical sinovenous thrombosis in a child with nephrotic syndrome and iron deficiency anaemia.

    Directory of Open Access Journals (Sweden)

    Meena A

    2000-07-01

    Full Text Available Cortical sinovenous thrombosis in a child with nephrotic syndrome and iron deficiency anaemia is described. The most probable mechanism for the hypercoagulable state was thrombocytosis associated with iron deficiency anaemia. The other possible contributing factor might have been the diuretic therapy during the phase of relapse.

  3. Steroid-dependent nephrotic syndrome in lupus nephritis. Response to chlorambucil.

    Science.gov (United States)

    Abuelo, J G; Esparza, A R; Garella, S

    1984-12-01

    Nephrotic syndrome associated with mesangial lupus nephritis developed in a young woman. The heavy proteinuria exhibited a striking steroid-dependent course during a three-year period of time, with ten relapses occurring whenever attempts were made to withdraw prednisone therapy. A prolonged remission was induced by the administration of chlorambucil.

  4. High absolute risks and predictors of venous and arterial thromboembolic events in patients with nephrotic syndrome

    NARCIS (Netherlands)

    Mahmoodi, Bakhtawar K.; ten Kate, Min Ki; Waanders, Femke; Veeger, Nic J. G. M.; Brouwer, Jan-Leendert P.; Vogt, Liffert; Navis, Gerjan; van der Meer, Jan

    2008-01-01

    Background-No data are available on the absolute risk of either venous thromboembolism (VTE) or arterial thromboembolism (ATE) in patients with nephrotic syndrome. Reported risks are based on multiple case reports and small studies with mostly short-term follow-up. We assessed the absolute risk of V

  5. Statin use in patients with nephrotic syndrome is associated with a lower risk of venous thromboembolism

    NARCIS (Netherlands)

    Resh, Mohammad; Mahmoodi, Bakhtawar K.; Navis, Gerjan J.; Veeger, Nic J. G. M.; Lijfering, Willem M.

    2011-01-01

    Background: Nephrotic syndrome (NS) is a well-known risk factor for venous thromboembolism (VTE), however preventive measures are not routinely taken. In non-renal populations, statins are associated with lower risk of VTE. Hence, we set up this single-center retrospective cohort study to assess whe

  6. MAC-2BP is increased in plasma during nephrotic syndrome and is produced by PBMC

    DEFF Research Database (Denmark)

    Andersen, René Frydensbjerg; Kamperis, Konstantinos; Rittig, Søren

    OBJECTIVES: Edema is a hallmark of nephrotic syndrome (NS) and has largely been attributed to sodium retention. Yet low plasma sodium is frequently observed in children with acute NS suggesting that a disturbance in renal water handling may coexist together with sodium retention. The aim of the s...

  7. Vitamin D status is insufficient in the majority of children at diagnosis of nephrotic syndrome

    DEFF Research Database (Denmark)

    Nielsen, Cecilie Ane; Jensen, Jens-Erik Bech; Cortes, Dina

    2015-01-01

    INTRODUCTION: Children with nephrotic syndrome (NS) are treated for at least 12 weeks with high doses of prednisolone, which may be harmful to the bones. Vitamin D deficiency is also harmful to the bones. METHODS: This was a prospective study of consecutive children with first episode of NS...

  8. Infant Boy with Microcephaly Gastroesophageal Refl ux and Nephrotic Syndrome (Galloway-Mowat Syndrome): A Case Report.

    Science.gov (United States)

    Malaki, Majid; Rafeey, Mandana

    2012-01-01

    In this case report, we present the first diagnosed case of Galloway-Mowat syndrome in Iran. A 7 month old infant boy withmicrocephaly that had prominently stunted head growth afterbirth, gastroesophageal reflux, multiple craniofascial characters,hypothyroidism and nephrotic syndrome diagnosed at 5 monthsof age associated with rapid decline in renal function and heavyproteinuria in 2 months .

  9. Nephritic-nephrotic syndrome as a presentation of BK virus infection

    Directory of Open Access Journals (Sweden)

    Nima Derakhshan

    2011-01-01

    Full Text Available BK virus (BKV is increasingly found as an important cause of allograft nephro-pathy. Nephrotic syndrome is not a usual manifestation of BKV nephropathy. Here, we report a 12-year-old boy, a case of end-stage renal disease due to nephronophthisis, who got the kidney trans-planted from a 16-year-old cadaver, and after 18 months of uneventful transplantation on triple immunosuppressive therapy (Mycophenolate mofetil (MMF, cyclosporin and prednisolone, pre-sented with nephrotic feature (edema, heavy proteinuria, hypoalbuminemia and hyperlipidema. Kidney biopsy was in favor of BKV infection and eventually ended in graft failure.

  10. A Case of Newly Diagnosed Klippel Trenaunay Weber Syndrome Presenting with Nephrotic Syndrome

    Directory of Open Access Journals (Sweden)

    Egemen Cebeci

    2015-01-01

    Full Text Available Klippel Trenaunay Weber syndrome (KTWS is a rare disease characterized by hemihypertrophy, variceal enlargement of the veins, and arteriovenous (AV malformations. Renal involvement in KTWS is not known except in rare case reports. Herein, we present a case of KTWS with nephrotic syndrome. A 52-year-old male was admitted due to dyspnea and swelling of the body for the last three months. The pathological physical findings were diffuse edema, decreased lung sounds at the right basal site, increased diameter and decreased length of the left leg compared with the right one, diffuse variceal enlargements, and a few hemangiomatous lesions on the left leg. The pathological laboratory findings were hypoalbuminemia, hyperlipidemia, increased creatinine level (1.23 mg/dL, and proteinuria (7.6 g/day. Radiographic pathological findings were cystic lesions in the liver, spleen, and kidneys, splenomegaly, AV malformation on the left posterolateral thigh, and hypertrophy of the soft tissues of the proximal left leg. He was diagnosed to have KTWS with these findings. Renal biopsy was performed to determine the cause of nephrotic syndrome. The pathologic examination was consistent with focal segmental sclerosis (FSGS. He was started on oral methylprednisolone at the dosage of 1 mg/kg and began to be followedup in the nephrology outpatient clinic.

  11. Preliminary research on syndrome types of Chinese medicine in children with primary nephrotic syndrome.

    Science.gov (United States)

    Sun, Wen; Yu, Jian; Zeng, Gu-Lan; Zhang, Bing-Feng

    2017-08-17

    To provide an objective reference for the syndrome types of Chinese medicine (CM) associated with pediatric primary nephrotic syndrome (PNS). A cross-sectional study was performed. Data on clinical symptoms, CM syndrome types, biochemical indices, and medications used were collected from 98 children with PNS. Then, the correlation between CM syndromes and biochemical indices, as well as medications used, was analyzed. The four most common symptoms in children with PNS were brown urine, red tongue, excessive sweating, and swelling of the face and limbs. The syndromes of qi deficiency of Fei (Lung) and Shen (Kidney) (FSQD) and yin deficiency of Gan (Liver) and Shen (GSYD) were the most common main CM syndrome types. FSQD syndrome score correlated significantly with the total cholesterol level, urine protein/creatinine ratio, and urine IgG and albumin levels (Ptypes were FSQD and GSYD syndromes. FSQD syndrome may be caused by some factors related to lipid levels, protein loss, and the use of immunosuppressive agents. The use of maintenance glucocorticoids may cause GSYD syndrome.

  12. Second-Line Immunosuppressive Treatment of Childhood Nephrotic Syndrome: A Single-Center Experience

    Directory of Open Access Journals (Sweden)

    J. Kim

    2014-01-01

    Full Text Available Objective: Most cases of idiopathic nephrotic syndrome in childhood are responsive to corticosteroids. However, there is a small group of children that demonstrate steroid resistance (steroid-resistant nephrotic syndrome; SRNS, steroid dependence, or that frequently relapse (frequent-relapse steroid-sensitive nephrotic syndrome; FR-SSNS which are more clinically difficult to treat. Therefore, second-line immunosuppressants, such as alkylating agents, calcineurin inhibitors, antimetabolites and, more recently, rituximab, have been used with varying success. The objective was to evaluate the response rates of various second-line therapies in the treatment of childhood nephrotic syndrome. Study Design: A retrospective chart review of pediatric subjects with idiopathic nephrotic syndrome was conducted at a single tertiary care center (2007-2012. Drug responses were classified as complete response, partial response, and no response. Results: Of the 188 charts reviewed, 121 children were classified as SSNS and 67 children as SRNS; 58% were classified as FR-SSNS. Sixty-five subjects were diagnosed with focal segmental glomerulosclerosis via biopsy. Follow-up ranged from 6 months to 21 years. The combined rate of complete and partial response for mycophenolate mofetil (MMF was 65% (33/51 in SSNS and 67% (6/9 in SRNS. For tacrolimus, the response rate was 96% (22/23 for SSNS and 77% (17/22 for SRNS. Eighty-three percent (5/6 of SSNS subjects treated with rituximab went into complete remission; 60% relapsed after B-cell repletion. Eight refractory subjects were treated with combined MMF/tacrolimus/corticosteroid therapy with a 75% response rate. Conclusion: Our experience demonstrates that older medications can be replaced with newer ones such as MMF, tacrolimus, and rituximab with good outcomes and better side effect profiles. The treatment of refractory cases with combination therapy is promising.

  13. Dysregulation of ENaC in Animal Models of Nephrotic Syndrome and Liver Cirrhosis.

    Science.gov (United States)

    Kim, Soo Wan

    2006-03-01

    Nephrotic syndrome and liver cirrhosis are common clinical manifestations, and are associated with avid sodium retention leading to the development of edema and ascites. However, the mechanism for the sodium retention is still incompletely understood and the molecular basis remains undefined. We examined the changes of sodium (co)transporters and epithelial sodium channels (ENaCs) in the kidneys of experimental nephrotic syndrome and liver cirrhosis. The results demonstrated that puromycin- or HgCl2-induced nephrotic syndrome was associated with 1) sodium retention, decreased urinary sodium excretion, development of ascites, and increased plasma aldosterone level; 2) increased apical targeting of ENaC subunits in connecting tubule and collecting duct segments; and 3) decreased protein abundance of type 2 11β-hydroxysteroid dehydrogenase (11βHSD2). Experimental liver cirrhosis was induced in rats by CCl4 treatment or common bile duct ligation. An increased apical targeting of alpha-, beta-, and gamma-ENaC subunits in connecting tubule, and cortical and medullary collecting duct segments in sodium retaining phase of liver cirhosis but not in escape phase of sodium retention. Immunolabeling intensity of 11βHSD2 in the connecting tubule and cortical collecting duct was significantly reduced in sodium retaining phase of liver cirrhosis, and this was confirmed by immunoblotting. These observations therefore strongly support the view that the renal sodium retention associated with nephrotic syndrome and liver cirrhosis is caused by increased sodium reabsorption in the aldosterone sensitive distal nephron including the connecting tubule and collecting duct, and increased apical targeting of ENaC subunits plays a role in the development of sodium retention in nephrotic syndrome and liver cirrhosis.

  14. Corticosteroids and obesity in steroid-sensitive and steroid-resistant nephrotic syndrome

    Directory of Open Access Journals (Sweden)

    Nina Lestari

    2015-07-01

    Full Text Available Background Children with nephrotic syndrome need high-dose corticosteroids to achieve remission. Studies have estimated a 35-43% risk of obesity in these patients after corticosteroid treatment. Objective To determine the prevalence of obesity in children who received corticosteroids for nephrotic syndrome, and to compare the risk of obesity in children with steroid-sensitive nephrotic syndrome (SSNS and steroid-resistant nephrotic syndrome (SRNS. Methods We performed a retrospective cohort study in 50 children with SSNS or SRNS who received corticosteroid treatment. Obesity was defined to be a BMI-for-age Z-score above +2.0 SD, according to the WHO Growth Reference 2007. Central obesity was defined to be a waist-to-height ratio > 0.50. Results The overall prevalence of obesity was 22%, with 29% and 14% in the SSNS and SRNS groups, respectively. The overall prevalence of central obesity was 50%, with 54% and 46% in the SSNS and SRNS groups, respectively. The cumulative steroid doses in this study were not significantly different between the SSNS and SRNS groups. There were also no significant differences between groups for risk of obesity (RR 2.53; 95%CI 0.58 to 10.99 or central obesity (RR 1.39; 95%CI 0.45 to 4.25. Conclusion In children with nephrotic syndrome who received corticosteroids, the prevalence of obesity is 22% and of central obesity is 50%. In a comparison of SSNS and SRNS groups, cumulative steroid dose as well as risks of obesity and central obesity do not significantly differ between groups.

  15. Chronic Lyme borreliosis associated with minimal change glomerular disease: a case report.

    Science.gov (United States)

    Florens, N; Lemoine, S; Guebre-Egziabher, F; Valour, F; Kanitakis, J; Rabeyrin, M; Juillard, L

    2017-02-06

    There are only few cases of renal pathology induced by Lyme borreliosis in the literature, as this damage is rare and uncommon in humans. This patient is the first case of minimal change glomerular disease associated with chronic Lyme borreliosis. A 65-year-old Caucasian woman was admitted for an acute edematous syndrome related to a nephrotic syndrome. Clinical examination revealed violaceous skin lesions of the right calf and the gluteal region that occurred 2 years ago. Serological tests were positive for Lyme borreliosis and skin biopsy revealed lesions of chronic atrophic acrodermatitis. Renal biopsy showed minimal change glomerular disease. The skin lesions and the nephrotic syndrome resolved with a sequential treatment with first ceftriaxone and then corticosteroids. We report here the first case of minimal change disease associated with Lyme borreliosis. The pathogenesis of minimal change disease in the setting of Lyme disease is discussed but the association of Lyme and minimal change disease may imply a synergistic effect of phenotypic and bacterial factors. Regression of proteinuria after a sequential treatment with ceftriaxone and corticosteroids seems to strengthen this conceivable association.

  16. Posterior reversible encephalopathy syndrome in a 5-year-old boy with steroid-dependent nephrotic syndrome

    Directory of Open Access Journals (Sweden)

    Md. Habibur Rahman

    2016-01-01

    Full Text Available Posterior reversible encephalopathy syndrome (PRES is a rare clinical and radiological phenomenon is encountered in children compared to adults. In our center, a 5-yearold boy with steroid-dependent nephrotic syndrome (SDNS presented with headache and blurring of vision during relapse after a long course of immunosuppressive therapy. Evaluation by computed tomography scan of the brain showed that the child had hypodense areas throughout the occipital region of the brain. All signs of PRES, except papilledema, resolved after seven days of supportive treatment evidenced by subsequent radiological evaluation. PRES should be kept in mind in any nephrotic child who is on prolonged immunosuppressive therapy.

  17. Expression of glomerular ecto-ATPase in idiopathic nephrotic syndrome

    NARCIS (Netherlands)

    Cheung, PK; Baller, JFW; vanderHorst, MLC; Bakker, WW; Plesner, L; Kirley, TL; Knowles, AF

    1997-01-01

    The pathogenesis of glomerular alterations leading to increased glomerular permeability in disorders like Minimal Change Disease (MCD) is obscure. One of the preliminary observed glomerular alterations in MCD involves diminished expression of glomerular ecto-ATP-diphosphohydrolase (denoted as

  18. Nephrotic syndrome among children in Kano: A clinicopathological ...

    African Journals Online (AJOL)

    2013-11-14

    Nov 14, 2013 ... Minimal change disease was found in four children (20%), membranoproliferative glomerulonephritis ... generally has a favorable response to glucocorticoid therapy .... antibody to hepatitis C virus (anti‑HCV) was also positive.

  19. Late Onset Cobalamin Disorder and Hemolytic Uremic Syndrome: A Rare Cause of Nephrotic Syndrome

    Directory of Open Access Journals (Sweden)

    Gianluigi Ardissino

    2017-01-01

    Full Text Available Hemolytic uremic syndrome (HUS is an unrare and severe thrombotic microangiopathy (TMA caused by several pathogenetic mechanisms among which Shiga toxin-producing Escherichia coli infections and complement dysregulation are the most common. However, very rarely and particularly in neonates and infants, disorders of cobalamin metabolism (CblC can present with or be complicated by TMA. Herein we describe a case of atypical HUS (aHUS related to CblC disease which first presented in a previously healthy boy at age of 13.6 years. The clinical picture was initially dominated by nephrotic range proteinuria and severe hypertension followed by renal failure. The specific treatment with high dose of hydroxycobalamin rapidly obtained the remission of TMA and the complete recovery of renal function. We conclude that plasma homocysteine and methionine determinations together with urine organic acid analysis should be included in the diagnostic work-up of any patient with TMA and/or nephrotic syndrome regardless of age.

  20. Increased fasting plasma acylation-stimulating protein concentrations in nephrotic syndrome.

    Science.gov (United States)

    Ozata, Metin; Oktenli, Cagatay; Gulec, Mustafa; Ozgurtas, Taner; Bulucu, Fatih; Caglar, Kayser; Bingol, Necati; Vural, Abdulgaffar; Ozdemir, I Caglayan

    2002-02-01

    Acylation-stimulating protein (ASP) is an adipocyte-derived protein that has recently been suggested to play an important role in the regulation of lipoprotein metabolism and triglyceride (TG) storage. ASP also appears to have a role in the regulation of energy balance. In addition to its role as a hormonal regulator of body weight and energy expenditure, leptin is now implicated as a regulatory molecule in lipid metabolism. However, little is known about the alterations in fasting plasma ASP and leptin concentrations in the nephrotic syndrome. As hyperlipidemia is one of the most striking manifestations of the nephrotic syndrome, we have investigated fasting plasma ASP and leptin levels and their relation to lipid levels in this syndrome. Twenty-five patients with untreated nephrotic syndrome and 25 age-, sex-, and body mass index-matched healthy controls were included in the study. Fasting plasma lipoproteins, TG, total cholesterol, lipoprotein(a), apolipoprotein AI (apoAI), apoB, urinary protein, plasma albumin, third component of complement (C3), ASP, and leptin levels were measured in both groups. Total cholesterol, TG, low and very low density lipoproteins, lipoprotein(a), apoB, and urinary protein levels were increased in the patient group, whereas plasma albumin, high density lipoprotein cholesterol, and apoAI levels were decreased compared with those in the control group (P Fasting ASP concentrations showed no correlation with body mass index, proteinuria, plasma albumin, leptin, or any lipid parameter in either group, but C3 levels (in patient group: r(s) = 0.92; P < 0.001; in control group: r(s) = 0.68; P < 0.001). Our findings showed that plasma ASP levels were significantly elevated, whereas leptin levels were normal in the nephrotic syndrome. Increased ASP levels in the setting of dyslipidemia in the nephrotic syndrome raise the possibility of an ASP receptor defect in adipocytes, which also suggests the existence of so-called ASP resistance. Moreover

  1. Nephrotic syndrome associated with hepatointestinal schistosomiasis Síndrome nefrótica associada à esquistossomose hepatointestinal

    OpenAIRE

    H. Abensur; Nussenzveig,I.; L.B. Saldanha; M.S.C. Petalozzi; Barros,M.T.; M. Marcondes; R.T. Barros

    1992-01-01

    Schistosomal nephropathy has long been related to the hepatosplenic form of schistosomiasis. In the last few years, 24 patients with hepatointestinal schistosomiasis and the nephrotic syndrome were studied. Aiming at evaluating a possible etiologic participation of schistosomiasis in the development of the nephropathy, this group was comparatively studied with a group of 37 patients with idiopathic nephrotic syndrome. Both groups had a different distribution of the histologic lesions. In the ...

  2. Unusual pediatric co-morbility: autoimmune thyroiditis and cortico-resistant nephrotic syndrome in a 6-month-old Italian patient.

    Science.gov (United States)

    Urbano, Flavia; Acquafredda, Angelo; Aceto, Gabriella; Penza, Rosa; Cavallo, Luciano

    2012-10-23

    We report on a case of autoimmune thyroiditis in a 6-month-old patient with cortico-resistant nephrotic syndrome. Normal serum levels of thyroid hormons and thyroid-stimulating hormone were detected with high titers of circulant antithyroid antibodies and a dysomogeneous ultrasound appearance of the gland, typical of autoimmune thyroiditis. The research of maternal thyroid antibodies was negative. This is the first case of autoimmune thyroiditis found in such a young patient with pre-existing nephrotic syndrome ever described in literature. This association is random because nephrotic syndrome does not have an autoimmune pathogenesis and the genes involved in autoimmune thyroiditis are not related to those of nephrotic syndrome.

  3. Minimal change disease caused by exposure to mercury-containing skin lightening cream: a report of 4 cases.

    Science.gov (United States)

    Tang, Hon-Lok; Mak, Yuen-Fun; Chu, Kwok-Hong; Lee, William; Fung, Samuel Kaâ Shun; Chan, Thomas Yan-Keung; Tong, Kwok-Lung

    2013-04-01

    Mercury is a known cause of nephrotic syndrome and the underlying renal pathology in most of the reported cases was membranous nephropathy. We describe here 4 cases of minimal change disease following exposure to mercury-containing skin lightening cream for 2 - 6 months. The mercury content of the facial creams was very high (7,420 - 30,000 parts per million). All patients were female and presented with nephrotic syndrome and heavy proteinuria (8.35 - 20.69 g/d). The blood and urine mercury levels were 26 - 129 nmol/l and 316 - 2,521 nmol/d, respectively. Renal biopsy revealed minimal change disease (MCD) in all patients. The use of cosmetic cream was stopped and chelation therapy with D-penicillamine was given. Two patients were also given steroids. The time for blood mercury level to normalize was 1 - 7 months, whereas it took longer for urine mercury level to normalize (9 - 16 months). All patients had complete remission of proteinuria and the time to normalization of proteinuria was 1 - 9 months. Mercury-containing skin lightening cream is hazardous because skin absorption of mercury can cause minimal change disease. The public should be warned of the danger of using such products. In patients presenting with nephrotic syndrome, a detailed history should be taken, including the use of skin lightening cream. With regard to renal pathology, apart from membranous nephropathy, minimal change disease should be included as another pathological entity caused by mercury exposure or intoxication.

  4. Levamisole in steroid-sensitive nephrotic syndrome: usefulness in adult patients and laboratory insights into mechanisms of action via direct action on the kidney podocyte.

    Science.gov (United States)

    Jiang, Lulu; Dasgupta, Ishita; Hurcombe, Jenny A; Colyer, Heather F; Mathieson, Peter W; Welsh, Gavin I

    2015-06-01

    Minimal change nephropathy (MCN) is the third most common cause of primary nephrotic syndrome in adults. Most patients with MCN respond to corticosteroid therapy, but relapse is common. In children, steroid-dependent patients are often given alternative agents to spare the use of steroids and to avoid the cumulative steroid toxicity. In this respect, levamisole has shown promise due to its ability to effectively maintain remission in children with steroid-sensitive or steroid-dependent nephrotic syndrome. Despite clinical effectiveness, there is a complete lack of molecular evidence to explain its mode of action and there are no published reports on the use of this compound in adult patients. We studied the effectiveness of levamisole in a small cohort of adult patients and also tested the hypothesis that levamisole's mode of action is attributable to its direct effects on podocytes. In the clinic, we demonstrate that in our adult patients, cohort levamisole is generally well tolerated and clinically useful. Using conditionally immortalized human podocytes, we show that levamisole is able to induce expression of glucocorticoid receptor (GR) and to activate GR signalling. Furthermore, levamisole is able to protect against podocyte injury in a puromycin aminonucleoside (PAN)-treated cell model. In this model the effects of levamisole are blocked by the GR antagonist mifepristone (RU486), suggesting that GR signalling is a critical target of levamisole's action. These results indicate that levamisole is effective in nephrotic syndrome in adults, as well as in children, and point to molecular mechanisms for this drug's actions in podocyte diseases.

  5. Kinetics of Rituximab Excretion into Urine and Peritoneal Fluid in Two Patients with Nephrotic Syndrome

    Science.gov (United States)

    Schwarz, Anke; Wagner, A. D.; Haller, Hermann; Schiffer, Mario

    2017-01-01

    Clinical observations suggest that treatment of Rituximab might be less effective in patients with nephrotic range proteinuria when compared to nonnephrotic patients. It is conceivable that the reason for this is that significant amounts of Rituximab might be lost in the urine in a nephrotic patient and that these patients require a repeated or higher dosage. However, this has not been systematically studied. In this case report we describe two different patients with nephrotic range proteinuria receiving Rituximab. The first patient received Rituximab for therapy resistant cryoglobulinemic membranoproliferative glomerulonephritis and the other for second line treatment of Felty's syndrome. We employed flow cytometry to determine the amount of Rituximab excretion in both urine and peritoneal fluid specimens in these patients following administration of Rituximab. We found that a significant amount of Rituximab is lost from the circulation by excretion into the urine. Furthermore we saw a close correlation of the excretion of Rituximab to the excretion of IgG molecules suggesting selectivity of proteinuria as the determining factor of Rituximab excretion. Further larger scale clinical studies could have the potential to evaluate an optimal cut-off value of IgG urinary loss before a possible administration of Rituximab therefore contributing to a more individualized treatment approach in patients with nonselective and nephrotic range proteinuria.

  6. Kinetics of Rituximab Excretion into Urine and Peritoneal Fluid in Two Patients with Nephrotic Syndrome

    Directory of Open Access Journals (Sweden)

    Klaus Stahl

    2017-01-01

    Full Text Available Clinical observations suggest that treatment of Rituximab might be less effective in patients with nephrotic range proteinuria when compared to nonnephrotic patients. It is conceivable that the reason for this is that significant amounts of Rituximab might be lost in the urine in a nephrotic patient and that these patients require a repeated or higher dosage. However, this has not been systematically studied. In this case report we describe two different patients with nephrotic range proteinuria receiving Rituximab. The first patient received Rituximab for therapy resistant cryoglobulinemic membranoproliferative glomerulonephritis and the other for second line treatment of Felty’s syndrome. We employed flow cytometry to determine the amount of Rituximab excretion in both urine and peritoneal fluid specimens in these patients following administration of Rituximab. We found that a significant amount of Rituximab is lost from the circulation by excretion into the urine. Furthermore we saw a close correlation of the excretion of Rituximab to the excretion of IgG molecules suggesting selectivity of proteinuria as the determining factor of Rituximab excretion. Further larger scale clinical studies could have the potential to evaluate an optimal cut-off value of IgG urinary loss before a possible administration of Rituximab therefore contributing to a more individualized treatment approach in patients with nonselective and nephrotic range proteinuria.

  7. [Disseminated tuberculosis revealing IgA nephropathy with nephrotic syndrome : A case report].

    Science.gov (United States)

    Morbieu, Caroline; Michel, Pierre-Antoine; Brocheriou, Isabelle; Canestri, Ana; Boffa, Jean-Jacques

    2016-07-01

    A 27-year-old man without any medical history presented concomitantly a pulmonary and urinary tuberculosis and a nephrotic syndrome with hematuria and renal failure. The renal biopsy showed increased mesangial matrix, few focal segmental lesions, and IgA deposits confirming the diagnosis of IgA nephropathy. Nephrotic syndrome remission occurred quickly after antituberculous treatment. The association between tuberculosis and IgA nephropathy has been previously reported in 9 patients. Renal outcome was always favorable with antituberculous treatment. No relapse occurred, with a maximal follow-up of 42 months. Here, we discuss this singular association and previous similar cases. Copyright © 2016 Association Société de néphrologie. Published by Elsevier SAS. All rights reserved.

  8. Rare case of spot diagnosis of nephrotic syndrome in a Saudi male

    Directory of Open Access Journals (Sweden)

    A Askar

    2011-01-01

    Full Text Available A 26- year- old Saudi male school teacher presented with clinical features of nephrotic syndrome. Physical examination revealed loss of subcutaneous fat over the face and upper arms (partial lipodystrophy and drusen in both eyes. The serum level of complement C3 was low, with normal C4. Renal biopsy revealed features consistent with membranoproliferative glomerulo- nephritis (MPGN type II [Dense Deposit Disease (DDD]. He was treated with prednisolone and mycophenolate mofetil with partial remission. He also received lipitor and lisinopril. The occurrence of partial lipo- dystrophy and drusen in a patient with nephrotic syndrome provides a rare clinical opportunity of making a bedside diagnosis of type II MPGN even before kidney biopsy.

  9. Juvenile idiopathic arthritis complicated by amyloidosis with secondary nephrotic syndrome - effective treatment with tocilizumab.

    Science.gov (United States)

    Kwiatkowska, Małgorzata; Jednacz, Ewa; Rutkowska-Sak, Lidia

    2015-01-01

    A case report of a boy with juvenile idiopathic arthritis since the age of 2 years, generalized onset, complicated by nephrotic syndrome due to secondary type A amyloidosis is presented. In the patient the disease had an especially severe course, complicated by frequent infections, making routine treatment difficult. Amyloidosis was diagnosed in the 5(th) year of the disease based on a rectal biopsy. Since the disease onset the boy has been taking prednisolone and sequentially cyclosporine A, methotrexate, chlorambucil, etanercept, and cyclophosphamide. Clinical and laboratory remission was observed after treatment with tocilizumab. After 42 months of treatment with tocilizumab the boy's condition is good. There is no pain or joint edema, and no signs of nephrotic syndrome.

  10. Clinical Use of Diuretics in Heart Failure, Cirrhosis, and Nephrotic Syndrome

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    Ahmed Hassaan Qavi

    2015-01-01

    Full Text Available Diuretics play significant role in pharmacology and treatment options in medicine. This paper aims to review and evaluate the clinical use of diuretics in conditions that lead to fluid overload in the body such as cardiac failure, cirrhosis, and nephrotic syndrome. To know the principles of treatment it is essential to understand the underlying pathophysiological mechanisms that cause the need of diuresis in the human body. Various classes of diuretics exist, each having a unique mode of action. A systemic approach for management is recommended based on the current guidelines, starting from thiazides and proceeding to loop diuretics. The first condition for discussion in the paper is cardiac failure. Treatment of ascites in liver cirrhosis with spironolactone as the primary agent is highlighted with further therapeutic options. Lastly, management choices for nephrotic syndrome are discussed and recommended beginning from basic sodium restriction to combined diuretic therapies. Major side effects are discussed.

  11. Expression of glomerular ecto-ATPase in idiopathic nephrotic syndrome

    NARCIS (Netherlands)

    Cheung, PK; Baller, JFW; vanderHorst, MLC; Bakker, WW; Plesner, L; Kirley, TL; Knowles, AF

    1997-01-01

    The pathogenesis of glomerular alterations leading to increased glomerular permeability in disorders like Minimal Change Disease (MCD) is obscure. One of the preliminary observed glomerular alterations in MCD involves diminished expression of glomerular ecto-ATP-diphosphohydrolase (denoted as ecto-A

  12. Colchicine therapy in amyloidosis related with plasmacytic castleman disease presenting with nephrotic syndrome

    Directory of Open Access Journals (Sweden)

    Saime Paydas

    2015-01-01

    Full Text Available Castleman disease (CD is a neoplasm that presents with single or multiple lymphadenopathy. The disease is characterized by fever, weight loss, anemia, polyclonal hyperglobulinemia, splenomegaly, thrombocytosis and peripheral lymphadenopathy. In this paper, we report a young man with plasmacytic type CD and amyloid A (AA deposition who presented with intra-abdominal mass and nephrotic syndrome. He was successfully treated with colchicine following surgery.

  13. Computed tomographic findings in a case of renal vein thrombosis with nephrotic syndrome.

    Science.gov (United States)

    Adler, J; Greweldinger, J; Hallac, R; Frier, S

    1981-01-01

    Renal vein thrombosis is a complication of the nephrotic syndrome presumably related to compression of renal veins by edematous parenchyma and a concomitant hypercoagulable state. The diagnosis has been made by demonstrating marked widening of the left renal vein as it crosses horizontally anterior to the aorta on computed tomography. Inferior venacavography confirmed the presence of thrombosis within the vessels. CT is suggested as a method for noninvasive imaging of the renal veins which might eliminate the need for venography.

  14. Peritoneal protein losses in children with steroid-resistant nephrotic syndrome on continuous-cycler peritoneal dialysis.

    Science.gov (United States)

    Kopanati, Sashikala; Baum, Michel; Quan, Albert

    2006-07-01

    Glomerular protein permeability rises in nephrotic syndrome and may result from the effect of an unidentified "circulating factor." The effect of this "circulating factor" on the permeability of other body membranes is unknown. In this study we examine the peritoneal membrane protein permeability in patients with nephrotic syndrome on chronic-cycler peritoneal dialysis. We conducted a retrospective study of peritoneal protein losses in the dialysate effluent of 60 pediatric peritoneal dialysis patients (ages 5.1-22 years) over a 6-year period (January 1997-December 2002). Nineteen patients had steroid-resistant nephrotic syndrome (SRNS), while 41 had other non-nephrotic etiologies of renal failure. Total and normalized peritoneal protein losses are higher in SRNS than in non-nephrotic patients (12,603+/-5,403 mg/day vs 4,475+/-469 mg/day, Ppermeability, was higher in SRNS patients (3.50+/-1.00% vs 0.68+/-0.06%, Pdialysis, dialysis prescription, numbers of peritonitis episodes, catheter replacements, or hospitalizations. In summary, these results demonstrate that peritoneal protein losses in patients with SRNS are twice as great as in those without nephrotic syndrome. These results are consistent with the systemic effect of a "circulating factor" in SRNS and underscore the importance of adequate protein intake in patients on peritoneal dialysis.

  15. Effects of gluten-free, dairy-free diet on childhood nephrotic syndrome and gut microbiota.

    Science.gov (United States)

    Uy, Natalie; Graf, Lauren; Lemley, Kevin V; Kaskel, Frederick

    2015-01-01

    Emerging evidence suggests an association between food sensitivity and gut microbiota in children with nephrotic syndrome. Diminished proteinuria resulted from eliminating cow's milk and the use of an oligoantigenic diet which excluded gluten, especially in patients with immune-related conditions, i.e., celiac disease and nephrotic syndrome. The mechanisms underlying the association of diet, gut microbiota, and dysregulation of the immune system are unknown. Gut microbiota is influenced by a number of factors including diet composition and other environmental epigenetic exposures. The imbalance in gut microbiota may be ameliorated by gluten-free and dairy-free diets. Gluten-free diet increased the number of unhealthy bacteria while reducing bacterial-induced cytokine production of IL-10. Thus, gluten-free diet may influence the composition and immune function of gut microbiota and should be considered a possible environmental factor associated with immune-related disease, including nephrotic syndrome. Furthermore, the imbalance of gut microbiota may be related to the development of cow's milk protein allergy. Investigations are needed to fill the gaps in our knowledge concerning the associations between the gut microbiome, environmental exposures, epigenetics, racial influences, and the propensity for immune dysregulation with its inherent risk to the developing individual.

  16. Effect of Poria cocos on Puromycin Aminonucleoside-Induced Nephrotic Syndrome in Rats.

    Science.gov (United States)

    Lee, So Min; Lee, Yun Jung; Yoon, Jung Joo; Kang, Dae Gill; Lee, Ho Sub

    2014-01-01

    Nephrotic syndrome is associated with altered renal handling of water and sodium and changes in the levels of aquaporins (AQPs) and epithelial Na channels (ENaCs). The dried sclerotia of Poria cocos Wolf (WPC) have been used for treating chronic edema and nephrosis. We evaluated the effects of WPC on puromycin aminonucleoside- (PAN-) induced renal functional derangement and altered renal AQP2 and ENaC expression. In the nephrotic syndrome rat model, animals were injected with 75 mg/kg PAN and then treated with Losartan (30 mg·kg(-1) ·day(-1)) or WPC (200 mg·kg(-1) ·day(-1)) for 7 days. In the WPC group, proteinuria and ascites improved significantly. Plasma levels of triglyceride, total cholesterol, and low-density lipoprotein- (LDL-) cholesterol reduced significantly in the WPC group. In addition, the WPC group exhibited attenuation of the PAN-induced increase in AQP2 and ENaC α/β subunit protein and mRNA levels. WPC suppressed significantly PAN-induced organic osmolyte regulators, reducing serum- and glucocorticoid-inducible protein kinase (Sgk1) and sodium-myo-inositol cotransporter (SMIT) mRNA expression. Our results show that WPC improves nephrotic syndrome, including proteinuria and ascites, through inhibition of AQP2 and ENaC expression. Therefore, WPC influences body-fluid regulation via inhibition of water and sodium channels, thereby, improving renal disorders such as edema or nephrosis.

  17. Podocin Inactivation in Mature Kidneys Causes Focal Segmental Glomerulosclerosis and Nephrotic Syndrome

    Science.gov (United States)

    Mollet, Géraldine; Ratelade, Julien; Boyer, Olivia; Muda, Andrea Onetti; Morisset, Ludivine; Lavin, Tiphaine Aguirre; Kitzis, David; Dallman, Margaret J.; Bugeon, Laurence; Hubner, Norbert; Gubler, Marie-Claire; Esquivel, Ernie L.

    2009-01-01

    Podocin is a critical component of the glomerular slit diaphragm, and genetic mutations lead to both familial and sporadic forms of steroid-resistant nephrotic syndrome. In mice, constitutive absence of podocin leads to rapidly progressive renal disease characterized by mesangiolysis and/or mesangial sclerosis and nephrotic syndrome. Using established Cre-loxP technology, we inactivated podocin in the adult mouse kidney in a podocyte-specific manner. Progressive loss of podocin in the glomerulus recapitulated albuminuria, hypercholesterolemia, hypertension, and renal failure seen in nephrotic syndrome in humans. Lesions of FSGS appeared after 4 wk, with subsequent development of diffuse glomerulosclerosis and tubulointerstitial damage. Interestingly, conditional inactivation of podocin at birth resulted in a gradient of glomerular lesions, including mesangial proliferation, demonstrating a developmental stage dependence of renal histologic patterns of injury. The development of significant albuminuria in this model occurred only after early and focal foot process effacement had progressed to diffuse involvement, with complete absence of podocin immunolabeling at the slit diaphragm. Finally, we identified novel potential mediators and perturbed molecular pathways, including cellular proliferation, in the course of progression of renal disease leading to glomerulosclerosis, using global gene expression profiling. PMID:19713307

  18. Use of Rituximab in Children with Steroid- and Calcineurin-Inhibitor-Dependent Idiopathic Nephrotic Syndrome

    Science.gov (United States)

    Ravani, Pietro; Ponticelli, Alessandro; Siciliano, Chiara; Fornoni, Alessia; Magnasco, Alberto; Sica, Felice; Bodria, Monica; Caridi, Gianluca; Wei, Changli; Belingheri, Mirco; Ghio, Luciana; Merscher-Gomez, Sandra; Edefonti, Alberto; Pasini, Andrea; Montini, Giovanni; Murtas, Corrado; Wang, Xiangyu; Muruve, Daniel; Vaglio, Augusto; Martorana, Davide; Pani, Antonello; Scolari, Francesco; Reiser, Jochen; Ghiggeri, Gian Marco

    2013-01-01

    In children with idiopathic nephrotic syndrome rituximab can maintain short-term remission with withdrawal of prednisone and calcineurin-inhibitors. Long-term effects including number of repeated infusions to maintain remission are unknown. We treated with rituximab 46 consecutive children with idiopathic nephrotic syndrome lasting for at least one year (6.3±4.1 years), who were maintained in remission with oral prednisone and calcineurin inhibitors. They received 1–5 rituximab courses during a median follow-up of three years (range 1–5). Oral agents were tapered after each infusion, and completely withdrawn within 45 days. Rituximab was well tolerated. Six-month probabilities of remission were 48% after the first infusion and 37% after subsequent infusions. One- and two-year-remission probabilities were respectively 20% and 10%. Median time intervals between complete oral-agent withdrawal and relapse were 5.6 and 8.5 months respectively following the first and subsequent courses. Time to reconstitution of CD20 cells correlated with the duration of remission, but was not associated with variation in FcyR, CD20 or SMPDL-3B polymorphisms. Podocyte Src phosphorylation was normal. Rituximab can be safely and repeatedly used as prednisone and calcineurin-inhibitor-sparing therapy in a considerable proportion of children with dependent forms of idiopathic nephrotic syndrome. Further research is needed to identify patients who will benefit most from rituximab therapy. PMID:23739238

  19. Podocin inactivation in mature kidneys causes focal segmental glomerulosclerosis and nephrotic syndrome.

    Science.gov (United States)

    Mollet, Géraldine; Ratelade, Julien; Boyer, Olivia; Muda, Andrea Onetti; Morisset, Ludivine; Lavin, Tiphaine Aguirre; Kitzis, David; Dallman, Margaret J; Bugeon, Laurence; Hubner, Norbert; Gubler, Marie-Claire; Antignac, Corinne; Esquivel, Ernie L

    2009-10-01

    Podocin is a critical component of the glomerular slit diaphragm, and genetic mutations lead to both familial and sporadic forms of steroid-resistant nephrotic syndrome. In mice, constitutive absence of podocin leads to rapidly progressive renal disease characterized by mesangiolysis and/or mesangial sclerosis and nephrotic syndrome. Using established Cre-loxP technology, we inactivated podocin in the adult mouse kidney in a podocyte-specific manner. Progressive loss of podocin in the glomerulus recapitulated albuminuria, hypercholesterolemia, hypertension, and renal failure seen in nephrotic syndrome in humans. Lesions of FSGS appeared after 4 wk, with subsequent development of diffuse glomerulosclerosis and tubulointerstitial damage. Interestingly, conditional inactivation of podocin at birth resulted in a gradient of glomerular lesions, including mesangial proliferation, demonstrating a developmental stage dependence of renal histologic patterns of injury. The development of significant albuminuria in this model occurred only after early and focal foot process effacement had progressed to diffuse involvement, with complete absence of podocin immunolabeling at the slit diaphragm. Finally, we identified novel potential mediators and perturbed molecular pathways, including cellular proliferation, in the course of progression of renal disease leading to glomerulosclerosis, using global gene expression profiling.

  20. Impact of the 4G/5G polymorphism in the plasminogen activator inhibitor-1 gene on primary nephrotic syndrome.

    Science.gov (United States)

    Luo, Yuezhong; Wang, Chao; Tu, Haitao

    2014-03-01

    The aim of the present study was to investigate whether the four guanosines (4G)/five guanosines (5G) polymorphism in the gene coding for plasminogen activator inhibitor-1 (PAI-1) affects the clinical features of primary nephrotic syndrome (PNS). A cohort of 200 biopsy-diagnosed PNS patients was studied, with 40 healthy subjects as controls. The PAI-1 gene polymorphism was detected by polymerase chain reaction and DNA sequencing. Associations between the PAI-1 4G/5G polymorphism and clinical features and pathological types of PNS were analyzed. The results indicated that the PAI-1 genotype distribution is significantly different between patients with PNS and healthy controls, with significantly higher numbers of the 4G/4G genotype and lower numbers of the 5G5G genotype detected in PNS patients compared to controls (both P5G genotypes, as well as of the 4G allele. The increased 4G frequency was also detected in patients with minimal change disease (MCD). Significantly increased international normalized ratio (INR) and prolonged activated partial thromboplastin time (APTT) were observed in 4G/4G compared to 5G/5G PNS subjects. The response to steroids was not significantly different among the three genotypes. In conclusion, the 4G allele of the PAI-1 gene appears to be associated with PNS, especially in MN and IgAN patients. These findings suggest that specific targeting may be required for the treatment of PNS patients with the 4G/4G genotype.

  1. Rituximab treatment for adults with refractory nephrotic syndrome: a single-center experience and review of the literature.

    Science.gov (United States)

    Kisner, Tuelay; Burst, Volker; Teschner, Sven; Benzing, Thomas; Kurschat, Christine E

    2012-01-01

    Minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS) are common causes of nephrotic syndrome (NS) in adults. However, induction of remission and sustained control of proteinuria is often difficult. Recently, B cell-directed therapy using the anti-CD20 antibody rituximab has been suggested as induction regimen in pediatric FSGS and MCD patients. Data on rituximab use in adults are still limited. We report on rituximab use in five consecutively treated adult patients (mean age 42.2 ± 9.9 years) with FSGS or relapsing MCD (2 FSGS, 3 MCD) who failed to respond to standard immunosuppressive treatment. Median follow-up was 8 months (3-25). Rituximab induced complete remission in 2 MCD patients and partial remission in 3 patients. Proteinuria was reduced by 86.8% (42.9-95.2) 3 months and by 73.0% (60.1-95.5) 6 months after therapy. In 1 patient with severe FSGS, partial remission was not evident before 6 months after rituximab treatment. Relapses occurred in 2 patients. No severe adverse events related to rituximab were observed. Our findings suggest that B cell-directed therapies are novel treatment options for adults with refractory NS. Response to rituximab varied, with MCD patients exhibiting a faster and more pronounced response compared to FSGS patients. Copyright © 2012 S. Karger AG, Basel.

  2. Focal segmental glomerulosclerosis with idiopathic nephrotic syndrome: three types of clinical response.

    Science.gov (United States)

    Arbus, G S; Poucell, S; Bacheyie, G S; Baumal, R

    1982-07-01

    In a retrospective analysis, 51 patients with focal segmental glomerulosclerosis and idiopathic nephrotic syndrome, who were treated with steroid or cyclophosphamide therapy, were divided into three clinical groups according to the remission profile of their nephrotic syndrome. Group 1 patients (19.37%) consistently responded to medication; none has progressive renal failure (mean follow-up 10.6 years). Group 2 patients (25, 40%) failed to respond to medication; terminal renal failure has occurred in 12 of them. Group 3 patients (7, 14%) initially appeared to be responsive to medication and continued to respond for up to 18 months, but subsequently became unresponsive to any therapy; five of them have required dialysis or transplantation. This third group of patients could not be separated clinically or pathologically from group 1 patients, all of whom have an excellent prognosis. One should, therefore, be cautious about predicting the outcome of steroid-responsive nephrotic patients, especially those with FSGS, until at least 18 months after the onset of illness.

  3. Pharmacokinetics of cyclosporin--a microemulsion in children with idiopathic nephrotic syndrome.

    Science.gov (United States)

    Henriques, Luciana dos Santos; Matos, Fabíola de Marcos; Vaisbich, Maria Helena

    2012-10-01

    We present a prospective study of a microemulsion of cyclosporin to treat idiopathic nephrotic syndrome in ten children with normal renal function who presented cyclosporin trough levels between 50 and 150 ng/ml and achieved complete remission with cyclosporin. To compare the pharmacokinetic parameters of cyclosporin in idiopathic nephrotic syndrome during remission and relapse of the nephrotic state. The pharmacokinetic profile of cyclosporin was evaluated with the 12-hour area under the time-concentration curve (auc0-12) using seven time-point samples. This procedure was performed on each patient during remission and relapse with the same cyclosporin dose in mg/kg/day. The 12-hour area under the time-concentration curve was calculated using the trapezoidal rule. All of the pharmacokinetic parameters and the resumed 4-hour area under the time-concentration curve were correlated with the 12-hour area under the time-concentration curve. ClinicalTrials.gov: NCT01616446. There were no significant differences in any parameters of the pharmacokinetic of cyclosporin during remission and relapse, even when the data were normalized by dose. The best correlation with the 12-hour area under the time-concentration curve was the 4-hour area under the time-concentration curve on remission and relapse of the disease, followed by the 2-hour level after cyclosporin (c2) dosing in both disease states. These data indicate that the same parameters used for cyclosporin therapeutic monitoring estimated during the nephrotic state can also be used during remission. Larger controlled studies are needed to confirm these findings.

  4. Successful treatment of icodextrin-single peritoneal dialysis for refractory nephrotic syndrome induced by idiopathic membranous nephropathy.

    Science.gov (United States)

    Takada, Daisuke; Mii, Akiko; Higo, Seiichiro; Obara, Yoshihiro; Kurabayashi, Yuichi; Kurosawa, Norio; Miura, Shiro; Kawachi, Hiroshi; Shimizu, Akira

    2012-05-01

    The patient was a 76-year-old male who developed nephrotic syndrome. Idiopathic membranous nephropathy was diagnosed by renal biopsy and clinical findings. The patient had been refractory to predonisolone and cyclosporine A therapies, and overhydration recurred repeatedly during the clinical course. One year after an initial hospitalization, he had to be hospitalized a second time because of overhydration. During the hospitalization, he underwent fluid removal by the extracorporeal ultrafiltration method (ECUM), as his response to diuretics was too weak to permit the control of cardiac insufficiency. The ECUM alleviated his overhydration, but no remission of nephrotic syndrome was achieved. The patient was then discharged temporarily, but overhydration developed again 2 months later. Peritoneal dialysis (PD) using an overnight dwell of a single dose of icodextrin was initiated to obtain stable fluid removal. This promptly alleviated the refractory subcutaneous edema, and type I incomplete remission of nephrotic syndrome was achieved about 2 weeks after the start of PD. The patient could be withdrawn from the PD therapy 4 months later. Subsequently, the urinary volume was maintained and the serum creatinine level was stabilized at about 2 mg/dl. In our patient, the protein leakage into the drainage was small enough to permit remission of the nephrotic syndrome with stable fluid removal. On this basis, we believe that PD using icodextrin is considered as one of the options for the treatment of refractory nephrotic syndrome with poor water control.

  5. Pattern of steroid resistant nephrotic syndrome in children living in the kingdom of Saudi Arabia: A single center study

    Directory of Open Access Journals (Sweden)

    Kari Jameela

    2009-01-01

    Full Text Available Steroid resistant nephrotic syndrome (SRNS remains a challenge facing pediatric nephrologists. The underlying histopathology usually affects the course of the disease and the response to treatment. We studied the pattern of histopathology in children with SRNS who presented to the King Abdul Aziz University Hospital (KAUH, Jeddah, Saudi Arabia. The records of all children with primary SRNS, who were seen between 2002 and 2007 were reviewed. Only patients who had undergone a renal biopsy were included in the study. The histopathology slides were reviewed by two renal pathologists independently. Patients with congenital nephrotic syndrome, lupus or sickle cell disease, were excluded from the study. Thirty-six children fulfilled the inclusion criteria, and included 25 girls and 11 boys with female to male ratio of 2.3:1. Fifty percent of the children (n=18 were Saudi and the remaining 50% were from various other racial backgrounds (9 Asians, 4 Arabs, 2 Africans and 3 from the Far East. Their mean age at presentation was 4.3 ± 3.0 years (range 1-12 years. The mean serum albumin at presentation was 15.6 ± 7.1 g/L and all of them had 4+ proteinuria on urinalysis. Five children had elevated serum creatinine at presentation while the mean serum creatinine was 50.4 ± 45.6 µmol/L. Three children had low serum complement levels at presentation and none were positive for hepatitis B surface antigen or antinuclear antibody (ANA. The renal histopathology was compatible with focal and segmental glomerulosclerosis (FSGS in 39% (n=14, IgM nephro-pathy in 28% (n=10, mesengioproliferative glomerulonephritis (MesPGN in 17% (n=6, mini-mal change disease (MCD and C1q nephropathy (C1qNP in 8% each (n=3 + 3 and IgA nephro-pathy in 3% (n=1. Our retrospective review shows that FSGS was the commonest underlying histopathology in children who presented with SRNS followed by IgM nephropathy and other variants of MCD such as MesPGN. C1qNP was the underlying cause in some

  6. Primary Nephrotic Syndrome in Adults as a Risk Factor for Pulmonary Embolism: An Up-to-Date Review of the Literature

    Directory of Open Access Journals (Sweden)

    Aibek E. Mirrakhimov

    2014-01-01

    Full Text Available Patients with nephrotic syndrome are at an increased risk for thrombotic events; deep venous thrombosis, renal vein thrombosis, and pulmonary embolism are quite common in patients with nephrotic syndrome. It is important to note that nephrotic syndrome secondary to membranous nephropathy may impose a greater thrombotic risk for unclear reasons. Increased platelet activation, enhanced red blood cell aggregation, and an imbalance between procoagulant and anticoagulant factors are thought to underlie the excessive thrombotic risk in patients with nephrotic syndrome. The current scientific literature suggests that patients with low serum albumin levels and membranous nephropathy may benefit from primary prophylactic anticoagulation. A thorough approach which includes accounting for all additional thrombotic risk factors is, therefore, essential. Patient counseling regarding the pros and cons of anticoagulation is of paramount importance. Future prospective randomized studies should address the question regarding the utility of primary thromboprophylaxis in patients with nephrotic syndrome.

  7. The plasma permeability factor in nephrotic syndrome: indirect evidence in pediatric peritoneal dialysis.

    Science.gov (United States)

    Azocar, Marta; Quiroz, Lily; Delucchi, Angela; Dinamarca, Hector; Emilfork, Marcos; Cano, Francisco

    2012-01-01

    Nephrotic syndrome (NS) in children has been associated with a systemic circulating permeability factor. Therefore, once peritoneal dialysis (PD) has been started, peritoneal protein losses should be higher in the nephrotic than in the non-nephrotic population. We compared peritoneal protein losses in children with and without NS on PD. Our retrospective 4-year study analyzed Hispanic patients with NS under PD. Data at dialysis entry and 6 months later were compared. Nutritional support was given according to recommended dietary allowances and recommendations from the Kidney Disease Outcomes Quality Initiative. Clinical and biochemical data were obtained, and 24-hour dialysate and urine samples were collected to measure protein losses. Dialysis dose (Kt/V), daily protein intake (DPI), normalized protein equivalent of nitrogen appearance (nPNA), peritoneal equilibration test (PET), and peritonitis rate were determined. All measurements took place at least 4 weeks after resolution of a peritonitis episode. All patients received automated PD using a HomeChoice PD System cycler (Baxter Healthcare Corporation, Deerfield, IL, USA), with an exchange volume of 1100 mL/m(2) and a dextrose concentration of 1.5% - 2.5%. A control group of non-NS children on PD matched by age and sex were also studied. Data are reported as mean ± standard deviation. Differences between groups were calculated using the Mann-Whitney U-test, and p permeability caused by a systemic permeability factor.

  8. Expression of Interleukin- 13 inPeripheral Blood Mononuclear Cells from Patients with Nephrotic Syndrome

    Institute of Scientific and Technical Information of China (English)

    2000-01-01

    Objective Nephrotic syndrome (NS) is a kind of renal diseases characterized mainly by proteinuria and hypoalbuminemia. The disturbance of cellular immunity plays a major role in the pathogenesis of NS. The change of interleukin (IL)-13 produced by Th2 cells was investigated in 50 children with NS. Methods All the patients were selected during their nephrotic stage and remission stage. The expression of IL-13 protein and mRNA was determined by ELISA and reverse transcription-polymerase chain reaction ( RT-PCR ) respectively. Results ①The production of IL-13 inphytoheamagglutinin (PHA)- stimulated monouuclear cells and the expression of IL-13 mRNA were siguificantly increased in patients in nephrotic stage as compared with controls. ②The expression of IL13 protein and mRNA in patients in remission stage was similar to that in the normal controls. ③Th_ere was no correlation between IL-13 and laboratory parameters. Conclusion The results suggest that the disturbance of cellulr immunity plays an important role in the pathogenesis of NS and they also demonstrate a protective role for IL-13 in the children uith NS.

  9. Minimal change disease in graft versus host disease: a podocyte response to the graft?

    Science.gov (United States)

    Huskey, Janna; Rivard, Chris; Myint, Han; Lucia, Scott; Smith, Maxwell; Shimada, Michiko; Ishimoto, Takuji; Araya, Carlos; Garin, Eduardo H; Johnson, Richard J

    2013-12-01

    Nephrotic syndrome is a rare complication of hematopoietic cell transplantation. It has been suggested that nephrotic syndrome may represent a limited form of graft-versus-host disease although the pathological link between these two entities remains unclear. In this paper, we report a case of a 61-year-old female who underwent nonmyeloablative allogenic stem cell transplantation for T-cell prolymphocytic leukemia and subsequently developed biopsy proven minimal change disease shortly after cessation of her immunosuppression therapy. Urinary CD80 was markedly elevated during active disease and disappeared following corticosteroid-induced remission. We hypothesize that alloreactive donor T cells target the kidney and induce podocyte expression of CD80 that results in proteinuria from limited 'graft versus host' disease.

  10. A Novel Biomarker Panel to Identify Steroid Resistance in Childhood Idiopathic Nephrotic Syndrome

    Directory of Open Access Journals (Sweden)

    Michael R Bennett

    2017-03-01

    Full Text Available Idiopathic nephrotic syndrome (NS is the most common glomerular disorder of childhood. Response to initial treatment with corticosteroids is an indicator of prognosis, as resistant patients often present more progressive disease. In this cross-sectional pilot study, we set out to discover a panel of noninvasive biomarkers that could distinguish steroid-resistant nephrotic syndrome (SRNS from steroid-sensitive nephrotic syndrome (SSNS. Information gleaned from such a panel could yield more individualized treatment plans and prevent unnecessary steroid exposure in patients unlikely to respond. Urine was collected from 50 pediatric patients diagnosed with idiopathic NS at Cincinnati Children’s Hospital Medical Center. Isobaric tags for relative and absolute quantitation (iTRAQ was used to discover 13 proteins that were differentially expressed in SSNS vs SRNS in a small 5 × 5 discovery cohort. Suitable assays were found for 9 of the 13 markers identified by iTRAQ and were used in a 25 SRNS × 25 SSNS validation cohort. Vitamin D–binding protein (VDBP, alpha-1 acid glycoprotein 1 (AGP1, alpha-1 acid glycoprotein 2 (AGP2, alpha-1-B glycoprotein (A1BG, fetuin-A, prealbumin, thyroxine-binding globulin and hemopexin, and alpha-2 macroglobulin were measured and combined with urine neutrophil gelatinase–associated lipocalin (NGAL, which had been previously shown to distinguish patients with SRNS. Urinary VDBP, prealbumin, NGAL, fetuin-A, and AGP2 were found to be significantly elevated in SRNS using univariate analysis, with area under the receiver operating characteristic curves (AUCs ranging from 0.65 to 0.81. Multivariate analysis revealed a panel of all 10 markers that yielded an AUC of 0.92 for identification of SRNS. A subset of 5 markers (including VDBP, NGAL, fetuin-A, prealbumin, and AGP2 showed significant associations with SRNS and yielded an AUC of 0.85.

  11. Giant Cell Arteritis in a 12-Year-Old Girl Presenting with Nephrotic Syndrome

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    Zeinab A. El-Sayed

    2014-01-01

    Full Text Available Giant cell arteritis (GCA is rare in children. The kidneys are generally spared. We present a case of GCA in a 12-year-old girl with severe headache and tender scalp especially over the right temporal area. The right superficial temporal artery was cord like and nodular and the pulsations were barely felt. Several small tender nodular swellings were felt in the occipital area. She had been previously diagnosed as a case of nephrotic syndrome due to underlying membranoproliferative glomerulonephritis. This report is aimed at drawing attention to this rare form of vasculitis in children aiming at decreasing its morbidities.

  12. Remission of nephrotic syndrome diminishes urinary plasmin content and abolishes activation of ENaC

    DEFF Research Database (Denmark)

    Andersen, René F; Buhl, Kristian Bergholt; Jensen, Boye L.;

    2013-01-01

    BACKGROUND: Urinary plasmin activates the epithelial Na(+) channel (ENaC) in vitro and may possibly be a mechanism of sodium retention in nephrotic syndrome (NS). This study used a paired design to test the hypothesis that remission of NS is associated with a decreased content of urinary plasmin...... and reduced ability of patients' urine to activate ENaC. METHODS: Samples were collected during active NS and at stable remission from 20 patients with idiopathic NS, aged 9.1 ± 3.2 years. Plasminogen-plasmin concentration was measured with an enzyme-linked immunosorbent assay. Western immunoblotting...

  13. Long-term Effect of TCM Decoctions in Treatment of Nephrotic Syndrome

    Institute of Scientific and Technical Information of China (English)

    魏连波; 叶任高; 栾图; 吕瑞和; 陈保田

    2002-01-01

    Fifty-seven cases of nephrotic syndrome were treated with TCM decoctions as accessory treatment for prednisone and cyclophosphamide, and the effects were observed in a follow-up period of 5-15 years. The long-term complete remission rate of 68.4% and recurrence rate of 26.3% in the treatment group were respectively higher and lower than those in the control group (P<0.01, and P<0.01). The results suggested that the TCM decoctions were very helpful in treating this condition.

  14. Levamisole vs. cyclophosphamide for frequently-relapsing steroid-dependent nephrotic syndrome.

    Science.gov (United States)

    Alsaran, K; Grisaru, S; Stephens, D; Arbus, G

    2001-10-01

    A retrospective analysis comparing the first-time use of levamisole (L) or cyclophosphamide (C) as second-line therapy for children with frequently relapsing, steroid-dependent (FR/SD) nephrotic syndrome, was conducted at our center. The relapse rate and the total cumulative dose of prednisone during the year prior to L/C therapy was compared to that during the year following the institution of therapy with L or C in 51 patients, between July 1992 and June 1997. An analysis of covariance was used to adjust the outcome for differences between the 2 groups of treatment in the year prior to second-line drug initiation. In the L group the mean relapse rate was lowered by 0.28 relapses/patient/month and the mean cumulative dose of prednisone was reduced by 336 mg/m2/month versus 0.32 relapses/patient/month and 387 mg/m2/month in the C group (p = 0.395. p = 0.577). No significant difference in the effectiveness of L vs. C for therapy of FR/SD nephrotic syndrome could be identified in our patients. We conclude that L may be considered an alternative for C as a first second-line agent for these patients.

  15. Selected thrombosis and atherosclerosis risk factors in children with idiopathic nephrotic syndrome

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    Beata Bieniaś

    2012-04-01

    Full Text Available The purpose of our study was to evaluate selected thrombosis and atherosclerosis risk factors in children with idiopathic nephrotic syndrome (INS at three  stages of the disease (I – in acute phase before steroid therapy, II – during steroid therapy after resolution of proteinuria, III – in remission after completion of steroid therapy.In all children, serum total homocysteine, lipoprotein (a, total cholesterol, low-density lipoprotein cholesterol, high-density lipoprotein cholesterol and triglycerides levels were measured at three stages of the disease. Plasma antithrombin III, fibrinogen and D-dimer levels were also determined.  At all stages of INS, the serum t-HCY levels were similar and significantly higher than in controls.  Serum lipoprotein (a level, plasma antithrombin III, fibrinogen and D-dimer levels were significantly higher at stage I than at stages II, III and controls.In conclusion, children with INS are at high risk of thrombosis and atherosclerosis. Keywords: Idiopathic nephrotic syndrome, Homocysteine, Lipoprotein (a, Antithrombin III, fibrinogen, D-dimer

  16. Long-term linear growth of children with severe steroid-responsive nephrotic syndrome.

    Science.gov (United States)

    Emma, Francesco; Sesto, Antonella; Rizzoni, Gianfranco

    2003-08-01

    The present study was designed to evaluate the risk of permanent linear growth impairment in a selected group of 42 children with steroid-dependent nephrotic syndrome (SDNS) and 14 children with frequently relapsing nephrotic syndrome (FRNS). Longitudinal height measurements were available in all patients from the onset of the disease for a mean follow-up of 11.7+/-3.5 years. During the prepubertal period, patients lost 0.49+/-0.6 height SD score (HtSDS) ( P<0.001). Twenty-three patients have reached their final height with an average loss of 0.92+/-0.8 HtSDS from the onset of their disease ( P<0.001) and 0.68+/-0.7 from their target HtSDS ( P<0.001). The pubertal growth spurt was mildly delayed in male but not female patients. Steroid therapy, calculated as the mean duration of prednisone (PDN) treatment or as the average cumulative PDN dose, was the only predictor of poor growth evolution. Partial catch-up growth occurred after PDN withdrawal. Children with early onset NS and adolescent patients, who were still receiving PDN after the age of 9 years in girls and 11 years in boys, were at higher risk for HtSDS loss. In conclusion, children with severe steroid-responsive NS are at risk of permanent growth retardation secondary to prolonged courses of steroid treatment.

  17. [Treatment outcome using prednisone in corticosteroid-responsive primary nephrotic syndrome in children].

    Science.gov (United States)

    Brumariu, O; Cucer, Florentina; Munteanu, Mihaela; Haliţchi, Codruţa; Müller, R; Russu, R

    2005-01-01

    In children, the nephrotic syndrome is usually corticoid-responsive; approximately 70% of patients experience relapses, frequently triggered by infections. Our paper presents the results obtained using a 4 month prednisone regimen. This retrospective study included 83 children afflicted with nephrotic syndrome over a 10 year span. We analyzed: age at diagnosis, boys/girls ratio, response to corticoid treatment - after one month of prednisone and at the completion of the treatment course, number of relapses and their frequency, complications of prednisone treatment. The median age at diagnosis was 4.8 years, males predominating M:F = 1.5:1. Complete response after 4 weeks of prednisone therapy was noted in 98.79% of cases. We had 116 episodes of relapses during the first year of follow-up, occurring in 67.4% of children (27.9% were frequent relapsers, 11.62% subsequently became corticoid-dependent). Late relapses, after the first year, occurred in 32.55% of cases. We noted mostly mild adverse effects of the prednisone treatment: occurrence of infections during therapy (16.27%), cushingoid facies (37.2%), hirsutism (4.6%), high blood pressure (4.65%), stretch marks (2.32%). In conclusion, the 4 month prednisone treatment regimen is efficient in inducing and maintaining a remission. The incidence of relapses is 32.55%, comparable to the figure cited in larger studies. Serious adverse effects are significantly lower with this regimen compared to other corticoid treatment schemes. Key wo

  18. Concurrent nephrotic syndrome and acute renal failure caused by chronic lymphocytic leukemia (CLL): a case report and literature review.

    Science.gov (United States)

    Dou, Xianrui; Hu, Haitang; Ju, Yongle; Liu, Yongdong; Kang, Kaifu; Zhou, Shufeng; Chen, Wenfang

    2011-10-13

    Kidney injury associated with lymphocytic leukemia (CLL) is typically caused by direct tumor infiltration which occasionally results in acute renal failure. Glomerular involvement presenting as proteinuria or even nephrotic syndrome is exceptionally rare. Here we report a case of 54-year-old male CLL patient with nephrotic syndrome and renal failure. The lymph node biopsy confirmed that the patients had CLL with remarkable immunoglobulin light chain amyloid deposition. The renal biopsy demonstrated the concurrence of AL amyloidosis and neoplastic infiltration. Combined treatment of fludarabine, cyclophosphamide and rituximab resulted in remission of CLL, as well as the renal disfunction and nephrotic syndrome, without recurrence during a 12-month follow-up. To our knowledge, this is the first case of CLL patient showing the nephrotic syndrome and acute renal failure caused by AL amyloidosis and neoplastic infiltration. Though AL amyloidosis caused by plasma cell dyscrasia usually responses poorly to chemotherapy, this patient exhibited a satisfactory clinical outcome due to successful inhibition of the production of amylodogenic light chains by combined chemotherapy.

  19. Clinical Observation of Tiaojining (调激宁) Granule Combined with Corticosterone in Treating Infantile Primary Nephrotic Syndrome

    Institute of Scientific and Technical Information of China (English)

    2000-01-01

    In order to observe the effect of Tiaojining granule (调激宁, TJNG) combined with corticosteroids (CS) to enhance the efficacy and alleviate the side-effects, the authors used the combination therapy of TJNG and CS in treating infantile primary nephrotic syndrome (IPNS) in 1990-1996, and the following is the summary of clinical data.

  20. A Unique Cause of Proteinuria in Pregnancy: Class II Lupus Nephritis with Concomitant Minimal Change Disease

    Science.gov (United States)

    Kunjal, Ryan; Adam-Eldien, Rabie; Makary, Raafat; Jo-Hoy, Francois; Heilig, Charles W.

    2016-01-01

    We report the case of a 22-year-old African American female who presented to another facility for routine follow-up in the 34th week of pregnancy with lower extremity swelling and nephrotic-range proteinuria. Although she was normotensive, it was initially thought that she had preeclampsia. She was monitored carefully and delivery was induced at 37 weeks of gestation. She was transferred to our hospital, where she was diagnosed with systemic lupus erythematosus (SLE) based on clinical and laboratory criteria. Renal biopsy revealed a surprising finding of minimal change disease (MCD) concomitant with class II lupus nephritis (LN). She was managed with pulses and then tapering doses of steroid therapy with dramatic resolution of the nephrotic syndrome. This case demonstrates not only the rare de novo occurrence of SLE in pregnancy, but the unique finding of MCD coexisting with class II LN. We propose that altered T cell activity may be the link between these seemingly distinct entities.

  1. Glomerular ultrafiltration and apical tubular action of IGF-I, TGF-beta, and HGF in nephrotic syndrome.

    Science.gov (United States)

    Wang, S N; Lapage, J; Hirschberg, R

    1999-10-01

    In nephrotic glomerulopathies, there is ultrafiltration of high molecular weight forms of insulin-like growth factor-I (IGF-I), hepatocyte growth factor (HGF), and transforming growth factor-beta (TGF-beta), which are bioactive in tubular fluid and act through apical tubular receptors. Experimental evidence indicates that ultrafiltered IGF-I, HGF, and TGF-beta may contribute to increased tubular phosphate and sodium absorption, synthesis of extracellular matrix proteins, and secretion of chemokines such as monocyte chemoattractant protein-1 (MCP-1). Through these mechanisms, glomerular proteinuria may contribute to tubulointerstitial pathobiology in nephrotic syndrome.

  2. The treatment of relapsing primary nephrotic syndrome in children

    Institute of Scientific and Technical Information of China (English)

    WANG Ya-ping; LIU Ai-min; DAI Yu-wen; YANG Cheng; TANG Hong-feng

    2005-01-01

    Objective: To explore better therapy and reduce the rate of re-relapse of primary nephritic syndrome in children who had been treated with corticosteroids but relapsed. Methods: Eighty relapsers were enrolled from Jan. 1994 to Apr. 2000, who were randomly divided into two groups. The treatment group (n=39) had been treated with tripterysium glucosides for three months,with the control group (n=41) members were treated with cyclophosphmide (CTX) by intermission intravenous pulse, with total dose of CTX not being more than 150 mg/kg. Prednisone, meanwhile, was given to both groups. The total treatment period of prednisone was prolonged by 12-18 months. Results: After following up for 3-7 years, the re-relapse rates of both groups were observed. The re-relapse rate of the treatment group was 28.2% to 29.3% in the CTX-controlled group. The re-relapse rates between two groups were almost similar, and with no observed significant difference (P>0.05). The side effect of tripterysium glucosides was less than that of CTX. Conclusion: For the treatment of relapsing nephritic syndrome in children, the combination of tripterysium glucosides and prolonged corticosteroid therapy is as effective as the regimen of CTX plus prolonged use of prednisone.

  3. Complete Remission of Minimal Change Disease Following an Improvement of Lung Mycobacterium avium Infection.

    Science.gov (United States)

    Yamashiro, Aoi; Uchida, Takahiro; Ito, Seigo; Oshima, Naoki; Oda, Takashi; Kumagai, Hiroo

    A 46-year-old woman suddenly developed peripheral edema. Her massive proteinuria, hypoproteinemia, and renal biopsy findings yielded the diagnosis of minimal change disease (MCD). In addition, lung Mycobacterium avium infection was diagnosed according to a positive culture of her bronchoalveolar lavage fluid. The lung lesion was improved by anti-nontuberculous mycobacteria therapy. Surprisingly, her proteinuria also gradually decreased and she attained complete remission of MCD without any immunosuppressive therapy. She has subsequently remained in complete remission. We herein report an interesting case of MCD with lung Mycobacterium avium infection, suggesting a causal relationship among infection, immune system abnormality, and MCD/nephrotic syndrome.

  4. Conformational Transitions and Glycation of Serum Albumin in Patients with Minimal-Change Glomerulopathy

    Science.gov (United States)

    Hong, Sae Yong; Lee, Eun Young; Yang, Jong Oh; Kim, Tae Yeong; Kim, Eun Hee; Cheong, Mi Young; Kim, Soo Hyun; Cheong, Chae Joon

    2004-01-01

    Background There has been a lack of study on the structural changes of serum albumin in patients with minimal change disease (MCD). To determine whether glycation and/or conformational transitions of albumin are involved in the pathogenesis of albuminuria, nine patients with MCD were enrolled in a prospective follow-up study for comparison of these parameters in serum albumin during the remission and relapse of nephrotic syndrome. Methods Circular dichroism measurements were made with purified albumin. Ellipticities at each wavelength were transformed to mean residue ellipticity. Monosaccharide composition was analyzed by high-pH anion-exchange chromatography with pulsed amperometric detection. Results There was no difference in the proportions of α-helix, β-conformation, and β-turn of albumin between the sera of control patients and those with nephrotic syndrome. However, the proportion of the random configuration was slightly higher in the plasma albumin of patients in relapse than in those in remission. The proportion of the random configuration was lower in the albumin of the serum than in the urine of patients with nephrotic syndrome, but there was no difference in the proportions of α-helix, β-conformation, and β-turn of albumin between their plasma and urine. Conclusion Our results suggest that conformational changes in albumin are involved in albuminuria in patients with MCD. PMID:15481604

  5. Aberrant glomerular filtration of urokinase-type plasminogen activator in nephrotic syndrome leads to amiloride-sensitive plasminogen activation in urine

    DEFF Research Database (Denmark)

    Staehr, Mette; Buhl, Kristian B; Andersen, René F

    2015-01-01

    (uPA) in vitro. It was hypothesized that uPA is abnormally filtered to pre-urine and is inhibited in urine by amiloride in nephrotic syndrome. This was tested by determination of Na+-balance, uPA protein and activity and amiloride concentration in urine from rats with puromycin aminonucleoside (PAN......In nephrotic syndrome, aberrant glomerular filtration of plasminogen and conversion to active plasmin in pre-urine is thought to activate proteolytically ENaC and contribute to sodium retention and edema. The ENaC blocker amiloride is an off-target inhibitor of urokinase-type plasminogen activator......) induced nephrotic syndrome. Urine samples from 6 adult and 18 pediatric patients with nephrotic syndrome were analyzed for uPA activity and protein. PAN-treatment induced significant proteinuria in rats which coincided with increased urine uPA protein and activity, increased urine protease activity...

  6. Unusual pediatric co-morbility: autoimmune thyroiditis and cortico-resistant nephrotic syndrome in a 6-month-old Italian patient

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    Urbano Flavia

    2012-10-01

    Full Text Available Abstract We report on a case of autoimmune thyroiditis in a 6-month-old patient with cortico-resistant nephrotic syndrome. Normal serum levels of thyroid hormons and thyroid-stimulating hormone were detected with high titers of circulant antithyroid antibodies and a dysomogeneous ultrasound appearance of the gland, typical of autoimmune thyroiditis. The research of maternal thyroid antibodies was negative. This is the first case of autoimmune thyroiditis found in such a young patient with pre-existing nephrotic syndrome ever described in literature. This association is random because nephrotic syndrome does not have an autoimmune pathogenesis and the genes involved in autoimmune thyroiditis are not related to those of nephrotic syndrome.

  7. Congenital Syphilis Presenting with Only Nephrotic Syndrome: Reemergence of a Forgotten Disease.

    Science.gov (United States)

    Kim, Yun Hee; Song, Ji Ho; Kim, Chan Jong; Yang, Eun Mi

    2017-08-01

    Syphilis infection has re-emerged after years of declining incidence. The prevalence of congenital syphilis (CS) has increased in Korea and other countries during the last few decades. Untreated infants develop symptoms such as rhinorrhea, anemia, jaundice, cutaneous lesions, hepatosplenomegaly, and pseudoparalysis within weeks or months. Significant renal disease is uncommon in CS, and clinical renal involvement varies from mild transient proteinuria to frank nephrosis. We report a 2-month-old infant with CS who presented with only nephrotic syndrome (NS). The previously healthy infant presented with NS and showed no other syphilitic manifestations. Remission of the NS was achieved with adequate penicillin treatment. No recurrence of proteinuria was observed during the 1 year of follow-up. Although rare, this long forgotten disease continues to affect pregnant women, resulting in prenatal or postnatal mortality. We still consider the possibility of syphilitic nephropathy and therefore serologic testing for congenital NS. © 2017 The Korean Academy of Medical Sciences.

  8. A case of abdominal aortic thrombosis associated with the nephrotic syndrome

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    Jannet Labidi

    2017-01-01

    Full Text Available Thromboembolic disease is an important and frequent complication in patients with the nephrotic syndrome (NS, and the consequences are often severe. Usually, the venous system is affected. Arterial thrombosis has rarely been reported and occurs mainly in children. We report the case of a 27-year-old man with a history of NS due to focal and segmental glomerulosclerosis resistant to steroids and cyclosporine, admitted for bilateral pain in the calves. Aortogram revealed a suspended thrombus in the abdominal aorta just below the origin of the renal arteries with embolism into the left tibioperoneal trunk and the right anterior tibial artery. Endarterectomy was performed followed by systemic heparinization with a good outcome. Arterial thrombosis is rare and must be prevented.

  9. Urinary AQP2 excretion is increased during nephrotic syndrome and is associated with reduced urine production

    DEFF Research Database (Denmark)

    Andersen, René Frydensbjerg; Kamperis, Konstantinos; Frøkjær, Jørgen

    of the study was to investigate the urinary presence of the renal water channel AQP2 and it´s correlation to urine production in children with NS. METHODS: A total of 20 (7 girls) children with NS were included with a mean age of 9.1±3.2 yrs. Urine samples were collected from the second morning void at debut......OBJECTIVES: Edema is a hallmark of nephrotic syndrome (NS) and has largely been attributed to sodium retention. Yet low plasma sodium is frequently observed in children with acute NS suggesting that a disturbance in renal water handling may coexist together with sodium retention. The aim......, patients exhibit an increased excretion of AQP2 in urine compared with remission and increased uAQP2 concentration correlated with reduced urine production. Further studies are needed to investigate if uAQP2 contributes to a positive free water balance in NS independently of sodium retention....

  10. MAC-2BP is increased in plasma during nephrotic syndrome and is produced by PBMC

    DEFF Research Database (Denmark)

    Andersen, René Frydensbjerg; Kamperis, Konstantinos; Rittig, Søren

    of the study was to investigate the urinary presence of the renal water channel AQP2 and it´s correlation to urine production in children with NS. METHODS: A total of 20 (7 girls) children with NS were included with a mean age of 9.1±3.2 yrs. Urine samples were collected from the second morning void at debut......OBJECTIVES: Edema is a hallmark of nephrotic syndrome (NS) and has largely been attributed to sodium retention. Yet low plasma sodium is frequently observed in children with acute NS suggesting that a disturbance in renal water handling may coexist together with sodium retention. The aim......, patients exhibit an increased excretion of AQP2 in urine compared with remission and increased uAQP2 concentration correlated with reduced urine production. Further studies are needed to investigate if uAQP2 contributes to a positive free water balance in NS independently of sodium retention....

  11. Vitamin D status is insufficient in the majority of children at diagnosis of nephrotic syndrome

    DEFF Research Database (Denmark)

    Nielsen, Cecilie Ane; Jensen, Jens-Erik Bech; Cortes, Dina

    2015-01-01

    INTRODUCTION: Children with nephrotic syndrome (NS) are treated for at least 12 weeks with high doses of prednisolone, which may be harmful to the bones. Vitamin D deficiency is also harmful to the bones. METHODS: This was a prospective study of consecutive children with first episode of NS...... at the time of their diagnosis before treatment with glucocorticoids. The following plasma levels were measured: 25-hydroxy-vitamin-D (25(OH)D), albumin, ionised calcium, phosphate, parathyroid hormone (PTH), alkaline phosphatase and creatinine. The glomerular filtration rate (GFR) was estimated from...... the Schwartz formula, and only patients with normal values were included. RESULTS: A total of 14 children were examined, 13 (93%) had 25(OH)D deficiency including 12 (86%) with moderate or severe vitamin D deficiency. The plasma 25(OH)D was positively associated with plasma albumin (p = 0.031) and negatively...

  12. Síndrome nefrótico familiar Familial nephrotic syndrome

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    Sandalio Durán Álvarez

    2007-12-01

    Full Text Available Se han identificado mutaciones en genes que codifican proteínas podocitarias y existen evidencias que sugieren que el defecto primario del síndrome nefrótico idiopático con lesión mínima pueda estar en el podocito. Se analizó el grado de parentesco de 12 pacientes con síndrome nefrótico (hermanos o padres y se destacaron las principales características clínicas e histológicas. Entre los 334 niños nefróticos estudiados encontramos a 12, correspondientes a 7 familias, con síndrome nefrótico córtico-sensible (3,6 %. Hubo predomino del sexo masculino (relación 2:1. En 4 familias sin antecedentes de la enfermedad hallamos a 9 niños afectados, dos de ellos, gemelos idénticos. En las tres familias restantes, dos de las madres y un padre, habían padecido la enfermedad. Todos los niños respondieron al tratamiento con esteroides y en los tres pacientes con estos antecedentes, la evolución fue similar a la de sus progenitores. El síndrome nefrótico con lesión mínima y buena respuesta a los esteroides que se presenta en las familias puede no ser un hecho casual y su aparición clínica puede estar relacionada con alguna mutación genética que la condicione. Para definir claramente este aspecto se necesitan más investigaciones sobre el tema.Gene mutations that codify podocytary proteins have been identified, and there are evidences that suggest that the primary defect of their idiopathic nephrotic syndrome with minimal injury may be in the podocyte. The kinship degree of 12 patients with nephrotic syndrome (siblings or parents was analyzed, and the main clinical and histological characteristics were stressed. Among the 334 nephrotyic children studied, we found 12, corresponding to 7 families, with corticosensitive nephrotic syndrome (3.6 %. There was a prevalence of males (ratio 2:1. In 4 families without history of the disease there were 9 children affected, two of them, identical twins. In the other three families, two of the

  13. Albumin and Furosemide Combination for Management of Edema in Nephrotic Syndrome: A Review of Clinical Studies

    Directory of Open Access Journals (Sweden)

    Margaret Duffy

    2015-10-01

    Full Text Available The treatment of edema in patients with nephrotic syndrome is generally managed by dietary sodium restriction and loop diuretics. However, edema does not improve in some patients despite adequate sodium restriction and maximal dose of diuretics. In such patients, combination of albumin and a loop diuretic may improve edema by diuresis and natriuresis. The response to this combination of albumin and a diuretic has not been observed in all studies. The purpose of this review is to discuss the physiology of diuresis and natriuresis of this combination therapy, and provide a brief summary of various studies that have used albumin and a loop diuretic to improve diuretic-resistant edema. Also, the review suggests various reasons for not observing similar results by various investigators.

  14. Nephrotic Syndrome Following H1N1 Influenza in a 3-Year-Old Boy

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    Pio Liberatore

    2012-06-01

    Full Text Available Background: The pandemic influenza A/H1N1, spread through the world in 2009, producing a serious epidemic in Italy. Complications are generally limited to patients at the extremes of age (65years and those with comorbid medical illness. The most frequent complications of influenza involve the respiratory system.Case Presentation: A 3-year-old boy with a recent history of upper respiratory tract infection developed a nephrotic syndrome. Together with prednisone, furosemide and albumin bolus, a therapy with oseltamivir was started since the nasopharyngeal swab resulted positive for influenza A/H1N1. Clinical conditions andlaboratory findings progressively improved during hospitalization, becoming normal during a 2 month follow up.Conclusion: The possibility of a renal involvement after influenza A/H1N1 infection should be considered.

  15. [Treating the symptoms in nephrotic syndrome: which therapeutic strategies are evidence based in the treatment of proteinuria?].

    Science.gov (United States)

    Kisner, T; Müller, R-U; Benzing, T

    2011-02-01

    Glomerular diseases are among the most common renal pathologies leading frequently to end-stage renal disease. Clinical disease can be divided into five different groups the features of which are determined by the underlying pathophysiology. One of these five clinical syndromes is the nephrotic syndrome, which is characterized by proteinuria > 3.5 g/day accompanied by hypalbuminemia, hyperlipoproteinemia and pronounced edema. The nephrotic syndrome may be the clinical manifestation of a row of underlying diseases. The pathophysiological basics had remained elusive for decades, yet recently significant progress which allows for establishing new therapeutic strategies has been made. A major breakthrough in understanding the function of the glomerular filter unit has been possible in the last years through both genetic and cell biological studies, which have revealed a crucial role for the visceral epithelial cells of the glomerulus - the podocytes. By now various factors have been found causing podocyte damage, such as toxines, immunological phenomena or systemic disease like diabetes mellitus.

  16. ANGIOTENSIN CONVERTING ENZIME GENE I/D POLYMORPHISM AND PROMISSING OF RENOPROTECTIVE AND IMMUNOSUPRESSIVE THERAPY IN CHILDREN WITH NEPHROTIC SYNDROME

    Directory of Open Access Journals (Sweden)

    Zh.P. Sharnova

    2007-01-01

    Full Text Available To investigate the role of i/d polymorphism gene angiotensin converting enzyme (ACE in promissing of renoprotective and immunosupressive therapy in children with nephrotic syndrome syndrome (NS we determined the genotypes of ACE in 76 children with ns including 22 children with chronic renal failure (CRF. the analysis of treatment effect with inhibitor ace in patients with steroid resistant ns (SRNS demonstrated decreasing of renoprotective effect of these drugs in patients with DD genotype compared with ii and id genotypes (р = 0,033 by similar degree of the glomerular filtration rate (GFR, proteinuria and blood pressure decrease in these patients. Percentage of DD genotype in patients with ns refractory to therapy of cyclosporin a were height compared with patients, sensitive to this therapy.Key words: nephrotic syndrome, chronic renal failure, polymorphism of genes, renin-angiotensin system.

  17. Clinical Study of Gushen Tablet(固肾片) in Reducing Children's Nephrotic Syndrome Relapse

    Institute of Scientific and Technical Information of China (English)

    云鹰; 高雅; 马玉宏; 刘望乐; 赵蒙; 高智铭

    2003-01-01

    Objective: To explore the effect of Gushen tablet (固肾片, GST) in reducing the relapse of children′s nephrotic syndrome and the possible mechanism of drugs used. Methods: Fifty children with primary nephrotic syndrome who had been induced and alleviated with regular glucocorticoid (GC) were randomly divided into two groups: the GST group used GST and standard middle-long term course of GC,and the control group adopted standard middle-long term course of GC and immunoinhibitory or immunomodulatory agents for treatment. The 0.5, 1 and 2 years after the treatment the relapse episodes, time for urinary protein negative conversion after relapse, the episodes of patienfs infection and relapse after infection were evaluated. Before and after treatment the plasma cortisol and T lymphocyte subpopulation were determined. Results: The relapse rate of GST group: the rates after 0.5, 1, 2 years were 20.0 %, 30.0 %and 40.9%, and the frequent relapse rate were 0, 6.7% and 9.2% respectively, which were lower than those of control group (60.0%, 70. 0%, 69.2% and 25.0%, 15.0%, 15.4% respectively) ; in the GST group no relapse occurred within 0. 5 year, the relapse rate after 1 and 2 years reduced by 40. 0% and 28.3%, compared with those of the control group (all P<0.05) ; during the observation period, the mean infection/every child patient was 1.86 episodes in GST group, after infection the nephrotic relapse rate was 28. 3%, which was lower than that of the control group (2.25 episodes, 71. 1%, P<0. 05); the relapse per patient in GST group was 0.8 episodes, time for urinary protein negative conversion was 12.00±8.98 days, lower than those of control group (1.6 episodes, 20.75±11.95 days, P<0.05) ; 3 months after GST treatment the plasma cortisol level normalized, and the CD4/CD8 ratio elevated (P<0. 05). Conclusion: GST could possibly reduce the relapse of children nephrosis, and the frequent relapse and relapse episodes, and the time for post-relaptic urinary

  18. Defect in urinary acidification in nephrotic syndrome and its correction by furosemide.

    Science.gov (United States)

    Rodriguez-Soriano, J; Vallo, A; Castillo, G; Oliveros, R

    1982-01-01

    6 children with idiopathic nephrotic syndrome were investigated during clinical relapse to examine the interrelation between distal urinary acidification and urinary sodium excretion. Blood and urine studies were initiated 4 h after completion of ammonium chloride loading, prior to and following the intravenous administration of furosemide. Values for plasma bicarbonate before and after furosemide administration were not significantly different. In the control periods, when urinary sodium excretion was very low, a defect in urinary acidification was demonstrated (UPH: 6.09 +/- (SD) 0.27; UTAV and UNH4V: 12.6 +/- 3.1 and 36.4 +/- 15.8 mumol/min/1.73 m2, respectively.) Following furosemide-induced natriuresis UPH fell to 4.81 +/- 0.25 (p less than 0.0005), and UTA2V and UNH4V increased to 46.3 +/- 15.8 and 125.6 +/- 49.5 mumol/min/1.73 m2, respectively (p less than 0.002). No overall correlation existed between urinary acidity, both considered as hydrogen ion concentration and as hydrogen ion excretion, and rate of urinary sodium excretion; but significant correlations were present between hydrogen ion concentration in the urine and both UC1V-UNAV (r = 0.38, p less than 0.05), and UC1V - (UNaV + UKV) (r = 0.64, p less than 0.01). These results indicate that the defect in distal urinary acidification observed in nephrotic syndrome is probably due to decreased delivery of sodium to the distal nephron. The enhanced secretion of hydrogen ion observed after furosemide administration may be related both to increased sodium delivery and to greater sodium than chloride reabsorption in the collecting duct.

  19. Nephrotic Syndrome

    Science.gov (United States)

    ... kidney disease. Diabetes can lead to kidney damage (diabetic nephropathy) that affects the glomeruli. Systemic lupus erythematosus. This ... will I always have it? What are my treatment options? And which do you recommend for me? ...

  20. Nephrotic syndrome

    Science.gov (United States)

    ... by: Charles Silberberg, DO, private practice specializing in nephrology, affiliated with New York Medical College, Division of Nephrology, Valhalla, NY. Review provided by VeriMed Healthcare Network. ...

  1. Renal manifestations of human brucellosis: First report of minimal change disease

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    Nikolaos Sabanis

    2016-01-01

    Full Text Available Human brucellosis is considered a great example of the complexity of clinical manifestations possibly affecting multiple organs or systems. Renal manifestations of human brucellosis have been documented in few case reports and one case series. Herein, we present a case of Nephrotic syndrome (NS due to minimal change disease in the course of acute brucellosis. A 53-year-old male farmer was admitted to our department with acute brucellosis and NS. Renal biopsy revealed minimal change disease. Combined treatment with prednisone (1 mg/kg, rifampicin (600 mg/day, and doxycycline (200 mg/day was initiated. Complete remission of NS was achieved at the end of the fourth week. One year later, the patient remained in complete remission of NS without any sign of relapse of brucellosis.

  2. Renal manifestations of human brucellosis: First report of minimal change disease.

    Science.gov (United States)

    Sabanis, Nikolaos; Gavriilaki, Eleni; Paschou, Eleni; Tsotsiou, Eleni; Kalaitzoglou, Asterios; Kavlakoudis, Christos; Vasileiou, Sotirios

    2016-05-01

    Human brucellosis is considered a great example of the complexity of clinical manifestations possibly affecting multiple organs or systems. Renal manifestations of human brucellosis have been documented in few case reports and one case series. Herein, we present a case of Nephrotic syndrome (NS) due to minimal change disease in the course of acute brucellosis. A 53-year-old male farmer was admitted to our department with acute brucellosis and NS. Renal biopsy revealed minimal change disease. Combined treatment with prednisone (1 mg/kg), rifampicin (600 mg/day), and doxycycline (200 mg/day) was initiated. Complete remission of NS was achieved at the end of the fourth week. One year later, the patient remained in complete remission of NS without any sign of relapse of brucellosis.

  3. Systemic mastocytosis associated with minimal change disease

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    Tony Amin

    2016-07-01

    Full Text Available Systemic Mastocytosis (SM has not previously been reported in association with minimal change disease (MCD. Mastocytosis is a rare myeloproliferative disease that is characterised by uncontrolled proliferation of aberrant mast cells. This can lead to a wide variety of symptoms and can present as either a cutaneous or a systemic disease. Systemic manifestations usually include bone marrow, intestine, liver and splenic infiltration of mast cells, with reports of renal manifestations being rare. This is a case report of a 70-year-old man who was known to have Systemic Mastocytosis and who presented with nephrotic range proteinuria. Renal biopsy diagnosed Minimal Change Disease with mast cell infiltration being an identified by C-kit staining. The patient was treated with steroids and is currently in remission of the proteinuria.

  4. 老年肾病综合征的临床特点及病理类型分析%Nephrotic syndrome in the elderly:clinical and pathological analyses

    Institute of Scientific and Technical Information of China (English)

    刘小聪; 邵思南; 张燕林

    2015-01-01

    目的:探讨老年肾病综合征的临床表现和病理特点。方法收集60岁以上老年肾病综合征患者41例,对其临床表现及肾活检病理进行分析。结果41例患者中,原发性肾小球疾病28例(68.29%),病理类型以膜性肾病最常见,其次为局灶节段性肾小球硬化、微小病变性肾病;继发性肾病综合征13例(31.71%)。除肾病综合征表现外,伴血尿25例(60.98%),高血压24例(58.54%),肾功能异常20例(48.78%)。病程中出现感染11例(26.83%)。肾活检后无严重并发症。结论老年肾病综合征最常见的病因为原发性肾小球疾病,病理类型以膜性肾病最常见。主要临床表现为浮肿、血尿、高血压、肾功能减退,并发症主要为感染。行肾活检对老年患者是安全的。%Objective To analyze the clinical manifestations and pathologic characters of nephrotic syndrome in elderly patients.Methods We collected 41 nephrotic syndrome patients who were more than 60 years old in our hospital from January 2009 to January 2014 and analyzed their clinical manifestations and pathologic characters.Re-sults All of the 41 cases with nephrotic syndrome were diagnosed by renal biopsy, with 28 cases in primary glomeru-lar disease(68.29%), in which membranous nephropathy was the most common pathological type, followed by focal segmental glomerular sclerosis and minimal change disease, and with 13 cases in secondary nephrotic syndrome (31.71%).Except for nephrotic syndrome, hematuria was found in 25 cases(60.98%), hypertension in 24 cases (58.54%), renal dysfunction in 20 cases(48.78%).Infection was the major complication(26.83%).No serious complications were found after the renal biopsy.Conclusion The most common disease in the elderly patients with nephrotic syndrome is primary glomerular disease, in which membranous nephropathy is the most common pathological type.Major clinical manifestations are edema

  5. Case reports Juvenile idiopathic arthritis complicated by amyloidosis with secondary nephrotic syndrome – effective treatment with tocilizumab

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    Małgorzata Kwiatkowska

    2015-08-01

    Full Text Available A case report of a boy with juvenile idiopathic arthritis since the age of 2 years, generalized onset, complicated by nephrotic syndrome due to secondary type A amyloidosis is presented. In the patient the disease had an especially severe course, complicated by frequent infections, making routine treatment difficult. Amyloidosis was diagnosed in the 5th year of the disease based on a rectal biopsy. Since the disease onset the boy has been taking prednisolone and sequentially cyclosporine A, methotrexate, chlorambucil, etanercept, and cyclophosphamide. Clinical and laboratory remission was observed after treatment with tocilizumab. After 42 months of treatment with tocilizumab the boy’s condition is good. There is no pain or joint edema, and no signs of nephrotic syndrome.

  6. Síndromes nefróticos congénitos y hereditarios Congenital and heritable nephrotic syndromes

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    Sandalio Durán Álvarez

    2011-03-01

    Full Text Available En los últimos años se han identificado muchos síndromes nefróticos familiares y esporádicos que no responden a los tratamientos habituales (esteroides e inmunosupresores, evolucionan con relativa rapidez a la insuficiencia renal crónica y se producen por mutaciones genéticas. La mayoría de los síndromes nefróticos que se trasmiten genéticamente y que pueden ser congénitos, presentarse en el primer año de la vida, o en el niño mayor, son atribuidos a mutaciones en los genes NPHS1, NPHS2, WT1 y LAMB2. Otros síndromes nefróticos producidos por mutaciones genéticas pueden no manifestarse hasta la adultez. El objetivo fundamental de esta revisión fue llamar la atención sobre los síndromes nefróticos producidos por mutaciones genéticas en los que no sólo no se obtienen resultados con los tratamientos inmunosupresores, si no en los que dichos tratamientos pueden ser perjudiciales para el paciente.In past years many familial and sporadic nephrotic syndromes refractory to usual treatments (steroids and immunosuppressives, evolve quickly to a chronic renal failure produced by genetic mutations. Most of nephrotic syndromes genetically transmitted and that may be congenital, present in the first year of life or in the older child, are attributable to NPHS1, NPHS2, WT1 and KLAMB2 gen mutations. Other nephrotic syndromes produced by genetic mutations may not appear until adulthood. The main objective of present review was to alert on the nephrotic syndromes produced by genetic mutations without response to immunosuppressive treatments, but on those in which such treatment may be dangerous for patient.

  7. Nephrotic presentation in hydatid cyst disease with predominant tubulointerstital disease

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    Feroz Aziz

    2009-06-01

    Full Text Available Feroz Aziz1, Tanmay Pandya1, Himanshu V Patel1, Paladugu Ramakrishna1, Kamal R Goplani1, Manoj Gumber1, Aruna V Vanikar2,  Kamal Kanodia2, Pankaj R Shah1, Hargovind L Trivedi11Department of Nephrology and Transplantation Medicine; 2Department of Pathology, Lab Medicine, Transfusion Services and Immunohematology, G.R. Doshi and K.M. Mehta Institute of Kidney Diseases and Research Centre (IKDRC, Ahmedabad, Gujarat, IndiaAbstract: Renal involvement, which can rarely occur in echinococcosis, more commonly manifests as hydatid cyst of the kidney. Scattered case reports of nephrotic syndrome secondary to hydatid cyst in the liver or lung have been reported for over two decades. The glomerular picture varied from minimal change lesion to mesangiocapillary glomerulonephritis. We report a case of predominantly tubulointerstitial nephritis with mesangioproliferative glomerulonephritis in a patient with hepatic hydatid cyst which responded to cyst resection alone. Keywords: echinococcosis, hydatid cyst, kidney, nephrotic syndrome, tubulointerstitial nephritis

  8. Cell biology and genetics of minimal change disease.

    Science.gov (United States)

    Saleem, Moin A; Kobayashi, Yasuko

    2016-01-01

    Minimal change disease (MCD) is an important cause of nephrotic syndrome and is characterized by massive proteinuria and hypoalbuminemia, resulting in edema and hypercholesterolemia. The podocyte plays a key role in filtration and its disruption results in a dramatic loss of function leading to proteinuria. Immunologic disturbance has been suggested in the pathogenesis of MCD. Because of its clinical features, such as recurrent relapse/remission course, steroid response in most patients, and rare familial cases, a genetic defect has been thought to be less likely in MCD. Recent progress in whole-exome sequencing reveals pathogenic mutations in familial cases in steroid-sensitive nephrotic syndrome (SSNS) and sheds light on possible mechanisms and key molecules in podocytes in MCD. On the other hand, in the majority of cases, the existence of circulating permeability factors has been implicated along with T lymphocyte dysfunction. Observations of benefit with rituximab added B cell involvement to the disease. Animal models are unsatisfactory, and the humanized mouse may be a good model that well reflects MCD pathophysiology to investigate suggested "T cell dysfunction" directly related to podocytes in vivo. Several candidate circulating factors and their effects on podocytes have been proposed but are still not sufficient to explain whole mechanisms and clinical features in MCD. Another circulating factor disease is focal segmental glomerulosclerosis (FSGS), and it is not clear if this is a distinct entity, or on the same spectrum, implicating the same circulating factor(s). These patients are mostly steroid resistant and often have a rapid relapse after transplantation. In clinical practice, predicting relapse or disease activity and response to steroids is important and is an area where novel biomarkers can be developed based on our growing knowledge of podocyte signaling pathways. In this review, we discuss recent findings in genetics and podocyte biology in MCD.

  9. Cell biology and genetics of minimal change disease

    Science.gov (United States)

    Saleem, Moin A.; Kobayashi, Yasuko

    2016-01-01

    Minimal change disease (MCD) is an important cause of nephrotic syndrome and is characterized by massive proteinuria and hypoalbuminemia, resulting in edema and hypercholesterolemia. The podocyte plays a key role in filtration and its disruption results in a dramatic loss of function leading to proteinuria. Immunologic disturbance has been suggested in the pathogenesis of MCD. Because of its clinical features, such as recurrent relapse/remission course, steroid response in most patients, and rare familial cases, a genetic defect has been thought to be less likely in MCD. Recent progress in whole-exome sequencing reveals pathogenic mutations in familial cases in steroid-sensitive nephrotic syndrome (SSNS) and sheds light on possible mechanisms and key molecules in podocytes in MCD. On the other hand, in the majority of cases, the existence of circulating permeability factors has been implicated along with T lymphocyte dysfunction. Observations of benefit with rituximab added B cell involvement to the disease. Animal models are unsatisfactory, and the humanized mouse may be a good model that well reflects MCD pathophysiology to investigate suggested “T cell dysfunction” directly related to podocytes in vivo. Several candidate circulating factors and their effects on podocytes have been proposed but are still not sufficient to explain whole mechanisms and clinical features in MCD. Another circulating factor disease is focal segmental glomerulosclerosis (FSGS), and it is not clear if this is a distinct entity, or on the same spectrum, implicating the same circulating factor(s). These patients are mostly steroid resistant and often have a rapid relapse after transplantation. In clinical practice, predicting relapse or disease activity and response to steroids is important and is an area where novel biomarkers can be developed based on our growing knowledge of podocyte signaling pathways. In this review, we discuss recent findings in genetics and podocyte biology in

  10. Prediction of Negative Conversion Days of Childhood Nephrotic Syndrome Based on the Improved Backpropagation Neural Network with Momentum

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    Yi-jun Liu

    2015-12-01

    Full Text Available Childhood nephrotic syndrome is a chronic disease harmful to growth of children. Scientific and accurate prediction of negative conversion days for children with nephrotic syndrome offers potential benefits for treatment of patients and helps achieve better cure effect. In this study, the improved backpropagation neural network with momentum is used for prediction. Momentum speeds up convergence and maintains the generalization performance of the neural network, and therefore overcomes weaknesses of the standard backpropagation algorithm. The three-tier network structure is constructed. Eight indicators including age, lgG, lgA and lgM, etc. are selected for network inputs. The scientific computing software of MATLAB and its neural network tools are used to create model and predict. The training sample of twenty-eight cases is used to train the neural network. The test sample of six typical cases belonging to six different age groups respectively is used to test the predictive model. The low mean absolute error of predictive results is achieved at 0.83. The experimental results of the small-size sample show that the proposed approach is to some degree applicable for the prediction of negative conversion days of childhood nephrotic syndrome.

  11. Astragalus in the Prevention of Upper Respiratory Tract Infection in Children with Nephrotic Syndrome: Evidence-Based Clinical Practice

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    Chuan Zou

    2013-01-01

    Full Text Available Aims. To explore whether Astragalus or its formulations could prevent upper respiratory infection in children with nephrotic syndrome and how best to use it. Methods. We transformed a common clinical question in practice to an answerable question according to the PICO principle. Databases, including the Cochrane Library (Issue 5, 2012, PUBMED (1966–2012.8, CBM (1978–2012.8, VIP (1989–2012.8, and CNKI (1979–2012.8, were searched to identify Cochrane systematic reviews and clinical trials. Then, the quality of and recommendations from the clinical evidence were evaluated using the GRADEpro software. Results. The search yielded 537 papers. Only two studies with high validity were included for synthesis calculations. The results showed that Astragalus granules could effectively reduce URTI in children with nephrotic syndrome compared with prednisone treatment alone (23.9% versus 42.9%; RR = 0.56 and 95% CI = 0.33–0.93. The dose of Astragalus granules was 2.25 gram (equivalent to 15 gram crude Astragalus twice per day, at least for 3–6 months. The level of evidence quality was low, but we still recommended the evidence to the patient according to GRADEpro with the opinion of the expert. Followup showed the incidence of URTI in this child decreased significantly. Conclusions. Astragalus granules may reduce the incidence of URTI in children with nephrotic syndrome.

  12. Sphingosine-1-phosphate lyase mutations cause primary adrenal insufficiency and steroid-resistant nephrotic syndrome

    Science.gov (United States)

    Prasad, Rathi; Hadjidemetriou, Irene; Meimaridou, Eirini; Buonocore, Federica; Saleem, Moin; Hurcombe, Jenny; Bierzynska, Agnieszka; Barbagelata, Eliana; Bergadá, Ignacio; Cassinelli, Hamilton; Das, Urmi; Krone, Ruth; Hacihamdioglu, Bulent; Sari, Erkan; Yesilkaya, Ediz; Storr, Helen L.; Clemente, Maria; Fernandez-Cancio, Monica; Camats, Nuria; Ram, Nanik; Achermann, John C.; Van Veldhoven, Paul P.; Guasti, Leonardo; Braslavsky, Debora; Guran, Tulay; Metherell, Louise A.

    2017-01-01

    Primary adrenal insufficiency is life threatening and can present alone or in combination with other comorbidities. Here, we have described a primary adrenal insufficiency syndrome and steroid-resistant nephrotic syndrome caused by loss-of-function mutations in sphingosine-1-phosphate lyase (SGPL1). SGPL1 executes the final decisive step of the sphingolipid breakdown pathway, mediating the irreversible cleavage of the lipid-signaling molecule sphingosine-1-phosphate (S1P). Mutations in other upstream components of the pathway lead to harmful accumulation of lysosomal sphingolipid species, which are associated with a series of conditions known as the sphingolipidoses. In this work, we have identified 4 different homozygous mutations, c.665G>A (p.R222Q), c.1633_1635delTTC (p.F545del), c.261+1G>A (p.S65Rfs*6), and c.7dupA (p.S3Kfs*11), in 5 families with the condition. In total, 8 patients were investigated, some of whom also manifested other features, including ichthyosis, primary hypothyroidism, neurological symptoms, and cryptorchidism. Sgpl1–/– mice recapitulated the main characteristics of the human disease with abnormal adrenal and renal morphology. Sgpl1–/– mice displayed disrupted adrenocortical zonation and defective expression of steroidogenic enzymes as well as renal histology in keeping with a glomerular phenotype. In summary, we have identified SGPL1 mutations in humans that perhaps represent a distinct multisystemic disorder of sphingolipid metabolism. PMID:28165343

  13. A Prospective Observational Survey on the Long-Term Effect of LDL Apheresis on Drug-Resistant Nephrotic Syndrome

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    Eri Muso

    2015-08-01

    Full Text Available Background/Aims: LDL apheresis (LDL-A is used for drug-resistant nephrotic syndrome (NS as an alternative therapy to induce remission by improvement of hyperlipidemia. Several clinical studies have suggested the efficacy of LDL-A for refractory NS, but the level of evidence remains insufficient. A multicenter prospective study, POLARIS (Prospective Observational Survey on the Long-Term Effects of LDL Apheresis on Drug-Resistant Nephrotic Syndrome, was conducted to evaluate its clinical efficacy with high-level evidence. Methods: Patients with NS who showed resistance to primary medication for at least 4 weeks were prospectively recruited to the study and treated with LDL-A. The long-term outcome was evaluated based on the rate of remission of NS 2 years after treatment. Factors affecting the outcome were also examined. Results: A total of 58 refractory NS patients from 40 facilities were recruited and enrolled as subjects of the POLARIS study. Of the 44 subjects followed for 2 years, 21 (47.7% showed remission of NS based on a urinary protein (UP level Conclusions: Almost half of the cases of drug-resistant NS showed remission 2 years after LDL-A. Improvement of nephrotic parameters at termination of the LDL-A treatment was a predictor of a favorable outcome.

  14. Reduced 11beta-hydroxysteroid dehydrogenase activity in patients with the nephrotic syndrome.

    Science.gov (United States)

    Vogt, B; Dick, B; N'Gankam, V; Frey, F J; Frey, B M

    1999-02-01

    Patients with the nephrotic syndrome (NS) exhibit abnormal renal sodium retention which cannot completely explained by a secondary hyperaldosteronism due to reduced renal perfusion. As an alternative mechanism to explain this phenomenon we postulate a cortisol-mediated mineralocorticoid effect as a consequence of a reduced activity of 11beta-hydroxysteroid dehydrogenase (11beta-HSD). A down-regulation of 11beta-HSD, i.e. of the shuttle of active to inactive glucocorticosteroids, has been shown to cause mineralocorticoid effects. Therefore we investigated the activity of 11beta-HSD by measuring the urinary ratio of (tetrahydrocortisol + 5alpha-tetrahydrocortisol)/tetrahydrocortisone [(THF+5alpha-THF)/THE] by gas-chromatography in 29 NS patients with biopsy-proven glomerulonephritis and 29 healthy control subjects. The ratio of (THF+5alpha-THF)/THE was higher in NS patients (median 1.49, range 0.45-4.07) than in the control subjects (0.98, 0.60-1.36; pnew mechanism contributing to the exaggerated sodium retention in patients with the NS.

  15. Concerted Action of ANP and Dopamine D1-Receptor to Regulate Sodium Homeostasis in Nephrotic Syndrome

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    Cátia Fernandes-Cerqueira

    2013-01-01

    Full Text Available The edema formation in nephrotic syndrome (NS is associated with a blunted response to atrial natriuretic peptide (ANP. The natriuretic effects of ANP have been related to renal dopamine D1-receptors (D1R. We examined the interaction between ANP and renal D1R in rats with puromycin aminonucleoside-induced NS (PAN-NS. Urinary sodium, cyclic guanosine monophosphate (cGMP excretion, and D1R protein expression and localization in renal tubules were evaluated in PAN-NS and control rats before and during volume expansion (VE. The effects of zaprinast (phosphodiesterase type 5 inhibitor, alone or in combination with Sch-23390 (D1R antagonist, were examined in both groups. The increased natriuresis and urinary cGMP excretion evoked by acute VE were blunted in PAN-NS despite increased levels of circulating ANP. This was accompanied in PAN-NS by a marked decrease of D1R expression in the renal tubules. Infusion of zaprinast in PAN-NS resulted in increased urinary excretion of cGMP and sodium to similar levels of control rats and increased expression of D1R in the plasma membrane of renal tubular cells. Combined administration of Sch-23390 and zaprinast prevented natriuresis and increased cGMP excretion induced by zaprinast alone. We conclude that D1R may play a major role in the ANP resistance observed in PAN-NS.

  16. Successful treatment of nephrotic syndrome due to FMF amyloidosis with azathioprine: report of three Turkish cases.

    Science.gov (United States)

    Sayarlioglu, Hayriye; Erkoc, Reha; Sayarlioglu, Mehmet; Dogan, Ekrem; Soyoral, Yasemin

    2006-12-01

    Secondary amyloidosis is a well-known complication of certain familial Mediterranean fever (FMF). We presented three Turkish patients with FMF and biopsy proven amyloidosis. These patients were treated with colchicine 1.5 mg/day. They have experienced five to six peritonitis attacks with fever within 1 year. On admission, the laboratory test results were as follows: serum creatinine 2.3, 0.6, and 0.5 mg/dl; albumin 4.2, 1.9, and 1.8 g/dl; and urinary protein excretion 4, 15, and 10 g/day, respectively. All the patients were started azathioprine (AZA) 100 mg/day and attacks were completely stopped. Laboratory findings were as follows after 1 year of AZA treatment: serum creatinine 1, 0.8, and 0.6 mg/dl; albumin 4.3, 3, and 3.5 g/dl; and urinary protein excretion 3, 8, and 1.5 g/day, respectively. Treatment with azathioprine in addition to colchicine could ameliorate the nephrotic syndrome and control the attacks very effectively in these cases.

  17. [Increased lipoprotein(a) in a paediatric patient associated with nephrotic syndrome].

    Science.gov (United States)

    Menéndez Valladares, Paloma; Arrobas Velilla, Teresa; Bermúdez de la Vega, José Antonio; Romero Pérez, María Del Mar; Fabiani Romero, Fernando; González Rodríguez, Concepción

    A common complication in paediatric patients with nephrotic syndrome (NS) is hyperlipidaemia. About 20% of children do not respond to treatment with corticosteroids, presenting with a cortico-resistant NS (CRNS), which can progress to kidney failure. It has been observed that paediatric patients with CRNS have an elevated low density lipoprotein cholesterol (LDL-c), very low density lipoprotein cholesterol (VLDL-c), and triglycerides levels, as well as elevated Lipoprotein-a [Lp (a)] levels. The case is presented of a 5 year old boy, diagnosed with CRNS, presenting with dyslipidaemia with increased LDL-c, Apo-B100, and Lp(a) levels. After the poor prognosis of the renal function, immunosuppressant treatment was started with tacrolimus and atorvastatin to control dyslipidaemia. Although tacrolimus causes an elevation of total cholesterol and LDL-c, the significant alterations of the children lipid profile suggest the existence of a high cardiovascular risk. In these cases, it would be interesting to have reference values in children in our health area. Copyright © 2016 Sociedad Española de Arteriosclerosis. Publicado por Elsevier España, S.L.U. All rights reserved.

  18. Drug-induced interstitial nephritis in a child with idiopathic nephrotic syndrome

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    Printza Nikoleta

    2009-01-01

    Full Text Available Acute renal failure (ARF is a rare but severe complication of active idiopathic nephrotic syndrome (INS in children. It may be due to several causes with different outcomes. Both the clinical picture of the patient as well as laboratory, imaging and histopathological findings may help in the diagnosis. We present a case of drug-induced acute interstitial nephritis (AIN, complicated with ARF, in a 2½ -year-old girl with active INS. The child was referred to the Hippokration General Hospital, Thessaloniki, Greece hospital with steroid-resistant NS; renal biopsy was performed, which did not show any remarkable findings and cyclosporine was admi-nistered in addition to steroid therapy. The first day after biopsy, the child developed gross hematuria and abdominal pain and an antibiotic was added to her treatment. In the following days, fever, vomiting, hypertension and ARF occurred. Ultrasound study revealed enlarged kidneys with increased echogenity and loss of corticomedullary differentiation. The antibiotic and cyclos-porine were stopped and the child was managed with furosemide, nifedipine and steroids. A second renal biopsy was performed, which confirmed the diagnosis of acute interstitial nephritis. The child did not require dialysis therapy. Her urine output improved gradually and the serum creatinine normalized one month after the initial episode. Our case re-emphasizes the need for investigation of factors precipitating ARF in children with idiopathic NS.

  19. Nephrotic syndrome after allogeneic hematopoietic stem cell transplantation: etiology and pathogenesis.

    Science.gov (United States)

    Luo, Xiao-dan; Liu, Qi-fa; Zhang, Yu; Sun, Jing; Wang, Guo-bao; Fan, Zhi-ping; Yi, Zheng-shan; Ling, Yi-wen; Wei, Yong-qiang; Liu, Xiao-li; Xu, Bing

    2011-02-15

    In this study we investigated the etiology and pathogenesis of nephrotic syndrome (NS) after allogeneic hematopoietic stem cell transplantation (allo-HSCT) in 257 patients with hematopoietic malignancies who survived more than 2 months post allo-HSCT. Associations of NS with the conditioning regimen, graft versus host disease (GVHD), and other variables were analyzed. Pathologic features of the kidney, regulatory T cells (Tregs), interferon-γ (IFN-γ), and tumor necrosis factor-α (TNF-α) were studied. NS was identified in 9 patients. The number of Tregs at day+30, 60, 90, and 180 was lower in NS patients than non-NS patients (P=0.001, 0.001, 0.007, 0.003). Serum levels of IFN-γ and TNF-α were higher in NS patients (P=0.032, 0.001, respectively). NS post allo-HSCT was associated with the occurrence of chronic GVHD (P=0.02). NS post-HSCT is an immune disorder that may involve immune complex deposition, Th1 cytokines, and Tregs.

  20. [A case of cryptococcal meningitis with nephrotic syndrome and renal insufficiency under immunosuppressive therapy].

    Science.gov (United States)

    Tsuchida, Hiroki; Ichikawa, Daisuke; Shima, Yoshinori; Yasuda, Takashi; Sato, Takeo; Kimura, Kenjiro

    2007-01-01

    A 76 year-old woman was admitted to our hospital because of pyrexia and fatigue. One year earlier, she was diagnosed as nephrotic syndrome(NS) caused by focal segmental glomerulosclerosis and immunosuppressive therapy was started with marked amelioration of proteinuria. Thereafter, her renal function worsened, but only supportive treatment was continued. After admission, a cerebrospinal fluid (CSF) examination revealed Cryptococcus neoformans (C. neoformans) by india ink staining and a subsequent CSF culture confirmed C. neoformans infection. Accordingly, we made the diagnosis of cryptococcal meningitis and immediately started multiple anti fungal drugs with dosage modification according to her impaired renal function. Immunosuppressive therapy for NS was temporarily terminated. The inflammatory signs and symptoms soon were markedly improved, but the anti cryptococcal antibody titer in the serum and CSF remained high. Immunosuppressive therapy was started again at a low dosage because urinary protein had increased again. One hundred and eight days from admission, she was discharged with a regimen of multiple anti fungal drugs. Proteinuria and renal insufficiency was almost stable during hospitalization. Most fungal infection develops in patients in an immunosuppressive state induced by immunosuppressive drugs, HIV infection and so on. Patients with NS are frequently in an immunosuppressive state because of urinary loss of immunoglobulins and the use of immunosuppressive drugs. Therefore, it should be remembered that patients with NS are at a high risk of suffering from fungal infection.

  1. Polimiosite associada à síndrome nefrótica Polymyositis associated with nephrotic syndrome

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    Renato Oliveira Freire

    2010-08-01

    Full Text Available A polimiosite (PM é uma doença sistêmica do grupo das miopatias inflamatórias idiopáticas, clinicamente caracterizada por fraqueza muscular proximal e simétrica. Há relatos na literatura de PM associada a neoplasias, doenças autoimunes e infecções virais. Entretanto, a associação entre PM e nefropatia não é frequente. Descrevemos o caso de um paciente com polimiosite que evoluiu com síndrome nefrótica devido à presença de glomerulonefrite mesangialPolymyositis (PM is a systemic disease of the idiopathic inflammatory myopathy group, clinically characterized by symmetric and proximal muscle weakness. There are reports in literature of PM associated with malignancies, autoimmune diseases, and viral infections. However, the association between PM and nephropathy is not common. We describe a case report of a patient with polymyositis who developed nephrotic syndrome due to mesangial glomerulonephritis

  2. Relationship between Angiotensin-Converting Enzyme Insertion/Deletion Gene Polymorphism and Susceptibility of Minimal Change Nephrotic Syndrome: A Meta-Analysis

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    Tian-Biao Zhou

    2011-01-01

    Africans: D: =.81, DD: =.49. Furthermore, the II genotype seemed not to play a protective role against MCNS risk for Asians, Caucasians and Africans (=.12, =.09, =.76, resp.. Interestingly, there was also significant association between ACE I/D gene polymorphism and MCNS susceptibility in overall populations (D: =.007, DD: =.04, II: =.03. Conclusion. D allele or DD genotype might be a significant genetic molecular marker for MCNS susceptibility in Asians and overall populations, but not for Caucasians and Africans. More larger and rigorous genetic epidemiological investigations are required to further explore this association.

  3. Economic evaluation of human albumin use in patients with nephrotic syndrome in four Brazilian public hospitals: pharmacoeconomic study.

    Science.gov (United States)

    Toledo, Leonardo Augusto Kister de; Noblat, Antônio Carlos Beisl; Nascimento, Harrison Floriano do; Noblat, Lúcia de Araújo Costa Beisl

    2017-01-01

    In 2004, the Brazilian National Health Surveillance Agency (Agência Nacional de Vigilância Sanitária, ANVISA) published a resolution establishing guidelines for albumin use. Although the published data do not indicate any definitive conclusions about the benefits of albumin use in patients with nephrotic syndrome (NS), the guidelines recommend this procedure only in cases of edema that is refractory to use of diuretics. The aim here was to analyze albumin use among patients with nephrotic syndrome. Pharmacoeconomic study conducted in four large public referral hospitals for nephrology services in northeastern Brazil. Cost-effectiveness and cost-utility economic evaluations were performed on a concurrent cohort of patients with nephrotic syndrome, who were divided into two groups according to compliance or noncompliance with the guidelines. Quality-of-life data were obtained from the SF36 and CHQ-PF50 questionnaires. This study enrolled 109 patients (60% adults and 56% women); 41.3% were using albumin in accordance with the guidelines. The weight, diuresis and fluid balance parameters were more cost-effective for patients who adhered to the guidelines. Regarding days of hospitalization avoided, the incremental ratio showed a daily cost of R$ 55.33, and guideline-compliant patients were hospitalized for five days or fewer. The quality of life improved by 8%, and savings of R$ 3,458.13/QALY (quality-adjusted life year) for the healthcare system were generated through guideline compliance. The economic analyses of this study demonstrated that there were greater cost benefits for patients whose treatment followed the guidelines.

  4. 儿童难治性肾病综合征的治疗进展%Progress in treatment of refractory nephrotic syndrome

    Institute of Scientific and Technical Information of China (English)

    许云鹏; 郭艳芳

    2014-01-01

    难治性肾病综合征(RNS)是临床上原发型肾病综合征(PNS)中频复发型(FRNS)、激素依赖型(SDNS)和激素耐药型(SRNS)肾病综合征的总称。按其对激素治疗是否敏感及尿蛋白转阴情况,可将原发性肾病综合征分为频复发型、激素依赖型和激素耐药型。临床观察发现80%~90%的PNS患儿经初始激素治疗即可获完全缓解,但有76%~93%的患儿复发,并超过半数的激素耐药型肾病儿童在10年内发展为终末期肾病(ESRD)。该文章就近年来关于儿童RNS临床治疗的方法作一综述。%Refractory nephrotic syndrome (RNS) is the generic terms of recurrence of intermediate frequency (frequently relapse nephrotic syndrome, FRNS), steroid dependence (steroid-dependent nephrotic syndrome, SDNS) and prednisone resistant type (steroid-resistant nephrotic syndrome, SRNS) in clinical nephrotic syndrome (primary nephrotic syndrome, PNS). According to the sensitivity to prednisone therapy and whether urinary protein is negative, recurrence of primary nephrotic syndrome can be divided into frequency, prednisone dependent and prednisone drug resistance. Clinical observation has found that 80%-90% of children with PNS after initial prednisone therapy can relieve completely, but have a relapse in 76%-93% in later days, and more than half of the prednisone resistant type kidney disease in children in ten years can progress end-stage renal disease (ESRD). The article summarized the clinical treatment of refractory nephrotic syndrome in children.

  5. Assessment of quality of life in children with nephrotic syndrome at a teaching hospital in South India

    OpenAIRE

    Sonia Agrawal; Sriram Krishnamurthy; Bijaya Nanda Naik

    2017-01-01

    This study was conducted to assess the quality of life (QOL) in children between 2 and 18 years of age with primary idiopathic nephrotic syndrome (NS) using Pediatric Quality of Life Inventory (PedsQL 4.0 Generic Core Scales). This cross-sectional comparative study was conducted at a tertiary care hospital in South India between December 2014 and February 2015. In this questionnaire-based study, 50 children with primary idiopathic NS and an equal number of age-matched controls with other chro...

  6. Lithium-induced minimal change disease and acute kidney injury

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    Parul Tandon

    2015-01-01

    Full Text Available Context: Lithium carbonate is a psychiatric medication commonly used in the treatment of bipolar disorder. It has been implicated in inducing nephrogenic diabetes inspidus, chronic tubulointerstitial nephropathy, and acute tubular necrosis. We describe a case of lithium-induced minimal change disease (MCD and acute kidney injury (AKI. Case Report: A 32-year-old female with a medical history of bipolar disorder treated with chronic lithium therapy presented with anasarca, fatigue, and tremors. Work-up revealed supra-therapeutic lithium levels, hypoalbuminemia, and significant proteinuria. The patient was treated conservatively with fluids and discontinuation of lithium therapy. Subsequently, she developed significant AKI and persistent proteinuria. She underwent a renal biopsy that demonstrated effacement of podocyte foot processes consistent with lithium-induced MCD. This was treated with corticosteroids, which decreased the proteinuria and resolved all the patient′s symptoms. Conclusion: Lithium-induced MCD is a rare disease that affects patients of all ages. It is often associated with therapeutic lithium and is typically resolved with discontinuation of lithium. In some cases, concurrent AKI may result due to vascular obstruction from hyperalbuminuria and associated renal interstitial edema. Corticosteroids may be needed to reduce the proteinuria and prevent progression to chronic kidney disease. As such, patients on lithium therapy may benefit from monitoring of glomerular function via urinalysis to prevent the onset of nephrotic syndrome.

  7. The risk of urinary tract infection in children with nephrotic syndrome

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    Patricia Y. Gunawan

    2016-08-01

    Full Text Available Background Urinary tract infections (UTI may affect any part of the urinary system: the kidneys, ureters, bladder, or urethra. Nephrotic syndrome (NS is the most common glomerular disorder in childhood, comprising a group of symptoms that include proteinuria, hypoalbuminemia, hypercholesterolemia, and edema. The prevalence of UTI in NS patients is high, around 25-66.7%. The increased prevalence of UTI in NS is due to immunoglobulin loss, defective T cell function, the presence of ascites, and relative malnutrition. Objective To study the risk of UTI in children with NS. Methods We performed a retrospective study of NS and UTI patients from January 2004 to December 2013 in the Division of Nephrology at Prof. Dr. R.D. Kandou Hospital, Manado. Data was collected from medical records. Diagnosis of UTI was made based on urine culture results. Diagnosis of NS was made based on the group of symptoms mentioned above. Analysis was done using Chi-square test with SPSS version 22 software. Results Of 74 NS patients, 34 (46% had UTIs. During the same study period, 117 patients had UTIs. NS was more common in boys (64.9%, while NS with UTI was more common in girls (67.6%. The most common organisms causing UTI in NS patients were Eschericia coli and Citrobacter diversus (23% each. Imipenem and amikacin were most commonly used antibiotics to which the bacteria were sensitive. Increased risk of UTI was significant in children with NS (OR 1.8; P=0.03. Conclusion Children with NS are at significantly increased risk of UTIs.

  8. Treatment of Severe Edema in Children with Nephrotic Syndrome with Diuretics Alone — A Prospective Study

    Science.gov (United States)

    Kapur, Gaurav; Valentini, Rudolph P.; Imam, Abubakr A.; Mattoo, Tej K.

    2009-01-01

    Background and objective: Severe edema in children with nephrotic syndrome (NS) may be associated with volume contraction (VC) or volume expansion (VE). Usually, severe edema in children is treated with intravenous (IV) albumin and diuretics, which is appropriate for VC patients. However, in VE patients, this can precipitate fluid overload. The objective of this study was to evaluate treatment of severe edema in NS with diuretics alone. Design, setting, participants, & measurements: Thirty NS patients with severe edema were enrolled in this prospective study in two phases. VC was diagnosed based on fractional excretion of sodium (FeNa) <1%. VC patients received IV albumin and furosemide. VE patients received IV furosemide and oral spironolactone. On the basis of phase 1 observations, FeNa <0.2% identified VC in 20 phase 2 patients. Results: All phase 1 patients had FeNa <1%. Phase 1 patients when reanalyzed based on a FeNa cutoff of 0.2%; it was noted that VC patients had higher BUN, BUN/creatinine ratio, urine osmolality, and lower FeNa and urine sodium compared with VE patients. Similar results were observed in phase 2. VC patients had significantly higher renin, aldosterone, and antidiuretic hormone levels. In phase 2, 11 VE patients received diuretics alone and 9 VC patients received albumin and furosemide. There was no difference in hospital stay and weight loss in VC and VE groups after treatment. Conclusions: FeNa is useful in distinguishing VC versus VE in NS children with severe edema. The use of diuretics alone in VE patients is safe and effective. PMID:19406963

  9. Relationship between ionized calcium and serum albumin level in children with idiopathic nephrotic syndrome

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    Viiola Irene Winata

    2016-10-01

    Full Text Available Background Nephrotic syndrome (NS patients frequently have abnormalities in calcium metabolism that manifest as hypocalcemia and reduced intestinal absorption of calcium. Hypocalcemia is initially attributed to hypoalbuminemia but it may also relate to a low level of ionized calcium. The ionized calcium level depends on the severity and duration of proteinuria. Objective To assess the rel ationship between ionized calcium and serum albumin level in idiopathic NS children. Methods An analytical study with cross-sectional design was applied to NS and healthy children between 1-14 years old in the Child Health Department of Hasan Sadikin Hospital, Bandung from December 2009 to April 2010. Ionized calcium was examined by Ca2 + analyzer AVL 980 with ion-selective electrodes (ISE methods. Results A total of34 subjects were recruited, consist of 17 NS and 17 healthy children. The mean ionized calcium and serum albumin level in NS children was 4.56 (SD 0.23 mg/dLand 1.45 (SD 0.24 g/dL, respectively. Statistical difference between ionized calcium level in NS and in healthy children was significant (P<0.05. Pearson correlation test between ionized calcium and serum albumin was significant (P<0.05 with correlation coefficient (r 0.53. We found the following equation to estimate ionized calcium (y based on the serum albumin level (x: y=3.84+0.49x. Conclusion There is a moderately positive linear relationship between ionized calcium and serum albumin level in NS children.

  10. An unexpected presentation: minimal change disease in an adult with treatment-naïve hepatitis C.

    Science.gov (United States)

    Stokes, Audrey L; Alhamad, Tarek; Abendroth, Catherine S; Farag, Hosam A; Verma, Navin

    2013-12-01

    Minimal change disease is the most common glomerular disease affecting children; its prevalence among adults, however, is eclipsed by other glomerular pathologies. Each of these diseases has a number of classic associations, such as membranoproliferative glomerulonephritis with hepatitis C. We report the case of a middle-aged African-American male who presented with the nephrotic syndrome and acute renal failure and was concomitantly diagnosed with a new hepatitis C infection. He also had a history of urethral strictures with potential reflux nephropathy, which--in combination with his African-American race--also made focal segmental glomerulosclerosis a diagnostic possibility. Full laboratory evaluation did not distinguish the cause of his massive proteinuria; subsequent renal biopsy ultimately revealed minimal change disease. A full course of high-dose steroids eventually reduced his proteinuria, after which his renal failure resolved as well without need for hemodialysis.

  11. Minimal Change Disease as a Secondary and Reversible Event of a Renal Transplant Case with Systemic Lupus Erythematosus.

    Science.gov (United States)

    Gkrouzman, Elena; Kirou, Kyriakos A; Seshan, Surya V; Chevalier, James M

    2015-01-01

    Secondary causes of minimal change disease (MCD) account for a minority of cases compared to its primary or idiopathic form and provide ground for consideration of common mechanisms of pathogenesis. In this paper we report a case of a 27-year-old Latina woman, a renal transplant recipient with systemic lupus erythematosus (SLE), who developed nephrotic range proteinuria 6 months after transplantation. The patient had recurrent acute renal failure and multiple biopsies were consistent with MCD. However, she lacked any other features of the typical nephrotic syndrome. An angiogram revealed a right external iliac vein stenosis in the region of renal vein anastomosis, which when restored resulted in normalization of creatinine and relief from proteinuria. We report a rare case of MCD developing secondary to iliac vein stenosis in a renal transplant recipient with SLE. Additionally we suggest that, in the event of biopsy-proven MCD presenting as an atypical nephrotic syndrome, alternative or secondary, potentially reversible, causes should be considered and explored.

  12. Minimal Change Disease as a Secondary and Reversible Event of a Renal Transplant Case with Systemic Lupus Erythematosus

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    Elena Gkrouzman

    2015-01-01

    Full Text Available Secondary causes of minimal change disease (MCD account for a minority of cases compared to its primary or idiopathic form and provide ground for consideration of common mechanisms of pathogenesis. In this paper we report a case of a 27-year-old Latina woman, a renal transplant recipient with systemic lupus erythematosus (SLE, who developed nephrotic range proteinuria 6 months after transplantation. The patient had recurrent acute renal failure and multiple biopsies were consistent with MCD. However, she lacked any other features of the typical nephrotic syndrome. An angiogram revealed a right external iliac vein stenosis in the region of renal vein anastomosis, which when restored resulted in normalization of creatinine and relief from proteinuria. We report a rare case of MCD developing secondary to iliac vein stenosis in a renal transplant recipient with SLE. Additionally we suggest that, in the event of biopsy-proven MCD presenting as an atypical nephrotic syndrome, alternative or secondary, potentially reversible, causes should be considered and explored.

  13. An unusual case of anti-glomerular basement membrane disease presenting with nephrotic syndrome.

    Science.gov (United States)

    Okafor, Chidi C; Balogun, Rasheed A; Bourne, David T; Alhussain, Turki O; Abdel-Rahman, E M

    2011-12-01

    Anti-glomerular basement membrane (anti-GBM) disease is a vasculitic disease characterized by acute kidney injury, oliguria, hematuria and proteinuria. Proteinuria is rarely in the nephrotic range. A case of anti-GBM disease with proteinuria of 22.5 g/day is discussed. Immunofluorescence showed strong linear IgG deposits while electron microscopy showed widespread visceral epithelial cell foot cell process effacement. No electron dense immune complex-type deposits were identified. Pathology findings were not suggestive of simultaneous presentation of anti-GBM disease and other diseases associated with nephrotic range proteinuria. Anti-GBM disease should be considered in a comprehensive differential diagnosis of severe proteinuria.

  14. ANCA-positive vasculitis induced by levamisole-adulterated cocaine and nephrotic syndrome: The kidney as an unusual target

    Science.gov (United States)

    Álvarez Díaz, Hortensia; Marińo Callejo, Ana Isabel; García Rodríguez, José Francisco; Rodríguez Pazos, Laura; Gómez Buela, Inmaculada; Bermejo Barrera, Ana María

    2013-01-01

    Patient: Male, 36 Final Diagnosis: Levamisole-induced vasculopathy Symptoms: Purpuric skin lesions Medication: Levamisole Clinical Procedure: — Specialty: Internal Medicine Objective: Unusual clinical course Background: Levamisole has been detected in seized cocaine samples and a levamisole-induced vasculopathy (LIV) has been described, mainly focused on skin. Case Report: A 36-year-old Caucasian man with history of antibodies to hepatitis C infection (negative hepatitis C virus RNA and negative HIV serology), smoking, and intravenous use of cocaine and brown heroin, presented to the hospital with purpuric skin lesions on extremities and earlobes. One month before the current presentation, a skin punch biopsy of one of these lesions was performed, showing histopathologic findings suggestive of mixed cryoglobulinemia. Laboratory testing revealed leukopenia, renal failure, and nephrotic syndrome. Antimyeloperoxidase antineutrophil cytoplasmic antibodies (MPO-ANCA) were positive. The previous skin punch biopsy was revised and demonstrated pathologic findings consistent with leukocytoclastic vasculitis. An analysis of a cocaine sample for personal use, provided by the patient, was performed using mass spectrometry-gas chromatography and levamisole was detected. Three boluses of intravenous methylprednisolone were administered, followed by oral prednisone 1 mg/Kg per day. Skin lesions and renal function improved. Conclusions: To our knowledge, this is the first report of nephrotic syndrome induced by levamisole-adulterated cocaine, proven by cocaine sample toxicology. Lack of renal biopsy is a limitation of this report. PMID:24478818

  15. [A Case of Advanced Transverse Colon Cancer with Nephrotic Syndrome Treated with Curative Resection and Complete Adjuvant Chemotherapy].

    Science.gov (United States)

    Sato, Nobutaka; Fuyuno, Seiya; Hatada, Teppei; Furuhashi, Takashi; Abe, Toshihiko

    2017-05-01

    A 74-year-old woman was diagnosed as having transverse colon cancer after diagnosis of nephrotic syndrome caused by membranous nephropathy. Although she had hypoproteinemia and hypoalbuminemia, we judged that she had no major nutritional problem. In previous, similar case reports, the use of human serum albumin and fresh-frozen plasma was suggested to be important to avoid complications in the perioperative period. Thus, we used the same in our patient in the perioperative period. In addition, we paid special attention to perioperative nutrition management and used total parenteral nutrition in perioperative period. We performed laparoscopic assisted right hemicolectomy. On the 15th day after the surgical resection, the patient was discharged without any problems. We considered that postoperative adjuvant chemotherapy with XELOX (CapeOX)should be performed because the TNM pathological stage was pStage III b. Regarding adjuvant chemotherapy for gastrointestinal cancer with nephrotic syndrome, no previous reports detailed the indications for postoperative adjuvant chemotherapy. Upon introduction of adjuvant chemotherapy, we determined adaptation in accordance with the general adaptation criteria. While observing the patient's progress with a nephrologist, we safely completed the scheduled 8 courses adjuvant chemotherapy.

  16. Refining the Diagnosis of Congenital Nephrotic Syndrome on Long-term Stored Tissue: c.1097G>A (p.(Arg366His)) WT1 Mutation Causing Denys Drash Syndrome

    NARCIS (Netherlands)

    Hillen, L.M.; Kamsteeg, E.J.; Schoots, J.; Tiebosch, A.T.; Speel, E.J.; Roemen, G.M.; Peutz-Koostra, C.J.; Stumpel, C.T.

    2016-01-01

    Congenital nephrotic syndrome (CNS) caused by a mutation in the Wilms tumor 1 suppressor gene (WT1) is part of Denys Drash Syndrome or Frasier syndrome. In the framework of genetic counseling, the diagnosis of CNS can be refined with gene mutation studies on long-term stored formalin-fixed paraffin-

  17. IgM nephropathy: is it closer to minimal change disease or to focal segmental glomerulosclerosis?

    Science.gov (United States)

    Brugnano, R; Del Sordo, R; Covarelli, C; Gnappi, E; Pasquali, S

    2016-08-01

    Immunoglobulin (Ig)M nephropathy (IgMN), known since 1978, is a very controversial clinicopathological entity characterized by IgM diffuse deposits in the mesangium at immunofluorescence whereas light microscop identifies minimal glomerular lesion, hypercellularity and expansion of the mesangium or sclerotic focal, segmental lesion. Clinically, it is a nephrotic syndrome, especially in pediatric patients, or asymptomatic proteinuria and/or isolated hematuria. These characteristics narrowly define IgMN between minimal change disease and focal segmental glomerulosclerosis, so it is not often recognized as a separate pathology. Homogeneous epidemiologic, pathogenetic, clinical or histological data are not available. Recent research on the pathogenetic role of mesangial IgM has, however, renewed interest in IgMN and naturally the controversies.

  18. Minimal change disease associated with type 1 and type 2 diabetes mellitus.

    Science.gov (United States)

    Moyses Neto, Miguel; Silva, Gyl Eanes Barros; Costa, Roberto S; Romão, Elen A; Vieira Neto, Osvaldo Merege; Dantas, Marcio

    2012-07-01

    A 19-year-old female with type 1 diabetes for four years, and a 73-year-old female with type 2 diabetes for twenty years developed sudden-onset nephrotic syndrome. Examination by light microscopy, immunofluorescence, and electron microscopy (in one case) identified minimal change disease (MCD) in both cases. There was a potential causative drug (meloxicam) for the 73-year-old patient. Both patients were treated with prednisone and responded with complete remission. The patient with type 1 diabetes showed complete remission without relapse, and the patient with type 2 diabetes had two relapses; complete remission was sustained after associated treatment with cyclophosphamide and prednisone. Both patients had two years of follow-up evaluation after remission. We discuss the outcomes of both patients and emphasize the role of kidney biopsy in diabetic patients with an atypical proteinuric clinical course, because patients with MCD clearly respond to corticotherapy alone or in conjunction with other immunosuppressive agents.

  19. A novel fibrillin-1 mutation in an egyptian marfan family: A proband showing nephrotic syndrome due to focal segmental glomerulosclerosis

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    Mohammad Al-Haggar

    2017-01-01

    Full Text Available Marfan syndrome (MFS, the founding member of connective tissue disorder, is an autosomal dominant disease; it is caused by a deficiency of the microfibrillar protein fibrillin-1 ( FBN1 and characterized by involvement of three main systems; skeletal, ocular, and cardiovascular. More than one thousand mutations in FBN1 gene on chromosome 15 were found to cause MFS. Nephrotic syndrome (NS had been described in very few patients with MFS being attributed to membranoproliferative glomerulonephritis secondary to infective endocarditis. Focal segmental glomerulosclerosis (FSGS had been reported in NS in conjunction with MFS without confirming the diagnosis by mutational analysis of FBN1. We hereby present an Egyptian family with MFS documented at the molecular level; it showed a male proband with NS secondary to FSGS, unfortunately, we failed to make any causal link between FBN dysfunction and FSGS. In this context, we review the spectrum of renal involvements occurring in MFS patients.

  20. Gene-level integrated metric of negative selection (GIMS prioritizes candidate genes for nephrotic syndrome.

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    Matthew G Sampson

    Full Text Available Nephrotic syndrome (NS gene discovery efforts are now occurring in small kindreds and cohorts of sporadic cases. Power to identify causal variants in these groups beyond a statistical significance threshold is challenging due to small sample size and/or lack of family information. There is a need to develop novel methods to identify NS-associated variants. One way to determine putative functional relevance of a gene is to measure its strength of negative selection, as variants in genes under strong negative selection are more likely to be deleterious. We created a gene-level, integrated metric of negative selection (GIMS score for 20,079 genes by combining multiple comparative genomics and population genetics measures. To understand the utility of GIMS for NS gene discovery, we examined this score in a diverse set of NS-relevant gene sets. These included genes known to cause monogenic forms of NS in humans as well as genes expressed in the cells of the glomerulus and, particularly, the podocyte. We found strong negative selection in the following NS-relevant gene sets: (1 autosomal-dominant Mendelian focal segmental glomerulosclerosis (FSGS genes (p = 0.03 compared to reference, (2 glomerular expressed genes (p = 4×10(-23, and (3 predicted podocyte genes (p = 3×10(-9. Eight genes causing autosomal dominant forms of FSGS had a stronger combined score of negative selection and podocyte enrichment as compared to all other genes (p = 1 x 10(-3. As a whole, recessive FSGS genes were not enriched for negative selection. Thus, we also created a transcript-level, integrated metric of negative selection (TIMS to quantify negative selection on an isoform level. These revealed transcripts of known autosomal recessive disease-causing genes that were nonetheless under strong selection. We suggest that a filtering strategy that includes measuring negative selection on a gene or isoform level could aid in identifying NS-related genes. Our GIMS and TIMS

  1. Gene-level integrated metric of negative selection (GIMS) prioritizes candidate genes for nephrotic syndrome.

    Science.gov (United States)

    Sampson, Matthew G; Gillies, Christopher E; Ju, Wenjun; Kretzler, Matthias; Kang, Hyun Min

    2013-01-01

    Nephrotic syndrome (NS) gene discovery efforts are now occurring in small kindreds and cohorts of sporadic cases. Power to identify causal variants in these groups beyond a statistical significance threshold is challenging due to small sample size and/or lack of family information. There is a need to develop novel methods to identify NS-associated variants. One way to determine putative functional relevance of a gene is to measure its strength of negative selection, as variants in genes under strong negative selection are more likely to be deleterious. We created a gene-level, integrated metric of negative selection (GIMS) score for 20,079 genes by combining multiple comparative genomics and population genetics measures. To understand the utility of GIMS for NS gene discovery, we examined this score in a diverse set of NS-relevant gene sets. These included genes known to cause monogenic forms of NS in humans as well as genes expressed in the cells of the glomerulus and, particularly, the podocyte. We found strong negative selection in the following NS-relevant gene sets: (1) autosomal-dominant Mendelian focal segmental glomerulosclerosis (FSGS) genes (p = 0.03 compared to reference), (2) glomerular expressed genes (p = 4×10(-23)), and (3) predicted podocyte genes (p = 3×10(-9)). Eight genes causing autosomal dominant forms of FSGS had a stronger combined score of negative selection and podocyte enrichment as compared to all other genes (p = 1 x 10(-3)). As a whole, recessive FSGS genes were not enriched for negative selection. Thus, we also created a transcript-level, integrated metric of negative selection (TIMS) to quantify negative selection on an isoform level. These revealed transcripts of known autosomal recessive disease-causing genes that were nonetheless under strong selection. We suggest that a filtering strategy that includes measuring negative selection on a gene or isoform level could aid in identifying NS-related genes. Our GIMS and TIMS scores are

  2. An intriguing association of Turner syndrome with severe nephrotic syndrome: searching for a diagnosis.

    Science.gov (United States)

    Minzala, G; Ismail, G

    2016-10-01

    Systemic lupus erythematosus (SLE) is a chronic disease caused by an aberrant autoimmune response, with a large spectrum of clinical manifestations. It strikingly affects women. Recent papers reveal that the men with Klinefelter syndrome (47, XXY) have a higher incidence of lupus than the men in the general population, similar with that of genotypic females. On the other hand, there is a great lack of information regarding the association of SLE with Turner syndrome, but it seems to be a lower risk for females with Turner to develop SLE. We present a rare association of a Turner syndrome with SLE, with negative immunology for SLE and with diagnosis made on renal biopsy. These data suggest that the presence of two X chromosomes may predispose to SLE, the ligand (CD40 ligand) for one of the genes that contributes to the pathogenesis of SLE being located on the X chromosome.

  3. Genomic and clinical profiling of a national nephrotic syndrome cohort advocates a precision medicine approach to disease management.

    Science.gov (United States)

    Bierzynska, Agnieszka; McCarthy, Hugh J; Soderquest, Katrina; Sen, Ethan S; Colby, Elizabeth; Ding, Wen Y; Nabhan, Marwa M; Kerecuk, Larissa; Hegde, Shivram; Hughes, David; Marks, Stephen; Feather, Sally; Jones, Caroline; Webb, Nicholas J A; Ognjanovic, Milos; Christian, Martin; Gilbert, Rodney D; Sinha, Manish D; Lord, Graham M; Simpson, Michael; Koziell, Ania B; Welsh, Gavin I; Saleem, Moin A

    2017-04-01

    Steroid Resistant Nephrotic Syndrome (SRNS) in children and young adults has differing etiologies with monogenic disease accounting for 2.9-30% in selected series. Using whole exome sequencing we sought to stratify a national population of children with SRNS into monogenic and non-monogenic forms, and further define those groups by detailed phenotypic analysis. Pediatric patients with SRNS were identified via a national United Kingdom Renal Registry. Whole exome sequencing was performed on 187 patients, of which 12% have a positive family history with a focus on the 53 genes currently known to be associated with nephrotic syndrome. Genetic findings were correlated with individual case disease characteristics. Disease causing variants were detected in 26.2% of patients. Most often this occurred in the three most common SRNS-associated genes: NPHS1, NPHS2, and WT1 but also in 14 other genes. The genotype did not always correlate with expected phenotype since mutations in OCRL, COL4A3, and DGKE associated with specific syndromes were detected in patients with isolated renal disease. Analysis by primary/presumed compared with secondary steroid resistance found 30.8% monogenic disease in primary compared with none in secondary SRNS permitting further mechanistic stratification. Genetic SRNS progressed faster to end stage renal failure, with no documented disease recurrence post-transplantation within this cohort. Primary steroid resistance in which no gene mutation was identified had a 47.8% risk of recurrence. In this unbiased pediatric population, whole exome sequencing allowed screening of all current candidate genes. Thus, deep phenotyping combined with whole exome sequencing is an effective tool for early identification of SRNS etiology, yielding an evidence-based algorithm for clinical management.

  4. Detection of activated parietal epithelial cells on the glomerular tuft distinguishes early focal segmental glomerulosclerosis from minimal change disease.

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    Smeets, Bart; Stucker, Fabien; Wetzels, Jack; Brocheriou, Isabelle; Ronco, Pierre; Gröne, Hermann-Josef; D'Agati, Vivette; Fogo, Agnes B; van Kuppevelt, Toin H; Fischer, Hans-Peter; Boor, Peter; Floege, Jürgen; Ostendorf, Tammo; Moeller, Marcus J

    2014-12-01

    In rodents, parietal epithelial cells (PECs) migrating onto the glomerular tuft participate in the formation of focal segmental glomerulosclerosis (FSGS) lesions. We investigated whether immunohistologic detection of PEC markers in the initial biopsies of human patients with first manifestation of idiopathic nephrotic syndrome with no immune complexes can improve the sensitivity to detect sclerotic lesions compared with standard methods. Ninety-five renal biopsies were stained for claudin-1 (PEC marker), CD44 (activated PECs), and LKIV69 (PEC matrix); 38 had been diagnosed as early primary FSGS and 57 as minimal change disease. PEC markers were detected on the tuft in 87% of the biopsies of patients diagnosed as primary FSGS. PEC markers were detected in FSGS lesions from the earliest stages of disease. In minimal change disease, no PEC activation was observed by immunohistology. However, in 25% of biopsies originally diagnosed as minimal change disease the presence of small lesions indicative of a sclerosing process were detected, which were undetectable on standard periodic acid-Schiff staining, even though only a single histologic section for each PEC marker was evaluated. Staining for LKIV69 detected lesions with the highest sensitivity. Two novel PEC markers A-kinase anchor protein 12 and annexin A3 exhibited similar sensitivity. In summary, detection of PECs on the glomerular tuft by immunostaining improves the differentiation between minimal change disease and primary FSGS and may serve to guide clinical decision making.

  5. De novo minimal change disease after ABO-incompatible kidney transplantation.

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    Mochizuki, Yasushi; Iwata, Takahisa; Nishikido, Masaharu; Uramatsu, Tadashi; Sakai, Hideki; Taguchi, Takashi

    2012-07-01

    We report the clinical and pathological findings of a case of de novo minimal change disease (MCD) after ABO-incompatible living kidney transplantation. A 62-yr-old man with end-stage renal disease associated with type I diabetes received ABO-incompatible kidney transplantation from his 58-yr-old wife. Although allograft function was excellent immediately after surgery, massive proteinuria (35 g/d) appeared on post-transplantation day 5. After the allograft biopsy taken on post-transplantation day 6, he was treated with 12 cycles of plasma exchange, but the nephrotic-range proteinuria showed no remission. The biopsy specimen showed no significant pathological findings on light microscopy, but electron microscopy showed diffuse effacement of podocyte foot processes. Based on the diagnosis of de novo MCD, the patient received intravenous methylprednisolone pulse therapy, followed by high-dose steroid maintenance therapy. The steroid therapy induced complete remission of nephrotic syndrome and stable allograft function immediately, which was also maintained at one yr after the transplantation.

  6. Therapeutic Dilemmas Regarding Anticoagulation: An Experience in a Patient with Nephrotic Syndrome, Pulmonary Embolism, and Traumatic Brain Injury

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    Akimoto, Tetsu; Yamazaki, Tomoyuki; Kusano, Eiji; Nagata, Daisuke

    2016-01-01

    Patients with active bleeding complications who concomitantly develop overt pulmonary embolism (PE) present distinct therapeutic dilemmas, since they are perceived to be at substantial risk for the progression of the embolism in the absence of treatment and for aggravation of the hemorrhagic lesions if treated with anticoagulants. A 76-year-old patient with nephrotic syndrome, which is associated with an increased risk of thromboembolism, concurrently developed acute PE and intracranial bleeding because of traumatic brain injury. In this case, we prioritized the treatment for PE with the intravenous unfractionated heparin followed by warfarinization. Despite the transient hemorrhagic progression of the brain contusion after the institution of anticoagulation, our patient recovered favorably from the disease without any signs of neurological compromise. Several conundrums regarding anticoagulation that emerged in this case are also discussed. PMID:27840582

  7. Creatinine clearance, urinary excretion of glomerular basement membrane antigens and renal histology in congenital nephrotic syndrome of Finnish type.

    Science.gov (United States)

    Huttunen, N P

    1977-04-01

    The endogenous creatinine clearance and urinary excretion rate of glomerular basement membrane (GBM) antigens were followed from 2 to 19 months in fifteen patients with congenital nephrotic syndrome (CNF). The quantitative examination of renal morphology was made on fourteen of these patients. Creatinine clearance increased during the first few months of life and thereafter gradually decreased. The urinary excretion rate of GBM antigens rose during the course of the disease. The creatinine clearance did not correlate significantly with glomerular fibrosis but it did correlate with tubular atrophy and interstitial fibrosis. The urinary excretion of GBM antigens correlated significantly with glomerular and interstitial fibrosis and with tubular atrophy. It is concluded that there is a clear progress in the disease and the renal histological changes probably are caused by accumulation of GBM material in glomeruli.

  8. Canadian Society of Nephrology Commentary on the 2012 KDIGO clinical practice guideline for glomerulonephritis: management of nephrotic syndrome in children.

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    Samuel, Susan; Bitzan, Martin; Zappitelli, Michael; Dart, Allison; Mammen, Cherry; Pinsk, Maury; Cybulsky, Andrey V; Walsh, Michael; Knoll, Greg; Hladunewich, Michelle; Bargman, Joanne; Reich, Heather; Humar, Atul; Muirhead, Norman

    2014-03-01

    The KDIGO (Kidney Disease: Improving Global Outcomes) clinical practice guideline for management of glomerulonephritis was recently released. The Canadian Society of Nephrology convened a working group to review the recommendations and comment on their relevancy and applicability to the Canadian context. A subgroup of pediatric nephrologists reviewed the guideline statements for management of childhood nephrotic syndrome and agreed with most of the guideline statements developed by KDIGO. This commentary highlights areas in which there is lack of evidence and areas in need of translation of evidence into clinical practice. Areas of controversy or uncertainty, including the length of corticosteroid therapy for the initial presentation and relapses, definitions of steroid resistance, and choice of second-line agents, are discussed in more detail. Existing practice variation is also addressed.

  9. Spironolactone Plus Full-Dose ACE Inhibition in Patients with Idiopathic Membranous Nephropathy and Nephrotic Syndrome: Does It Really Work?

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    Giuseppe Remuzzi

    2010-01-01

    Full Text Available We have studied the effects of add-on spironolactone treatment (100 mg/day in 11 patients with idiopathic membranous nephropathy (IMN and > 3 gm proteinuria/day despite angiotensin converting enzyme (ACE inhibitor therapy titrated to a systolic/diastolic blood pressure < 120/80 mmHg. Blood pressure, 24-hour urinary protein excretion, and creatinine clearance were measured prior to, after two months of combined therapy, and after a 2-month withdrawal period of spironolactone. While systolic and diastolic blood pressure decreased significantly after spironolactone therapy, proteinuria did not improve. Serum potassium increased significantly as well, with three patients requiring resin-binding therapy. Thus, spironolactone seems to have no additional antiproteinuric effects over ACE inhibitor therapy in patients with IMN and nephrotic syndrome and carries the risk of significant hyperkalemia.

  10. SOCS3 and SOCS5 mRNA expressions may predict initial steroid response in nephrotic syndrome children

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    Witold Szaflarski

    2012-01-01

    Full Text Available Suppressors of Cytokine Signaling (SOCS inhibit Signal Transducers and Activators of Transcription (STATs phosphorylation by binding and inhibiting Janus Kinases (JaKs. The aim of the present study was to evaluate the influence of glucocorticosteroids on the JaK/STAT signaling pathway in the leukocytes of nephrotic syndrome (NS patients. The study group was composed of 34 steroid sensitive NS (SSNS children and 20 steroid resistant NS (SRNS subjects. Gene expression was assessed by real-time PCR using pre-designed human JaK/STAT PCR array. Protein expression was evaluated using ELISA assay (plasma concentration and immunofluorescence (in situ protein expression. In SSNS children, the initial increased expression of JaK1, JaK2, JaK3, STAT1, STAT2, STAT6, TYK2, SOCS1, SOCS2, SOCS3, SOCS4 and SOCS5 was reduced back to the control limits. Similarly, in SRNS patients the increased levels of almost all mRNA expressions for the abovementioned genes were decreased, with the exceptions of SOCS3 and SOCS5 expressions. These mRNA expressions were still significantly increased and correlated with early unfavorable course of nephrotic syndrome in children. Plasma levels of SOCS3, SOCS5, IL-6 and IL-20 were significantly increased in SRNS subjects after six weeks of steroids medication compared to SSNS and control participants. We conclude that SOCS3 and SOCS5 increased mRNA expressions might predict initial resistance to steroids in NS patients. (Folia Histochemica et Cytobiologica 2011; Vol. 49, No. 4, pp. 719–728

  11. 小儿肾病综合征合并低钠血症的护理体会%Nursing Experience of Hyponatremia in Children with Nephrotic Syndrome

    Institute of Scientific and Technical Information of China (English)

    饶萍

    2014-01-01

    目的分析原发性肾病综合征的观察和护理经验,探讨低钠血症的儿童的护理方法。方法对小儿原发性肾病综合征患者低钠血症的系统观察,积极采取干预措施。结果儿童原发性肾病综合征264例,低钠血症176例,其中轻度72例,中度64例,重度32例,极8例。经过仔细观察,积极治疗后有所改善。结论在原发性肾病综合征并发低钠血症的临床症状的儿童,及时了解,早期发现,早期诊断,早期治疗和护理,可减少严重后果的发生。%Objective To analysis, discussion of children with primary nephrotic syndrome observation. Methods The children with primary nephrotic syndrome patients comprehensive system of hyponatremia observed, active intervention measures. Results Children with primary nephrotic syndrome in 264 cases, 176 cases of hyponatremia, including 72 mild cases, 64 moderate cases and 32 severe cases, 8. After careful observation, active treatment improved. Conclusion In children with primary nephrotic syndrome complicated with hyponatremia, clinical symptoms of malaise, timely understanding of the disease, early detection, early diagnosis, early treatment and nursing, can reduce the occur ence of serious consequences.

  12. Acid-base disturbances in nephrotic syndrome: analysis using the CO2/HCO3 method (traditional Boston model) and the physicochemical method (Stewart model).

    Science.gov (United States)

    Kasagi, Tomomichi; Imai, Hirokazu; Miura, Naoto; Suzuki, Keisuke; Yoshino, Masabumi; Nobata, Hironobu; Nagai, Takuhito; Banno, Shogo

    2017-03-13

    The Stewart model for analyzing acid-base disturbances emphasizes serum albumin levels, which are ignored in the traditional Boston model. We compared data derived using the Stewart model to those using the Boston model in patients with nephrotic syndrome. Twenty-nine patients with nephrotic syndrome and six patients without urinary protein or acid-base disturbances provided blood and urine samples for analysis that included routine biochemical and arterial blood gas tests, plasma renin activity, and aldosterone. The total concentration of non-volatile weak acids (ATOT), apparent strong ion difference (SIDa), effective strong ion difference (SIDe), and strong ion gap (SIG) were calculated according to the formulas of Agrafiotis in the Stewart model. According to the Boston model, 25 of 29 patients (90%) had alkalemia. Eighteen patients had respiratory alkalosis, 11 had metabolic alkalosis, and 4 had both conditions. Only three patients had hyperreninemic hyperaldosteronism. The Stewart model demonstrated respiratory alkalosis based on decreased PaCO2, metabolic alkalosis based on decreased ATOT, and metabolic acidosis based on decreased SIDa. We could diagnose metabolic alkalosis or acidosis with a normal anion gap after comparing delta ATOT [(14.09 - measured ATOT) or (11.77 - 2.64 × Alb (g/dL))] and delta SIDa [(42.7 - measured SIDa) or (42.7 - (Na + K - Cl)]). We could also identify metabolic acidosis with an increased anion gap using SIG > 7.0 (SIG = 0.9463 × corrected anion gap-8.1956). Patients with nephrotic syndrome had primary respiratory alkalosis, decreased ATOT due to hypoalbuminemia (power to metabolic alkalosis), and decreased levels of SIDa (power to metabolic acidosis). We could detect metabolic acidosis with an increased anion gap by calculating SIG. The Stewart model in combination with the Boston model facilitates the analysis of complex acid-base disturbances in nephrotic syndrome.

  13. [Idiopathic nephrotic syndrome with minimal glomerular lesions in children (author's transl)].

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    Scharer, K; Gilli, G

    1975-01-01

    Minimal glomerular changes were found in 80% of the cases of nephrotic sindromes in children. 5 groups can be separated under light microscopy. Electron microscopy always shows foot processes fusion. 80% of the children show edema, one third has transient hematuria, proteinuria is selective for small molecules, plasma B 1 C globuline levels are normal. According with the International Study of kidney disease in children, prednisone therapy gives positive results in 84% of all cases (Early responders) but many of them relapse (Frequent relapsers); the long-term prognosis with steroid is poor in the majority of cases. Cyclophosphamide therapy (2,5-3 mg/kg/die for 6 week and for a maximum of 60-80 days) gives better results and lesser relapses, but caution is needed because of an higher toxicity.

  14. A case of steroid-induced psychosis in a child having nephrotic syndrome with toxic epidermal necrolysis

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    Sae Yoon Kim

    2010-03-01

    Full Text Available Toxic epidermal necrolysis (TEN and Stevens&#8211;Johnson syndrome (SJS are rare, life-threatening mucocutaneous diseases, usually attributable to drugs and infections. Corticosteroids have been used in the management of TEN for the last 30 years. This remains controversial and is still much debated. TEN can occur despite administration of high doses of systemic corticosteroids. The psychiatric side effects of corticosteroids can include headache, insomnia, depression, and mood disorders with or without psychotic episodes. Steroid-induced psychosis is dealt with by tapering or discontinuing the steroid; antipsychotics are also sometimes used. We report a case of an 11-year-old boy who was admitted with TEN. He had also been diagnosed as having nephrotic syndrome in the past. Remission was achieved through induction therapy and by maintaining the use of steroids. After a full-dose intravenous dexamethasone for TEN, he showed psychotic symptoms. We diagnosed him as having steroid-induced psychosis. We tapered the steroid use and initiated an atypical antipsychotic medication, olazapine and intravenous immunoglobulin (IV-IG. His symptoms dramatically improved and he was discharged.

  15. Cell biology and genetics of minimal change disease [version 1; referees: 2 approved

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    Moin A. Saleem

    2016-03-01

    Full Text Available Minimal change disease (MCD is an important cause of nephrotic syndrome and is characterized by massive proteinuria and hypoalbuminemia, resulting in edema and hypercholesterolemia. The podocyte plays a key role in filtration and its disruption results in a dramatic loss of function leading to proteinuria. Immunologic disturbance has been suggested in the pathogenesis of MCD. Because of its clinical features, such as recurrent relapse/remission course, steroid response in most patients, and rare familial cases, a genetic defect has been thought to be less likely in MCD. Recent progress in whole-exome sequencing reveals pathogenic mutations in familial cases in steroid-sensitive nephrotic syndrome (SSNS and sheds light on possible mechanisms and key molecules in podocytes in MCD. On the other hand, in the majority of cases, the existence of circulating permeability factors has been implicated along with T lymphocyte dysfunction. Observations of benefit with rituximab added B cell involvement to the disease. Animal models are unsatisfactory, and the humanized mouse may be a good model that well reflects MCD pathophysiology to investigate suggested “T cell dysfunction” directly related to podocytes in vivo. Several candidate circulating factors and their effects on podocytes have been proposed but are still not sufficient to explain whole mechanisms and clinical features in MCD. Another circulating factor disease is focal segmental glomerulosclerosis (FSGS, and it is not clear if this is a distinct entity, or on the same spectrum, implicating the same circulating factor(s. These patients are mostly steroid resistant and often have a rapid relapse after transplantation. In clinical practice, predicting relapse or disease activity and response to steroids is important and is an area where novel biomarkers can be developed based on our growing knowledge of podocyte signaling pathways. In this review, we discuss recent findings in genetics and

  16. Nephrotic syndrome associated with hepatointestinal schistosomiasis Síndrome nefrótica associada à esquistossomose hepatointestinal

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    H. Abensur

    1992-08-01

    Full Text Available Schistosomal nephropathy has long been related to the hepatosplenic form of schistosomiasis. In the last few years, 24 patients with hepatointestinal schistosomiasis and the nephrotic syndrome were studied. Aiming at evaluating a possible etiologic participation of schistosomiasis in the development of the nephropathy, this group was comparatively studied with a group of 37 patients with idiopathic nephrotic syndrome. Both groups had a different distribution of the histologic lesions. In the group with schistosomiasis there was a statistically significant prevalence of proliferative mesangial glomerulonephritis (33.3%, whereas in the control group there was prevalence of membranous glomerulonephritis (32.4%. On immunofluorescence, IgM was positive in 94.4% of the patients with schistosomiasis versus 55.0% in the control group (pA nefropatia esquistossomótica está classicamente vinculada à fornia hepatoesplênica da esquistossomose. Ao longo dos últimos anos 24 casos de pacientes esquistossomóticos hepato-intestinais e portadores de síndrome nefrótica foram estudados. Com o objetivo de verificar a possível participação etiológica da esquistossomose na gênese da nefropatia, analisamos este grupo comparativamente ao grupo de 37 doentes portadores de síndrome nefrótica idiopática. Ambos os grupos apresentaram distribuição distinta dos tipos histológicos de glomerulopatia. No grupo de esquistossomóticos houve predomínio estatisticamente significante de glomerulonefrite proliferativa mesangial (33.3%, enquanto no grupo controle houve predomínio da glomerulonefrite membranosa (32.4%. A positividade para IgM à imunofluorescência foi de 94.4% nos doentes esquistossomóticos versus 55.0% no grupo controle (p<0.01. No grupo de esquistossomóticos 8 pacientes evidenciaram glomerulonefrite proliferativa mesangial e 5, glomerulonefrite membranoproliferativa. Em ambos os tipos histológicos a imunofluorescência mostrou dep

  17. Rapid Response to Cyclosporin A and Favorable Renal Outcome in Nongenetic Versus Genetic Steroid-Resistant Nephrotic Syndrome.

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    Büscher, Anja K; Beck, Bodo B; Melk, Anette; Hoefele, Julia; Kranz, Birgitta; Bamborschke, Daniel; Baig, Sabrina; Lange-Sperandio, Bärbel; Jungraithmayr, Theresa; Weber, Lutz T; Kemper, Markus J; Tönshoff, Burkhard; Hoyer, Peter F; Konrad, Martin; Weber, Stefanie

    2016-02-05

    Treatment of congenital nephrotic syndrome (CNS) and steroid-resistant nephrotic syndrome (SRNS) is demanding, and renal prognosis is poor. Numerous causative gene mutations have been identified in SRNS that affect the renal podocyte. In the era of high-throughput sequencing techniques, patients with nongenetic SRNS frequently escape the scientific interest. We here present the long-term data of the German CNS/SRNS Follow-Up Study, focusing on the response to cyclosporin A (CsA) in patients with nongenetic versus genetic disease. Cross-sectional and longitudinal clinical data were collected from 231 patients with CNS/SRNS treated at eight university pediatric nephrology units with a median observation time of 113 months (interquartile range, 50-178). Genotyping was performed systematically in all patients. The overall mutation detection rate was high at 57% (97% in CNS and 41% in SRNS); 85% of all mutations were identified by the analysis of three single genes only (NPHS1, NPHS2, and WT1), accounting for 92% of all mutations in patients with CNS and 79% of all mutations in patients with SRNS. Remission of the disease in nongenetic SRNS was observed in 78% of patients after a median treatment period of 2.5 months; 82% of nongenetic patients responded within 6 months of therapy, and 98% of patients with nongenetic SRNS and CsA-induced complete remission (normalbuminemia and no proteinuria) maintained a normal renal function. Genetic SRNS, on the contrary, is associated with a high rate of ESRD in 66% of patients. Only 3% of patients with genetic SRNS experienced a complete remission and 16% of patients with genetic SRNS experienced a partial remission after CsA therapy. The efficacy of CsA is high in nonhereditary SRNS, with an excellent prognosis of renal function in the large majority of patients. CsA should be given for a minimum period of 6 months in these patients with nongenetic SRNS. In genetic SRNS, response to CsA was low and restricted to exceptional

  18. Cyclosporine C2 monitoring for the treatment of frequently relapsing nephrotic syndrome in children: a multicenter randomized phase II trial.

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    Iijima, Kazumoto; Sako, Mayumi; Oba, Mari Saito; Ito, Shuichi; Hataya, Hiroshi; Tanaka, Ryojiro; Ohwada, Yoko; Kamei, Koichi; Ishikura, Kenji; Yata, Nahoko; Nozu, Kandai; Honda, Masataka; Nakamura, Hidefumi; Nagata, Michio; Ohashi, Yasuo; Nakanishi, Koichi; Yoshikawa, Norishige

    2014-02-01

    An open-label, multicenter, randomized phase II trial was conducted from July 1, 2005 to March 29, 2011 to compare two protocols for treating children with frequently relapsing nephrotic syndrome using microemulsified cyclosporine. Ninety-three children with frequently relapsing nephrotic syndrome were randomly assigned to group A (n=46) or group B (n=47). In both groups, the 2-hour postdose cyclosporine level was monitored. For group A, the cyclosporine target was set to 600-700 ng/ml for the first 6 months and 450-550 ng/ml for the next 18 months; for group B, it was set to 450-550 ng/ml for the first 6 months and 300-400 ng/ml for the next 18 months. The primary end point was the sustained remission rate. At the end of the study, if there was no difference in safety profile between the two groups and the sustained remission rate in group A was superior to group B with a decision threshold of 8%, then the regimen for group A would be determined the better treatment. Eight children from an ineligible institution, where cyclosporine levels were not measured, were excluded from all analyses. At 24 months, the sustained remission rate was nonsignificantly higher in group A (n=43) than group B (n=42; 64.4% versus 50.0%; hazard ratio, 0.57; 95% confidence interval, 0.29 to 1.11; P=0.09), and the progression-free survival rate was significantly higher (88.1% versus 68.4%; hazard ratio, 0.33; 95% confidence interval, 0.12 to 0.94; P=0.03). The relapse rate was significantly lower in group A than group B (0.41 versus 0.95 times/person-year; hazard ratio, 0.43; 95% confidence interval, 0.19 to 0.84; P=0.02). The rate and severity of adverse events were similar in both treatment groups. The sustained remission rate was not significantly different between the two treatment groups, but the regimen with the higher 2-hour postdose cyclosporine level target improved progression-free survival and reduced the relapse rate.

  19. Oxidative Stress and Nuclear Factor κB (NF-κB) Increase Peritoneal Filtration and Contribute to Ascites Formation in Nephrotic Syndrome.

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    Udwan, Khalil; Brideau, Gaëlle; Fila, Marc; Edwards, Aurélie; Vogt, Bruno; Doucet, Alain

    2016-05-20

    Water accumulation in the interstitium (edema) and the peritoneum (ascites) of nephrotic patients is classically thought to stem from the prevailing low plasma albumin concentration and the decreased transcapillary oncotic pressure gradient. However, several clinical and experimental observations suggest that it might also stem from changes in capillary permeability. We addressed this hypothesis by studying the peritoneum permeability of rats with puromycin aminonucleoside-induced nephrotic syndrome. The peritoneum of puromycin aminonucleoside rats displayed an increase in the water filtration coefficient of paracellular and transcellular pathways, and a decrease in the reflection coefficient to proteins. It also displayed oxidative stress and subsequent activation of NF-κB. Scavenging of reactive oxygen species and inhibition of NF-κB prevented the changes in the water permeability and reflection coefficient to proteins and reduced the volume of ascites by over 50%. Changes in water permeability were associated with the overexpression of the water channel aquaporin 1, which was prevented by reactive oxygen species scavenging and inhibition of NF-κB. In conclusion, nephrotic syndrome is associated with an increased filtration coefficient of the peritoneum and a decreased reflection coefficient to proteins. These changes, which account for over half of ascite volume, are triggered by oxidative stress and subsequent activation of NF-κB.

  20. Nephrotic syndrome as a clinical manifestation of graft-versus-host disease (GVHD) in a marrow transplant recipient after cyclosporine withdrawal.

    Science.gov (United States)

    Oliveira, J S; Bahia, D; Franco, M; Balda, C; Stella, S; Kerbauy, J

    1999-01-01

    GVHD is one of the most frequent complications of BMT and recently nephrotic syndrome (NS) has been described as a manifestation of chronic GVHD. Here, we present an AA patient who developed NS 1 year after BMT when cyclosporine was stopped. Renal biopsy showed focal sclerosis associated with membranous deposits. He also had other clinical manifestations of chronic GVHD: sicca-like syndrome and colestasis. After 15 days of CsA therapy, he experienced a remarkable improvement in the NS and GVHD as a whole. We comment on immunological mechanisms that could be involved in the pathogenesis of this manifestation.

  1. Sonographic Diagnosis in a Rare Aetiology of Neonatal Scrotal Swellings: A Case Report of Congenital Nephrotic Syndrome

    Science.gov (United States)

    Grover, Shabnam Bhandari; Kumar, Nishith; Grover, Hemal; Taneja, Dinesh Kumar; Katyan, Amit

    2016-01-01

    Summary Background Common etiologies of scrotal swelling in neonates include hydrocoele, inguinal hernia and testicular torsion; less common is epididymo-orchitis. Congenital nephrotic syndrome (CNS), a rare entity, is known to present as progressive renal failure and its leading presentation with scrotal involvement has not been reported. Material/Methods We report a rare case of CNS with primary clinical presentation as scrotal cellulitis and epididymo-orchitis. In this neonate, scrotal and abdominal ultrasound examination was performed and the laboratory data were obtained. Results Sonography revealed bilaterally enlarged echogenic kidneys, testis and epididymis with echogenic peritoneal fluid tracking into both scrotal sacs. Laboratory data revealed proteinuria and severe depletion of serum IgG. Culture of the peritoneal fluid showed gram-negative organisms. A final diagnosis of CNS, complicated with peritonitis tracking into the scrotal sacs was arrived at. Conclusions CNS may have a rare presentation with distracting symptoms of scrotal cellulitis and epididymo-orchitis, as seen in our patient. However, diligent use of abdomino-scrotal sonography, supported by relevant laboratory data can clinch the accurate diagnosis. PMID:27757175

  2. Benefits of once-daily administration of cyclosporine a for children with steroid-dependent, relapsing nephrotic syndrome.

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    Suzuki, Koichi; Oki, Eishin; Tsuruga, Kazushi; Aizawa-Yashiro, Tomomi; Ito, Etsuro; Tanaka, Hiroshi

    2010-03-01

    Cyclosporine A (CsA) is an effective steroid-sparing agent for patients with steroid-dependent, relapsing nephrotic syndrome (SDRNS). The efficacy and safety of single-daily dose administration (SDD protocol) of CsA in selected patients with SDRNS has been reported. However, the efficacy of initial CsA treatment for children with SDRNS using the SDD protocol remains to be elucidated. The SDD protocol might be associated with lower clinical toxicity, compared to the conventional twice-daily dose administration (TDD protocol). Here we evaluated the efficacy and safety of the SDD protocol versus the TDD protocol in patients with SDRNS. The data from 19 patients (9.9 +/- 4.2 years old) were retrospectively collected and analyzed. Ten patients treated according to the SDD protocol for a mean of 27 months (SDD group), while 9 patients treated with the TDD protocol for a mean of 35 months (TDD group) as an initial CsA treatment. Although the mean daily CsA dose was significantly lower in the SDD group (1.5 +/- 0.4 mg/kg/day vs. 3.7 +/- 0.7 mg/kg/day, P SDD group showed nephrotoxicity. Despite a small number of patients, this study may support that the SDD protocol is at least as effective as the conventional TDD protocol, and is more cost-effective for selected children with SDRNS.

  3. Significance of technetium-99m human serum albumin diethylenetriamine pentaacetic acid scintigraphy in patients with nephrotic syndrome.

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    Tsuyoshi Takashima

    Full Text Available It is thought that a large amount of albumin leaking from the glomerulus in nephrotic syndrome (NS is reabsorbed at the proximal tubule and catabolized. Therefore, it is possible the final quantity of urinary protein does not always reflect the amount of leakage of protein from the glomerulus. We experienced two cases without nephrotic range proteinuria thought to involve hypoproteinemia due to the same pathophysiology as NS. On these patients, we performed protein leakage scintigraphy with technetium-99m human serum albumin diethylenetriamine pentaacetic acid (99mTc-HSAD to exclude a diagnosis of protein-losing gastroenteropathy and observed diffuse positive accumulation in the kidneys with more intense uptake in the kidney than the liver on the anterior view 24 hours after 99mTc-HSAD administration. In healthy adults intravenously given 99mTc-HSAD, the same dynamics are observed as in albumin metabolism, and the organ radioactivity of the liver and kidneys after 24 hours is equal. Therefore, we thought it was possible that the renal uptake 24 hours after 99mTc-HSAD administration was a characteristic finding of NS. In order to confirm it, the subjects were divided into two groups: the NS group (n = 10 and the non-NS group (n = 7. We defined more intense uptake in the kidney than the liver on the anterior view 24 hours after 99mTc-HSAD administration as Dense Kidney (+. Furthermore, we designed regions of interest in the right and left kidneys and liver on anterior and posterior images, then calculated the kidney-liver ratio. Nine of the ten patients had Dense Kidney (+ in the NS group, compared to none in the non-NS group. And the kidney-liver ratio was significantly higher in the NS group than in the non-NS group on each view in the bilateral kidneys. In conclusion, our results suggest that the renal uptake 24 hours after 99mTc-HSAD administration is a characteristic finding of NS.

  4. Recurrent Acute Renal Failure in a Patient with Minimal Change Disease

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    Abdelrahman Mohammad

    2001-01-01

    Full Text Available We report on a 16-year old female patient with biopsy proved minimal change disease and steroid state. She was subsequentlygiven cyclosporin for varying periods of time with inadequate response. She developed four episodes of acute renal failure in the background of severe nephrotic state, 10 years after the onset of her illness. Three of these episodes could be reversed with albumin infusion and judicious use of diuretics while the fourth necessitated six sessions of ultrafiltration. In none of the episodes of ARF could we find any cause other than the nephrotic state itself.

  5. Epithelial sodium channel (ENaC) subunit mRNA and protein expression in rats with puromycin aminonucleoside-induced nephrotic syndrome.

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    Audigé, A; Yu, Z R; Frey, B M; Uehlinger, D E; Frey, F J; Vogt, B

    2003-04-01

    In experimental nephrotic syndrome, urinary sodium excretion is decreased during the early phase of the disease. The molecular mechanism(s) leading to salt retention has not been completely elucidated. The rate-limiting constituent of collecting duct sodium transport is the epithelial sodium channel (ENaC). We examined the abundance of ENaC subunit mRNAs and proteins in puromycin aminonucleoside (PAN)-induced nephrotic syndrome. The time courses of urinary sodium excretion, plasma aldosterone concentration and proteinuria were studied in male Sprague-Dawley rats treated with a single dose of either PAN or vehicle. The relative amounts of alphaENaC, betaENaC and gammaENaC mRNAs were determined in kidneys from these rats by real-time quantitative TaqMan PCR, and the amounts of proteins by Western blot. The kinetics of urinary sodium excretion and the appearance of proteinuria were comparable with those reported previously. Sodium retention occurred on days 2, 3 and 6 after PAN injection. A significant up-regulation of alphaENaC and betaENaC mRNA abundance on days 1 and 2 preceded sodium retention on days 2 and 3. Conversely, down-regulation of alphaENaC, betaENaC and gammaENaC mRNA expression on day 3 occurred in the presence of high aldosterone concentrations, and was followed by a return of sodium excretion to control values. The amounts of alphaENaC, betaENaC and gammaENaC proteins were not increased during PAN-induced sodium retention. In conclusion, ENaC mRNA expression, especially alphaENaC, is increased in the very early phase of the experimental model of PAN-induced nephrotic syndrome in rats, but appears to escape from the regulation by aldosterone after day 3.

  6. Proteomics Approach Identifies Factors Associated With the Response to Low-Density Lipoprotein Apheresis Therapy in Patients With Steroid-Resistant Nephrotic Syndrome.

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    Kuribayashi-Okuma, Emiko; Shibata, Shigeru; Arai, Shigeyuki; Ota, Tatsuru; Watanabe, Sumiyo; Hisaki, Harumi; Okazaki, Tomoki; Toda, Tosifusa; Uchida, Shunya

    2016-04-01

    Low-density lipoprotein apheresis (LDL-A) has been shown to reduce proteinuria in a subgroup of nephrotic syndrome patients refractory to immunosuppressive therapy. Factors influencing the efficacy of LDL-A in nephrotic syndrome are completely unknown. Using a proteomics approach, we aimed to identify biological markers that predict the response to LDL-A in patients with steroid-resistant nephrotic syndrome (SRNS). Identification of plasma proteins bound to the dextran-sulfate column at the first session of LDL-A was determined by mass spectrometry. To investigate biological factors associated with the response to LDL-A, we compared profiles of column-bound proteins between responders (defined by more than 50% reduction of proteinuria after the treatment) and non-responders by 2-dimensional gel electrophoresis (2-DE) coupled to mass spectrometry in seven patients with SRNS. Evaluation of proteins adsorbed to LDL-A column in patients with SRNS revealed the identity of 62 proteins, which included apolipoproteins, complement components, and serum amyloid P-component (SAP). Comparative analysis of the column-bound proteins between responders and non-responders by 2-DE demonstrated that apolipoprotein E (APOE) and SAP levels were increased in non-responders as compared with responders. These results were confirmed by western blotting. Moreover, serum levels of APOE and SAP were significantly higher in the non-responder group than in the responder group by ELISA. Our data provide comprehensive analysis of proteins adsorbed by LDL-A in SRNS, and demonstrate that the serum levels of APOE and SAP may be used to predict the response to LDL-A in these patients.

  7. ACE I/D gene polymorphism can't predict the steroid responsiveness in Asian children with idiopathic nephrotic syndrome: a meta-analysis.

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    Tian-Biao Zhou

    Full Text Available BACKGROUND: The results from the published studies on the association between angiotensin-converting enzyme (ACE insertion/deletion (I/D gene polymorphism and the treatment response to steroid in Asian children with idiopathic nephrotic syndrome (INS is still conflicting. This meta-analysis was performed to evaluate the relation between ACE I/D gene polymorphism and treatment response to steroid in Asian children and to explore whether ACE D allele or DD genotype could become a predictive marker for steroid responsiveness. METHODOLOGY/PRINCIPAL FINDINGS: Association studies were identified from the databases of PubMed, Embase, Cochrane Library and CBM-disc (China Biological Medicine Database as of September 1, 2010, and eligible investigations were synthesized using meta-analysis method. Five investigations were identified for the analysis of association between ACE I/D gene polymorphism and steroid-resistant nephrotic syndrome (SRNS risk in Asian children and seven studies were included to explore the relationship between ACE I/D gene polymorphism and steroid-sensitive nephrotic syndrome (SSNS susceptibility. Five investigations were recruited to explore the difference of ACE I/D gene distribution between SRNS and SSNS. There was no a markedly association between D allele or DD genotype and SRNS susceptibility or SSNS risk, and the gene distribution differences of ACE between SRNS and SSNS were not statistically significant. II genotype might play a positive role against SRNS onset but not for SSNS (OR = 0.51, P = 0.02; OR = 0.95, P = 0.85; respectively, however, the result for the association of II genotype with SRNS risk was not stable. CONCLUSIONS/SIGNIFICANCE: Our results indicate that D allele or DD homozygous can't become a significant genetic molecular marker to predict the treatment response to steroid in Asian children with INS.

  8. The analysis of clinical features and outcomes of children with late steroid resistant primary nephrotic syndrome%儿童迟发激素耐药型肾病综合征的临床和转归分析

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    杨青; 陈敏广; 叶晓华; 林洪洲

    2015-01-01

    -sensitive nephrotic syndrome in initial treatment, 21 of them with late steroid-resistant nephrotic syndrome, accounting for 4.1%. In all of 21 patients, there were 11 males and 10 females, 16 cases were simple type while 5 cases with nephritis type.Before late steroid resistance,14 cases showed frequency relapse or steroid-dependent, seven cases were non-frequency relapse or relapse-free. The interval from steroid-sensitive to late steroid-resistant was (206.10± 212.64) d. Compared with steroid-sensitive group, the proteinuria negative time more longer in initial steroid treatment[(14.05± 7.71)dvs (10.26± 5.66)d,P=0.003], more common in women (11/10 vs 380/112,P=0.018). Compared with the relapse group, the interval from ifrst relapse to ifrst remission was more shorter in late steroid-resistant group(34.95± 39.44dvs 122.10± 168.07d,P= 0.019). There were 12 cases received renal biopsy and histology showed minimal change disease(MCD) in 7 cases, focal segmental glomerulosclerosis(FSGS) in 4 cases, C1q nephropathy in one case. About 85.7% (18/21 patients) achieved complete remission after treated by cyclosporine and cyclophosphamide, 1 case (4.8%) with FSGS reached partial remission after combined steroid and mycophenolate mofetil therapy, only one case with C1q nephropathy still treatment futility after multidrug therapy and death due to ESRD after three years. Conclusions Children with late steroid -resistant nephrotic syndrome are not uncommon. It's probably useful to predict the occurrence of late steroid-resistant by the proteinuria negative time after ifrst steroid therapy, the interval from ifrst relapse to ifrst remission and gender. The mainly renal pathological changes are MCD and FSGS, majority cases are good response to combined steroid and immunosuppressive therapy, the prognosis is good, but also need pay attention to a small portion of the cases continued resistance and show poor prognosis.

  9. Cytoplasmic Localization of WT1 and Decrease of miRNA-16-1 in Nephrotic Syndrome

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    Rangel-Ochoa, Gloria Azucena; Rodríguez-Padilla, Cristina

    2017-01-01

    Nephrotic syndrome (NS) is a glomerular disease that is defined by the leakage of protein into the urine and is associated with hypoalbuminemia, hyperlipidemia, and edema. Steroid-resistant NS (SRNS) patients do not respond to treatment with corticosteroids and show decreased Wilms tumor 1 (WT1) expression in podocytes. Downregulation of WT1 has been shown to be affected by certain microRNAs (miRNAs). Twenty-one patients with idiopathic NS (68.75% were SSNS and 31.25% SRNS) and 10 healthy controls were enrolled in the study. Podocyte number and WT1 location were determined by immunofluorescence, and the serum levels of miR-15a, miR-16-1, and miR-193a were quantified by RT-qPCR. Low expression and delocalization of WT1 protein from the nucleus to the cytoplasm were found in kidney biopsies of patients with SRNS and both nuclear and cytoplasmic localization were found in steroid-sensitive NS (SSNS) patients. In sera from NS patients, low expression levels of miR-15a and miR-16-1 were found compared with healthy controls, but only the miR-16-1 expression levels showed statistically significant decrease (p = 0.019). The miR-193a expression levels only slightly increased in NS patients. We concluded that low expression and delocalization from the WT1 protein in NS patients contribute to loss of podocytes while modulation from WT1 protein is not associated with the miRNAs analyzed in sera from the patients. PMID:28299339

  10. Cytoplasmic Localization of WT1 and Decrease of miRNA-16-1 in Nephrotic Syndrome

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    Pablo Zapata-Benavides

    2017-01-01

    Full Text Available Nephrotic syndrome (NS is a glomerular disease that is defined by the leakage of protein into the urine and is associated with hypoalbuminemia, hyperlipidemia, and edema. Steroid-resistant NS (SRNS patients do not respond to treatment with corticosteroids and show decreased Wilms tumor 1 (WT1 expression in podocytes. Downregulation of WT1 has been shown to be affected by certain microRNAs (miRNAs. Twenty-one patients with idiopathic NS (68.75% were SSNS and 31.25% SRNS and 10 healthy controls were enrolled in the study. Podocyte number and WT1 location were determined by immunofluorescence, and the serum levels of miR-15a, miR-16-1, and miR-193a were quantified by RT-qPCR. Low expression and delocalization of WT1 protein from the nucleus to the cytoplasm were found in kidney biopsies of patients with SRNS and both nuclear and cytoplasmic localization were found in steroid-sensitive NS (SSNS patients. In sera from NS patients, low expression levels of miR-15a and miR-16-1 were found compared with healthy controls, but only the miR-16-1 expression levels showed statistically significant decrease (p=0.019. The miR-193a expression levels only slightly increased in NS patients. We concluded that low expression and delocalization from the WT1 protein in NS patients contribute to loss of podocytes while modulation from WT1 protein is not associated with the miRNAs analyzed in sera from the patients.

  11. [Mutational analysis of MYO1E in children with sporadic steroid-resistant nephrotic syndrome in Chinese Han ethnic group].

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    Zhao, Feng; Yu, Zihua; Yang, Yonghui; Nie, Xiaojing; Huang, Jun; Wang, Chengfeng; Xia, Guizhi; Chen, Guangming

    2014-07-01

    Previous studies have demonstrated that two homozygous missense MYO1E mutations are associated with childhood autosomal recessive focal segmental glomerulosclerosis in steroid-resistant nephrotic syndrome (SRNS) families from Italy and Turkey. Non-disease-causing heterozygous MYO1E variants were also found in other SRNS patient cohorts. However, the role of MYO1E mutations in Chinese sporadic SRNS has not been established. Peripheral blood samples were collected for genetic analysis from 54 children with sporadic SRNS in Chinese Han ethnic group and a normal control group of 59 healthy adult volunteers. None of the patients carried mutations in NPHS2 or WT1. Genomic DNA was extracted from peripheral blood leukocytes. Twenty-eight exons and exon-intron boundaries of the MYO1E gene were amplified by polymerase chain reaction. Mutational analysis was performed by direct DNA sequencing and restriction endonuclease digestion. Fifty-one variants in the MYO1E gene were identified in 54 children with sporadic SRNS. Among them, 10 MYO1E mutations of IVS1-11T>C, IVS2-86T>A, 279T>C (D93D), IVS6-181G>A, 718C>T (L240F), 1678A>G (T560A), IVS16-35A>G, IVS18+48T>A, IVS19+38G>A and IVS25+13C>T were detected in 11 patients, whereas they were absent in the 59 normal Chinese controls. Forty-one variants in MYO1E were identified and all of them were published in single nucleotide polymorphism database from national center for biotechnology information. Furthermore, all the 10 MYO1E mutations were in heterozygous states. MYO1E mutations are not a major cause of Chinese children with sporadic SRNS in the study.

  12. Activation of the IL-2 Receptor in Podocytes: A Potential Mechanism for Podocyte Injury in Idiopathic Nephrotic Syndrome?

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    Zea, Arnold H.; Stewart, Tyrus; Ascani, Jeannine; Tate, David J.; Finkel-Jimenez, Beatriz; Wilk, Anna; Reiss, Krzysztof; Smoyer, William E.; Aviles, Diego H.

    2016-01-01

    The renal podocyte plays an important role in maintaining the structural integrity of the glomerular basement membrane. We have previously reported that patients with idiopathic nephrotic syndrome (INS) have increased IL-2 production. We hypothesized that podocytes express an IL-2 receptor (IL-2R) and signaling through this receptor can result in podocyte injury. To confirm the presence of the IL-2R, we tested a conditionally immortalized murine podocyte cell line by flow cytometry, qPCR, and Western blot. To test for the presence of the IL-2R in vivo, immunohistochemical staining was performed on human renal biopsies in children with FSGS and control. Podocytes were stimulated with IL-2 in vitro, to study signaling events via the JAK/STAT pathway. The results showed that stimulation with IL-2 resulted in increased mRNA and protein expression of STAT 5a, phosphorylated STAT 5, JAK 3, and phosphorylated JAK 3. We then investigated for signs of cellular injury and the data showed that pro-apoptotic markers Bax and cFLIP were significantly increased following IL-2 exposure, whereas LC3 II was decreased. Furthermore, mitochondrial depolarization and apoptosis were both significantly increased following activation of the IL-2R. We used a paracellular permeability assay to monitor the structural integrity of a podocyte monolayer following IL-2 exposure. The results showed that podocytes exposed to IL-2 have increased albumin leakage across the monolayer. We conclude that murine podocytes express the IL-2R, and that activation through the IL-2R results in podocyte injury. PMID:27389192

  13. Activation of the IL-2 Receptor in Podocytes: A Potential Mechanism for Podocyte Injury in Idiopathic Nephrotic Syndrome?

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    Arnold H Zea

    Full Text Available The renal podocyte plays an important role in maintaining the structural integrity of the glomerular basement membrane. We have previously reported that patients with idiopathic nephrotic syndrome (INS have increased IL-2 production. We hypothesized that podocytes express an IL-2 receptor (IL-2R and signaling through this receptor can result in podocyte injury. To confirm the presence of the IL-2R, we tested a conditionally immortalized murine podocyte cell line by flow cytometry, qPCR, and Western blot. To test for the presence of the IL-2R in vivo, immunohistochemical staining was performed on human renal biopsies in children with FSGS and control. Podocytes were stimulated with IL-2 in vitro, to study signaling events via the JAK/STAT pathway. The results showed that stimulation with IL-2 resulted in increased mRNA and protein expression of STAT 5a, phosphorylated STAT 5, JAK 3, and phosphorylated JAK 3. We then investigated for signs of cellular injury and the data showed that pro-apoptotic markers Bax and cFLIP were significantly increased following IL-2 exposure, whereas LC3 II was decreased. Furthermore, mitochondrial depolarization and apoptosis were both significantly increased following activation of the IL-2R. We used a paracellular permeability assay to monitor the structural integrity of a podocyte monolayer following IL-2 exposure. The results showed that podocytes exposed to IL-2 have increased albumin leakage across the monolayer. We conclude that murine podocytes express the IL-2R, and that activation through the IL-2R results in podocyte injury.

  14. CD2相关蛋白在肾病综合征中的表达及意义%Expression of CD2AP on podocytes with different pathological types of nephrotic syndrome

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    史秀岩; 张春; 付应峰; 朱忠华; 朱红艳; 易丽霞; 付玲

    2012-01-01

    目的 观察不同病理类型的原发性肾病综合征(nephrotic syndrome,NS)患者肾小球足细胞中CD2相关蛋白(CD2AP)的表达,探讨其与足细胞损伤的关系.方法 选取原发性NS患者54例,10例同期肾肿瘤切除患者正常肾组织作为对照.肾活检后常规染色观察肾脏组织病理改变,肾组织行免疫荧光法CD2AP和肾小球上皮细胞蛋白-1(GLEPP1)双重标记,对肾小球CD2AP的表达进行定位;分别用real time PCR和免疫组化SP法检测组织中CD2AP的表达,采用real time PCR检测nephrin的表达,透射电镜观察足细胞的结构变化,并定量测量足突密度.结果 (1)NS患者肾小球中CD2AP的表达及nephrin的表达下调,足细胞足突不同程度融合,足突密度降低.(2)病理表现为微小病变性肾病(minimal change disease,MCD)、局灶性节段性肾小球硬化(focal segmental glomerulosclerosis,FSGS)和膜性肾病(membranous nephropathy,MN)的NS患者CD2AP表达及nephrin表达较对照组明显降低,且CD2AP与nephrin表达呈正相关,病理表现为MCD和FSGS的NS患者CD2AP表达与足突密度呈正相关.结论 本研究首次发现原发性NS患者肾小球足细胞中CD2AP的表达降低,且在MCD和FSGS中与足细胞病变程度相关,提示CD2AP低表达在足细胞病变为主的肾小球疾病中发挥重要作用.CD2AP有利于诊断足细胞病变的早期检测,对CD2AP表达减低进行早期干预可能有助于延缓疾病进展.%Purpose To investigate the CD2AP expression of the renal podocytes in patients with nephrotic syndrome, and to study the association between expression of CD2AP and podocyte injury. Methods 54 patients with nephrotic syndrome ( NS ) were studied, and 10 patients with renal tumor undergone nephrectomy were enrolled as control. Renal pathological change after renal biopsy was bserved. CD2AP and GLEPP1 by immunofluorescence double staining were performed. The renal CD2AP was detected by immunohis-tochemistry and quantitive real

  15. [New insight in pathogenesis of podocyte disfunction in minimal change disease].

    Science.gov (United States)

    Liu, Shanshan; Chen, Jianghua

    2016-03-01

    Minimal change disease (MCD) is a common pathological type of nephrotic syndrome. Its main histology is the fusion of podocyte foot process. The pathogenesis of MCD is not clear, but previously it was thought to be related to immune mechanism. In recent years more studies show that podocyte injury is the key link in the pathogenesis of MCD. In MCD mouse model and human kidney tissues, the expressions of podocyte slit membrane protein-nephrin and podocin, skeleton protein-synaptopodin are decreased, and the expression of synaptopodin is correlated with the response to hormone therapy. In addition, newest studies focused on another two potocyte associated proteins, CD80 and Angiopoietin-like-4. CD80, a T cell stimulating molecule, is expressed in potocyte. Kappa B gene sequences can be activated by external microbes, antigens through acting potocytes, which can induce the upregulation of CD80 expression, cytoskeletal protein damage and the glomerular filtration rate changes, resulting in proteinuria. Angiopoietin-like-4 can be expressed in normal potocytes, but over-expression of angiopoietin-like-4 may injure the GBM charge barrier and induce the foot process fusion, leading to MCD. However, further studies on the factors inducing CD80 and Angiopoietin-like-4 expression, and the interaction between glomerular basement membrane and the two proteins are needed. Based on the mechanism of MCD, NF-kappa B inhibitors and sialylation therapy would be a novel non-immune therapy for MCD.

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

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    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.

  17. 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.

  18. A PRISMA-compliant meta-analysis of MDR1 polymorphisms and idiopathic nephrotic syndrome: Susceptibility and steroid responsiveness.

    Science.gov (United States)

    Han, Shi-Sheng; Xu, Yan-Qiu; Lu, Yan; Gu, Xiang-Chen; Wang, Yi

    2017-06-01

    Studies have investigated rs1128503, rs1045642, and rs2032582 in multidrug resistance protein 1 (MDR1) for association with susceptibility to idiopathic nephrotic syndrome (INS) and steroid resistance. However, because these findings were inconsistent, we performed a meta-analysis to determine whether there was evidence of a role of these MDR1 variants in INS. The PubMed, Embase, and Web of Science databases were systematically searched to identify studies that examined MDR1 polymorphisms with susceptibility to INS and/or to steroid resistance. Pooled odds ratios (ORs) and 95% confidence intervals (CIs) were calculated by a fixed-effects or random-effects model based on heterogeneity. We selected 9 case-control studies that included 928 patients with INS, of which steroid resistance data were available for 724 (236 were steroid resistant and 488 were steroid sensitive), and 879 healthy controls. All subjects were children. No significant relationships between these polymorphisms and INS susceptibility were identified. Significantly increased risk of steroid resistance was observed with rs1128503 allelic (OR = 1.49, 95% CI = 1.20-1.86) and genotypic (OR = 1.97, 95% CI = 1.18-3.30; OR = 2.03, 95% CI = 1.43-2.88) comparisons, and with allelic (OR = 1.56, 95% CI = 1.05-2.31) and genotypic (OR = 2.85, 95% CI = 1.15-7.07; OR = 2.21, 95% CI = 1.01-4.8) comparisons to rs2032582 in Caucasian populations. However, this association between rs2032582 and steroid resistance was not robust enough to withstand corrections for multiple comparisons. Similarly, we found that the rs1128503T-rs2032582G-rs1045642C (T-G-C) haplotype was associated with an increased risk of steroid resistance (OR = 2.02, 95% CI = 1.13-3.59), while the wild-type C-G-C haplotype was associated with a decreased risk (OR = 0.32, 95% CI = 0.12-0.88) in Caucasians; however, these findings were not significant following adjustments for multiple

  19. Clinicopathological features, diagnosis, and treatment of IgA nephropathy with minimal change disease related to exposure to mercury-containing cosmetics: a case report
.

    Science.gov (United States)

    Niu, Hong-Xin; Li, Shen-Heng; Li, Hong-Ying; Chen, Yi-Hua; Liu, Wei-Wei; Li, Pei-Lin; Long, Hai-Bo

    2017-04-01

    Membranous nephropathy and minimal change disease (MCD) have been involved in mercury-induced nephrotic syndrome. IgA nephropathy is not known to be a common pathological type. In the present article, we report a case of IgA nephropathy with MCD following exposure to mercury-containing skin lightening cream. The patient was a 39-year-old woman who presented with nephrotic syndrome. She had a 6-month history of using as many as 8 kinds of skin-lightening creams, and urinary mercury excretion was high. Renal biopsy revealed IgA nephropathy with MCD. The use of cosmetics was stopped and chelation therapy was given. After 4 courses (1 month) of chelation therapy, there was a complete remission of proteinuria and hematuria, and urine tests remained normal during the 5-year follow-up period. The unique clinical and pathological features of IgA nephropathy with MCD had raised the controversial question of whether MCD and IgA deposition are separate entities or a common pathophysiology. Repeated renal biopsy and similar cases were helpful and should be carried out as far as possible.
.

  20. A Case of the nephrotic syndrome in bone marrow transplantation recipient, histologically showing overlapped glomerular lesions of thrombotic microangiopathy and membranous nephropathy.

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    Masuzawa, Naoko; Nishimura, Ayako; Kitani, Takashi; Tamagaki, Keiichi; Sugitani, Mio; Nagoshi, Hisao; Kuroda, Junya; Konishi, Eiichi

    2017-09-20

    Nephrotic syndrome (NS) rarely occurs in post-hematopoietic stem cell transplantation (HSCT) recipients but represents the renal manifestation of graft-versus-host disease (GVHD). Membranous nephropathy (MN) accounts for almost two thirds of post-HSCT NS and is caused by immune complex deposition. Renal thrombotic microangiopathy (TMA) without fulfillment of clinical criteria for TMA has been underreported because of reduced opportunity for histological examination. However, renal TMA has recently been reported in association with GVHD and humoral immunological reactions. Although both MN and TMA after HSCT are associated with GVHD and immunological abnormalities, these diseases are exceptionally coexistent in renal biopsy specimens. We herein describe a case of post-HSCT NS, histologically showing overlapped lesions of TMA and MN. Renal biopsy specimen after presentation of NS revealed early stage MN and TMA with evidence of chronicity. TMA was thought to have preceded MN, and renal biopsy at the phase of pre-nephrotic proteinuria might reveal earlier histological changes of isolated renal TMA. Detection of subclinical renal TMA earlier by spontaneous renal biopsy can help prevent progression of renal injury or overlapping of other renal pathologies. We also demonstrated Th2 predominant intraglomerular infiltration of lymphocytes by immunohistochemistry. © 2017 Japanese Society of Pathology and John Wiley & Sons Australia, Ltd.

  1. A case of rheumatic fever with acute post-streptococcal glomerulonephritis and nephrotic syndrome caused by a cutaneous infection with beta-hemolytic streptococci

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    Carsten Sauer Mikkelsen

    2010-01-01

    Full Text Available A middle-aged patient of Greenlandic origin was referred for skin infection of the leg. An initial minor trauma of the skin of the distal right lower extremity was complicated by bullous erysipelas which cultured positive for group A β-hemolytic streptococci (GABHS. The clinical condition deteriorated and necrotizing fasciitis developed despite relevant surgical and antibiotic treatment. Approximately 3 weeks later, the patient developed arthralgia, impaired renal function with azotemia, hypertension and severe nephrotic syndrome with periorbital and peripheral edema. A kidney biopsy demonstrated endocapillary glomerulonephritis. Concomitantly, carditis with chest pain, moderately reduced left ventricular ejection fraction and mitral regurgitation were noted. The patient had no signs of pharyngitis in the whole period. The patient thus contracted poststreptococ glomerulonephritis and furthermore she fulfilled the criteria of acute rheumatic fever following a GABHS skin infection. We suggest a possible relation between a virulent GABHS clone causing NF and ARF.

  2. Relations of nuclear factor-kappa B activity in the kidney of children with primary nephrotic syndrome to clinical manifestations, pathological types, and urinary protein excretion

    Institute of Scientific and Technical Information of China (English)

    ZHAO Hong-yang; SUN Ruo-peng; DONG Jun-hua; ZHEN Jun-hui

    2005-01-01

    @@ The pathogenesis of childhood primary nephrotic syndrome (PNS) is unclear. However, an immune mechanism has generally been accepted as a cause. Imbalance of T lymphocyte and a variety of inflammatory cytokines, chemotactic and transcription factors are involved in the pathophysiology and manifestations of PNS,1,2 and nuclear factor kappa B (NF-κB) transcriptionally regulates the expression of these factors.3 Research has been focused on NF-κB and inflammatory regulated mediators of renal diseases, but seldom on different clinical manifestations and histopathological changes. In order to explore a potential mechanism for the pathogenesis of PNS in children and a basis for preventing its advance, we determined NF-κB activity in the kidney of children with PNS in vitro using immunohistochemical staining and the multimedia coloured pathological image analysis system and its relations to clinical manifestations, histopathological changes and 24-hour urinary protein excretion.

  3. Papulonodular mucinosis, Guillain-Barré syndrome and nephrotic syndrome in a patient with systemic lupus erythematosus: a case report

    OpenAIRE

    Su, Xiaole; Qiao, Xi; Li, Jing; Gao, Lifang; Wang, Chen; WANG Lihua

    2017-01-01

    Background Awareness of the spectrum of clinical manifestations of systemic lupus erythematosus (SLE), especially uncommon changes, is essential for diagnosis and effective management of patients. Case presentation A 26-year-old Chinese man with SLE initially manifested cutaneous papulonodular mucinosis and developed acute Guillain-Barré syndrome and class V lupus nephritis 2 years later. His cutaneous nodules had not been idententified for 2 years and were resected by surgical procedures twi...

  4. Childhood Nephrotic Syndrome

    Science.gov (United States)

    ... Ira Davis, M.D.; Ann Guillott, M.D.; Steve Alexander, M.D.; Deborah Kees-Folts, M.D.; Alicia Neu, M.D.; Steve Wassner, M.D.; John Brandt, M.D.; and ... Advisory & Coordinating Committees Strategic Plans & Reports Research Areas Jobs at NIDDK FAQs Visit Us News NIDDK News ...

  5. Congenital nephrotic syndrome

    Science.gov (United States)

    ... by: Charles Silberberg, DO, private practice specializing in nephrology, affiliated with New York Medical College, Division of Nephrology, Valhalla, NY. Review provided by VeriMed Healthcare Network. ...

  6. ¿Existe un síndrome nefrótico corticosensible con lesión mínima hereditario? Is there a corticosensitive nephrotic syndrome with a hereditary minimal lesion?

    Directory of Open Access Journals (Sweden)

    Sandalio Durán Álvarez

    2008-09-01

    Full Text Available Se presenta una revisión de las características hereditarias, presentación clínica y pronóstico de los síndromes nefróticos hereditarios que se han descubierto en los últimos años. La mayoría de los estudios están relacionados con síndromes nefróticos hereditarios con lesiones hísticas variadas y mala respuesta al tratamiento. Las mutaciones más frecuentes se producen en los genes NPHS2, NPHS1, WT1 y LAMB2, que producen glomeruloesclerosis focal segmentaria, síndrome nefrótico de tipo finés (enfermedad microquística, esclerosis mesangial difusa y síndrome de Pierson. También se han descrito mutaciones en los genes alfa-actinina 4 y TRPC6, las cuales producen síndrome nefrótico con glomeruloesclerosis focal de comienzo en el adulto. Se ha demostrado la presentación de un síndrome nefrótico familiar con lesión mínima y buena respuesta a los tratamientos, que parece ser producido por algún gen que puede codificar un factor desconocido, cuyo locus es 2p12-p13.2. Se han demostrado casos de síndrome nefrótico familiar que pueden tener patrones hereditarios autosómicos dominantes o recesivos, con evolución similar a la de sus progenitores o hermanos y que se corresponden con respuesta al tratamiento del síndrome nefrótico idiopático con lesión mínima. Se necesita continuar profundizando en el estudio de estos pacientes para precisar las distintas formas de herencia y el gen o genes involucrados en este síndrome nefrótico.A review of the hereditary characteristics, clinical presentation, and prognosis of the hereditary nephrotic syndromes discovered in the last years is presented. Most of the studies deal with hereditrary nephrotic syndromes with varied tissue lesions and a poor response to treatment. The most frequent mutations are produced in NPHS2, NPHS1, WTI, and LAMB2 genes that produce focal segmental glomerulonephritis, nephrotic syndrome of the Finish type (microcystic disease, diffuse mesangial sclerosis

  7. Extending initial prednisolone treatment in a randomized control trial from 3 to 6 months did not significantly influence the course of illness in children with steroid-sensitive nephrotic syndrome.

    Science.gov (United States)

    Sinha, Aditi; Saha, Abhijeet; Kumar, Manish; Sharma, Sonia; Afzal, Kamran; Mehta, Amarjeet; Kalaivani, Mani; Hari, Pankaj; Bagga, Arvind

    2015-01-01

    While studies show that prolonged initial prednisone therapy reduces the frequency of relapses in nephrotic syndrome, they lack power and have risk of bias. In order to examine the effect of prolonged therapy on frequency of relapses, we conducted a blinded, 1:1 randomized, placebo-controlled trial in 5 academic hospitals in India on 181 patients, 1-12 years old, with a first episode of steroid-sensitive nephrotic syndrome. Following 12 weeks of standard therapy, in random order, 92 patients received tapering prednisolone while 89 received matching-placebo on alternate days for the next 12 weeks. On intention-to-treat analyses, primary outcome of number of relapses at 1 year was 1.26 in the 6-month group and 1.54 in the 3-month group (difference -0.28; 95% confidence interval (CI) -0.75, 0.19). Relative relapse rate for 6- vs. 3-month therapy, adjusted for gender, age, and time to initial remission, was 0.70 (95% CI 0.47-1.10). Similar proportions of patients had sustained remission, frequent relapses, and adverse effects due to steroids. Adjusted hazard ratios for first relapse and frequent relapses with prolonged therapy were 0.57 (95% CI, 0.36-1.07) and 1.01 (95% CI, 0.61-1.67), respectively. Thus, extending initial prednisolone treatment from 3 to 6 months does not influence the course of illness in children with nephrotic syndrome. These findings have implications for guiding the duration of therapy of nephrotic syndrome.

  8. Plasmin in nephrotic urine activates the epithelial sodium channel

    DEFF Research Database (Denmark)

    Svenningsen, Per; Bistrup, Claus; Friis, Ulla G;

    2008-01-01

    Proteinuria and increased renal reabsorption of NaCl characterize the nephrotic syndrome. Here, we show that protein-rich urine from nephrotic rats and from patients with nephrotic syndrome activate the epithelial sodium channel (ENaC) in cultured M-1 mouse collecting duct cells and in Xenopus...... laevis oocytes heterologously expressing ENaC. The activation depended on urinary serine protease activity. We identified plasmin as a urinary serine protease by matrix-assisted laser desorption/ionization time of-flight mass spectrometry. Purified plasmin activated ENaC currents, and inhibitors...... of plasmin abolished urinary protease activity and the ability to activate ENaC. In nephrotic syndrome, tubular urokinase-type plasminogen activator likely converts filtered plasminogen to plasmin. Consistent with this, the combined application of urokinase-type plasminogen activator and plasminogen...

  9. 老年肾病综合征101例临床和病理分析%Clinical and pathological analysis of 101 elderly patients with nephrotic syndrome

    Institute of Scientific and Technical Information of China (English)

    陈伟军; 陆轶君; 张晓丽; 叶志斌

    2015-01-01

    目的 了解老年肾病综合征的临床与病理分布特点. 方法 回顾性分析我院101例老年肾病综合征的临床资料,包括临床表现、病理类型、治疗效果、并发症和预后等. 结果 101例老年肾病综合征患者中,原发性和继发性肾病综合征分别为79例和22例.79例原发性肾病综合征患者中,44例有肾脏病理资料,前4位分别是膜性肾病、IgA肾病、膜增生性肾小球肾炎和局灶节段性肾小球硬化症.22例继发性肾病综合征中,糖尿病肾病17例,其次是狼疮性肾炎和显微镜下血管炎.联合应用中等剂量激素和免疫抑制剂对病理诊断明确的原发性肾病综合征患者的治疗总有效率为84.1%.原发性和继发性老年肾病综合征的主要并发症均为感染、急性肾损伤和心血管疾病. 结论 老年肾病综合征中原发性所占比例较高,原发性肾病综合征肾小球疾病病理类型以膜性肾病、IgA肾病、膜增生性肾小球肾炎较为多见,继发性肾小球疾病中以糖尿病肾病最多见.对于病理诊断明确的患者,联合应用中等剂量激素和免疫抑制剂有一定的治疗作用.%Objective To study the clinical and pathological features of nephrotic syndrome in the elderly.Methods The clinical data of 101 elderly patients with nephrotic syndrome were retrospectively analyzed,including clinical manifestations,pathological type,therapeutic effect,complications and prognosis.Results Among the 101 cases of elderly patients with nephrotic syndrome,79 cases were primary nephrotic syndrome and 22 cases were secondary nephrotic syndrome.Of the 79 cases with primary syndrome,44 cases had renal pathological data.The most common types were membranous nephropathy,IgA nephropathy,mesangial proliferative glomerulonephritis and focal segmental glomerular sclerosis.The most common types of renal pathology of the 22 cases with secondary nephrotic syndrome were diabetic nephropathy (17 cases),followed by

  10. Eficacia del tratamiento dietético en el síndrome nefrótico Effectiveness of dietetic treatment in nephrotic syndrome

    Directory of Open Access Journals (Sweden)

    A. Calleja Fernández

    2009-12-01

    Full Text Available Presentamos el caso de un paciente diagnosticado de amiloidosis primaria y síndrome nefrótico que acude a la consulta de dietoterapia. En la consulta inicial se realizó una historia nutricional que incluyó una valoración antropométrica completa, composición corporal, bioquímica completa y análisis de la ingesta. El paciente presentó un exceso de agua corporal, proteinuria, niveles disminuidos de proteínas totales, albúmina, prealbúmina y colesterol HDL y concentraciones elevadas de colesterol total, de LDL y de triglicéridos. El consumo de proteínas y sodio era superior a la recomendación. Se elaboró una dieta personalizada. Tras seis meses de dieta el paciente presentó una pérdida de peso a partir de agua, manteniendo el estado nutricional, una disminución de la proteinuria, manteniéndose la función renal y una mejora del perfil lipídico. El tratamiento dietético del síndrome nefrótico es eficaz para disminuir la proteinuria recuperar y mejorar el perfil lipídico y prevenir la desnutrición del paciente.We present the case of a male patient suffering from a primary amyloidosis and a nephrotic syndrome who came to dietotherapy clinic. In the first visit we made a nutritional record including an anthropometric evaluation, body composition, biochemistry, and food intake. The patient had an excess of body water, proteinuria, low plasma protein, albumin, prealbumin and HDL cholesterol levels, and high concentrations of total cholesterol, LDL and triglycerides. The consumption of protein and sodium was higher than the recommendation. An individualized diet was made. Six months later, his weight and the excess of body water decreased, but the fat free mass remained unchanged. The levels of albumin and prealbumin increased, the proteinuria decreased. Total cholesterol, LDL and triglycerides decreased until normal levels. Dietetary treatment in nephrotic syndrome is effective to decrease proteinuria, improve cholesterol and

  11. 80例原发性肾病综合征合并甲状腺功能减退的临床和病理分析%Clinical and Pathological Analysis for 80 Cases of Primary Nephrotic Syndrome Complicating with Hypothyroidism

    Institute of Scientific and Technical Information of China (English)

    陈晓风; 李晶; 谷小丽; 王攀

    2016-01-01

    Objective: To discuss the clinical significance of 80 patients’clinical characteristics and pathological types who have primary nephrotic syndrome complicating with hypothyroidism.Method:80 patients of primary nephrotic syndrome complicating with hypothyroidism in our hospital were selected,they all received kidney biopsy and thyroid function test(TT3,TT4,FT3,FT4 and TSH),plasma albumin,24 hours urine protein quantitative and so on,and then the pathological types and clinical characteristics were analyzed.Result:The pathological types were membranous glomerulonephritis (MGN) of 43 cases, minimal change disease (MCD) of 29 cases, mesangial proliferative glomerulonephritis (MSPGN) of 4 cases,focal segmental glomerulosclerosis (FSGS) of 2 cases, and membranoproliferative glomerulonephritis (MPGN) of 2 cases. For patients with primary nephrotic syndrome,their serum albumin had positive correlation with serum TT3, FT3,TT4,FT4, and had negative correlation with serum TSH;24 hours urine protein quantitative had negative correlation with serum TT3,FT3,TT4,FT4,and had no obvious correlation with serum TSH.Conclusion:For patients of primary nephrotic syndrome complicating with hypothyroidism,the pathological types are membranous glomerulonephritis and minimal change disease as the common.The level of thyroxin and albumin decrease have a close correlation.So, the test for thyroxin has an important reference value to judge the lesion degree,prognosis and pathological types for patients with primary nephrotic syndrome complicating with hypothyroidism.%目的:对80例原发性肾病综合征合并甲状腺功能减退患者的临床特点和病理类型进行分析,探讨其临床意义。方法:本院住院的80例原发性肾病综合征合并甲状腺功能减退患者均进行肾脏病理穿刺、检测甲状腺功能(TT3、TT4、FT3、FT4及TSH)、血浆白蛋白、24 h尿蛋白定量等,并对其病理类型及临床特点进行分析。结果:肾脏病理

  12. The Correlation Analysis of Primary Nephrotic Syndrome with Platelet Activating Factor Acetylhydrolase%原发性肾病综合征与血小板活化因子水解酶的相关性分析

    Institute of Scientific and Technical Information of China (English)

    田鲁; 胡亚琳; 操轩

    2015-01-01

    Objective:To observe the correlation of primary nephrotic syndrome ( primary nephritic syn-drome,PNS) with platelet activating factor acetylhydrolase (platelet activating factor cetylhydrolase ,PAF-AH).Method:48 patients with primary nephrotic syndrome treated in the hospital from Jan .2013 to Jun. 2013 were selected as the research object , all were initial treatment patients .They were divided into simple nephrotic syndrome group (STNS group with 31 cases) and nephritic type nephrotic syndrome group (NTNS group with 17 cases);and according to the difference in effects of hormone treatment all patients were divided sensitive nephrotic syndrome ( steroid-sensitive nephritic syndrome , SSNS ) group ( 19 cases ) , steroid re-sistant nephrotic syndrome (steroid-resistent nephritic syndrome, SRNS) group (15 cases), steroid depend-ent nephrotic syndrome group ( steroid-dependent nephritic syndrome , SDNS ) group ( 14 cases ) , then se-lected healthy persons with 30 cases as control group , 78 cases were determined the activity of PAF-AH, then compared differences between two groups .Result:PAF-AH activity in STNS group was(51.9 ±8.3)μmoL· min-1 · L-1 and was higher than those in NTNS group and healthy control group , all P<0.05; and NTNS activity in PAF-AH group were higher than that in healthy control group , all P<0.05.The activity of PAF-AH in SSNS group were higher than those in SRNS group , SDNS group and the control group , P<0.05 group, SDNS group, SRNS and PAF-AH activity were higher than the control group , all P<0.05.By the a-nalysis of perason , PAF-AH activity and primary nephrotic syndrome had obvious correlation analysis ,rSTNS=0.618;rNTNS=0.524; rSSNS=0.717; rSRNS=0.567.; rSDNS=0.327, P<0.05.Conclusion: Platelet activating factor acetylhydrolase activity of patients with nephrotic syndrome is significantly higher than the normal population, and the different types of PNS patients ’ PAF-AH activity are different.%目的:观察

  13. Nephrotic syndrome with fever and widespread subcutaneous necrosis%肾病综合征伴高热、皮下广泛坏死

    Institute of Scientific and Technical Information of China (English)

    全军肾脏病研究所学术委员会

    2012-01-01

    A 23-year-old man developed severe necrotizing cellulitis on both anterior and posterior of his trunk after kneading. The patient had been treated with corticosteroids over twenty months for nephrotic syndrome. Skin biopsy of the wound area revealed cutaneous vasculitis and chronic inflammation with yeast-like organisms. The Periodic Acid Schiff staining supported that the structures were consistent with Cryptococcosis. A Cryptococcosus neoformans infection was confirmed by culture.%青年男性,临床表现肾病综合征、血清肌酐轻度升高,肾活检确诊为IgA肾病,肾小管损伤严重;长期接受大剂量糖皮质激素联合免疫抑制剂治疗.近期泡澡、搓澡后出现大面积皮肤红肿热痛,高热;血清隐球菌抗原阳性,皮肤病理检查符合隐球菌感染,皮肤破损脓液及皮肤坏死组织培养确诊新生隐球菌感染.

  14. C5b-9-targeted molecular MR imaging in rats with Heymann nephritis: a new approach in the evaluation of nephrotic syndrome.

    Directory of Open Access Journals (Sweden)

    Qiang Huang

    Full Text Available Membranous nephropathy (MN is the major cause of adult nephrotic syndrome, which severely affects patients' quality of life. Currently, percutaneous renal biopsy is required to definitively diagnose MN. However, this technique is invasive and may cause severe complications. Therefore, an urgent clinical need exists for dynamic noninvasive monitoring of the renal state. In-depth molecular imaging studies could assist in finding a solution. Membrane attack complex C5b-9 is the key factor in the development of MN, and this protein primarily deposits in the glomerulus. The present study bound polyclonal antibodies to C5b-9 with ultrasmall superparamagnetic iron oxide (USPIO nanoparticles to obtain C5b-9-targeted magnetic resonance molecular imaging probes. The probes were injected intravenously into rats with Heymann nephritis, a classic disease model of MN. The signal intensity in the T2*-weighted imaging of kidneys in vivo using 7.0 Tesla magnetic resonance imaging decreased significantly 24 hours after injection compared to the untargeted and control groups. This signal change was consistent with the finding of nanoparticle deposits in pathological glomeruli. This study demonstrated a novel molecular imaging technique for the assessment of MN.

  15. Relationship of Tumor Necrosis Factor-α and Nitrogen Oxide with Treatment of Frequent Relapse Nephrotic Syndrome by Shenkangling(肾康灵)Granule in Children

    Institute of Scientific and Technical Information of China (English)

    王莉玲; 郑健; 曾章超; 董飞侠; 吴群励

    2003-01-01

    Objective: To observe the relationship of tumor necrosis factor-α (TNF-α) and nitrogen oxide (NO) with the treatment of frequent relapse nephrotic syndrome (FRNS) and to explore the pathogenesis of FRNS and the therapeutic mechanism of Shenkangling(肾康灵,SKL) Granule in children.Methods: Sixty children suffering from FRNS were randomly divided into the treated group and control group, 30 in each, and the other 30 healthy children were taken as healthy group. The patients were treated with prednisone for a long-term course, and those with no effect or partial effect shown were treated with additional Tripterygium or Cytoxan in the control group, while in the treated group patients were treated with prednisone and additional SKL. The two groups were compared as to their changes of TNF-α,NO before and after treatment, and the relapses after treatment. Results: The levels of TNF-α and NO in the sick children before treatment were markedly higher than those after treatment and normal group (P<0. 01). The positive correlation between TNF-α of FRNS cases and relapse risk displayed more significance than that between the relapse of FRNS and NO. The difference between treated group and control group was significant (P<0. 01). Conclusion: TNF-α can be regarded as the monitoring parameter of the active phase in FRNS, and the higher the level, the more possible the relapse would occur. SKL could markedly reduce the relapse rate of FRNS in children.

  16. Influence of nephrotic state on the infectious profile in childhood idiopathic nephrotic syndrome Pacientes com síndrome nefrótica idiopática apresentam alterações na imunidade celular e humoral que predispõem a processos infecciosos

    Directory of Open Access Journals (Sweden)

    Emilia Maria Dantas Soeiro

    2004-01-01

    Full Text Available Patients with idiopathic nephrotic syndrome present alterations in their cellular and humoral immune reactions that predispose them to the development of infectious processes. PURPOSE: To characterize the infectious processes in patients with idiopathic nephrotic syndrome. PATIENTS AND METHODS: Ninety-two children and adolescents with idiopathic nephrotic syndrome were assessed retrospectively. The types of infection were grouped as follows: upper respiratory tract infections; pneumonia; skin infections; peritonitis; diarrhea; urinary tract infection ; herpes virus; and others. The patients were divided into 2 groups: Group I (steroid-responsive n = 75, with 4 subgroups-IA (single episode n = 10, IB (infrequent relapsers n = 5, IC (frequent relapsers n = 14, and ID (steroid-dependent n = 46; and Group II (steroid-resistant n = 17. The incidence-density of infection among the patients was assessed throughout the follow-up period. Comparisons for each group and subgroup were done during the periods of negative and nephrotic proteinuria. RESULTS: The analysis revealed a greater incidence-density of infections during the period of nephrotic proteinuria in all the groups and subgroups, with the exception of subgroup IA. During the period of nephrotic proteinuria, subgroups IC, ID, and Group II presented a greater incidence-density of infections as compared to subgroup IA. For the period of negative proteinuria, there was no difference in the incidence-density of infections between the groups and subgroups. Upper respiratory tract infections were the most frequent infectious processes. CONCLUSION: The nephrotic condition, whether as part of a course of frequent relapses, steroid dependence, or steroid resistance, conferred greater susceptibility to infection among the patients with idiopathic nephrotic syndrome. The results of this study suggest that the best preventive action against infection in this disease is to control the nephrotic state

  17. 多靶点治疗激素抵抗型肾病综合征的临床研究%Clinical trial on the effect of multi-target therapy on steroid-resistant nephrotic syndrome

    Institute of Scientific and Technical Information of China (English)

    王健英; 赵军; 王臻

    2009-01-01

    Objective To investigate the clinical effect of cyclosporine(CsA) combined with leflunomide and steroid in the treatment of steroid-resistant nephrotic syndrome. Methods The 14 patients with steroid-resistant nephrotic syndrome treated with leflunomide ,CsA and prednisone.Clinical data were observed in the 2nd,4th,6th,8th,and 12th week. Results After receiving multi-target therapy ,the proteinuria was decreased significantly(P<0.01),Serum albumin was increased significantly(P< 0.01), Complete relieving rate was 64.3% ,and total effective rate was 86.7%.Conclusion Multi-target therapy with CsA,leflunomide and steroid was an effective induction therapy with mild adverse effects for steroid-resistant nephrotic syndrome.%目的 研究多种免疫抑制剂联合治疗激素抵抗型肾病综合征的临床疗效.方法 14例患者均接受小剂量环孢素A(CsA)和来氟米特联合中等剂量的强的松治疗,在治疗后第2、4、6、8、12周动态监测各项临床指标.结果 24 h尿蛋白定量较治疗前明显下降,血浆白蛋白在治疗后明显升高,完全缓解率为64.3%,总有效率为86.7%.结论 多靶点治疗激素抵抗型肾病综合征有显著疗效,并且不良反映少,安全性较高.

  18. Effect of extended care on self-care abilities in patients with nephrotic syndrome%延续护理对肾病综合征自我护理能力的影响

    Institute of Scientific and Technical Information of China (English)

    阮柳红; 黎华; 伍慧贤

    2012-01-01

    Objective To observe the effect of extended care on self-care ability in patients with nephrotic syndrome.Methods 82 discharged patients with nephrotic syndrome were randomly divided into study group and control group.The study group received extended care.Rate of awareness of recurrence prevention,self-care abilities,and recurrence rate were compared between the two groups.All the patients were followed up for one year.Results The rate of awareness of recurrence prevention and self-care abilities were significantly greater and the recurrence rate was markedly lower in the study group than in the control group.Conclusions Extended care has a marked effect on self-care abilities in patients with nephrotic syndrome,resulting in a decrease in the recurrence rate.%目的 观察延续护理对肾病综合征自我护理能力的影响.方法 选择出院的肾病综合征患者82例并随机分为实验组和对照组,实验组增加出院后延续护理,比较两组的预防复发知识知晓率、自我护理能力及复发率,调查时间为1年.结果 实验组的预防复发知识知晓率及自我护理能力显著高于对照组(P< 0.01),复发率明显低于对照组(P<0.01),两组比较差异有显著性.结论 延续护理对出院肾病综合征患者的自我护理能力有显著影响,从而减低复发率.

  19. The influence of health education on anxiety emotion of parents of primary nephrotic syndrome children%健康教育对肾病综合征患儿家长焦虑情绪的影响

    Institute of Scientific and Technical Information of China (English)

    姚霞; 李小洁; 姚志红

    2009-01-01

    目的 探讨健康教育对肾病综合征患儿家长焦虑情绪的影响.方法 98例确诊为肾病综合征患儿的家长随机分为实验组48例及对照组50例,对照组患儿给予常规治疗及一般护理.实验组在对照组基础上给予健康教育.观察两组患儿家长焦虑情绪改变.结果 实验组肾病综合征息儿家长经健康教育后其焦虑情绪较对照组明显减轻P<0.01.结论 健康教育可有效减轻肾病综合征患儿家长的焦虑情绪,有利于肾病综合征患儿病情康复.%Objective To investigate the influence of health education on anxiety emotion of parents of primary nephrotic syndrome children.Methods 98 cases were randomly divided into two groups.The control group was given routine treatment and nursing,and the experimental group was additional given health education.The anxiety emtion of the two groups were observed.Results The anxiety emtion in the experimental group was distinctly decreased than that of control group(P <0.01).Conclusions Health education could efficiently decrease the anxiety emotion of parents of primary nephrotic syndrome children and is beneficial for the rehabilitation of the children with primary nephrotic syndrome.

  20. The Discussion of Genetic Research and Diagnosis of Nephrotic Syndrome%对遗传性肾病综合征的基因诊断研究与讨论

    Institute of Scientific and Technical Information of China (English)

    刘俊泽

    2015-01-01

    Study and discuss genetic diagnosis of hereditary nephrotic syndrome. Target sequence capture using genetic sequencing technology nephropathy diagnosis and treatment of disease. Regimen hereditary nephrotic syndrome is more complex, while the conventional regimen no significant effects, such as hormone therapy and immunosuppressive hair hair, so the definition of the class of diseases, molecular genetics classification, clinical features, and kidneys pathology and other related developments need to be understood and clear. Target sequence capture using sequencing techniques, can make a one-time NS for genetic testing, diagnosis and treatment. Using molecular biological methods for the diagnosis of hereditary kidney disease, help biology, clinical and pathological features and other common molecular genetic study of nephrotic syndrome, to establish genetic nephrotic syndrome diagnostic thinking.%研究讨论遗传性肾病综合征的基因诊断。采用靶序列捕获测序技术对遗传性肾病的病症进行诊断与治疗。遗传性肾病综合征的治疗方案较为复杂,而常规的治疗方案没有明显的效果,如激素和免疫抑制剂。所以,对该类疾病的定义、分子遗传学分类、临床特征以及肾脏病理等相关进展需要进行理解和明确。采用靶序列捕获测序技术,可以一次性对遗传性NS进行检测、诊断和治疗。采用靶序列捕获测序技术对遗传性肾病进行诊断,可以帮助对遗传性肾病综合征的常见分子生物学、临床及病理特征等进行研究,并且为建立遗传性肾病综合征的基因提供诊断依据。

  1. Reversible Renal Failure in Hypertensive Idiopathic Nephrotics Treated with Captopril

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    Olowu Wasiu

    2006-01-01

    Full Text Available Angiotensin converting enzyme inhibitor (ACEI- induced acute renal failure (ARF is not as commonly reported in children as in adults. We report two cases of idiopathic nephrotic syndrome that developed ARF following captopril (an ACEI treatment for prednisolone-induced hypertension. The two cases further alert us to the potential risk of ACEI-induced ARF in any nephrotic child on ACEI treatment. Low or high dose ACEIs should be given with extreme caution in active nephrotics in view of their relative hypovolemic state that may provoke ARF. The nephrotic children, who must be treated with ACEIs with or without diuretics, should be closely monitored for the development of ARF during the use of ACEIs.

  2. Hipotiroidismo, miocardiopatía dilatada y síndrome nefrótico durante el embarazo Hypothyroidism, dilated cardiomyopathy and nephrotic syndrome during pregnancy

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    Ariel K. Saad

    2011-02-01

    Full Text Available El hipotiroidismo en el embarazo es infrecuente, pero cuando ocurre suele asociarse con complicaciones maternas y fetales. Se presenta el caso de una mujer joven sin antecedentes de enfermedad cardiovascular que consulta por ortopnea, dolor torácico y edema de miembros inferiores. Los exámenes pusieron en evidencia la existencia de insuficiencia cardíaca, hipotiroidismo, síndrome nefrótico e insuficiencia renal. El eco-Doppler mostró dilatación de las cuatro cavidades cardíacas con deterioro grave de la función sistólica. El tratamiento con levotiroxina por vía intravenosa mejoró el cuadro clínico y los parámetros de laboratorio. Se analizan los efectos de la hormona tiroidea sobre el aparato cardiovascular y se comentan los mecanismos fisiopatológicos de la insuficiencia cardíaca en el embarazo.Hypothyroidism during pregnancy is infrequent, but its presence is associated with maternal and fetal complications. We present the case of a young pregnant woman with no previous history of cardiovascular disease, who consulted for orthopnea, chest pain and edema in both legs. Laboratory tests demonstrated a hypothyroid condition and a nephrotic syndrome with renal failure. The echo-Doppler exam showed a four chamber dilatation with systolic dysfunction. Treatment with intravenous levothyroxine improved her medical condition. We analyze the effects of thyroid hormone on the heart and vascular system and discuss the pathophysiologic mechanisms of heart failure during pregnancy.

  3. Urinary IgG and α2-Macroglobulin Are Powerful Predictors of Outcome and Responsiveness to Steroids and Cyclophosphamide in Idiopathic Focal Segmental Glomerulosclerosis with Nephrotic Syndrome

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    Claudio Bazzi

    2013-01-01

    Full Text Available Objective. To assess whether high-molecular-weight proteins excretion predicts outcome and therapy-responsiveness in patients with FSGS and nephrotic syndrome. Research Design and Methods. Thirty-eight patients measured at biopsy fractional excretion of IgG (FEIgG and urinary α2-macroglobulin/creatinine ratio (α2m/C. Low and high risk groups were defined by cutoffs assessed by ROC analysis. In all patients first-line therapy was with steroids alone or in combination with cyclophosphamide. Results. α2m/C and FEIgG were correlated with segmental sclerosis (r=0.546; r=0.522. Twenty-three patients (61% entered Remission and 9 (24% progressed to ESRD. Comparing low and high risk groups, by univariate analysis remission was predicted by FEIgG (77% versus 25%, P=0.016 and α2m/C (81% versus 17%, P=0.007 and ESRD at best by FEIgG (0% versus 75%, P<0.0001 and α2m/C (4% versus 67%, P<0.0001. By multivariate analysis FEIgG was the only independent predictor of remission and α2m/C the most powerful predictor of ESRD. Low and high risk groups of FEIgG and α2m/C in combination had very high predictive value of sustained remission and ESRD in response to therapy. Conclusions. FEIgG and α2m/C are powerful predictors of outcome and responsiveness to steroids and cyclophosphamide; their predictive value, if validated in prospective studies, may be useful in clinical practice suggesting first-line alternative treatments in high risk patients.

  4. Steroid-resistant idiopathic nephrotic syndrome in children: long-term follow-up and risk factors for end-stage renal disease

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    Alberto Zagury

    2013-09-01

    Full Text Available INTRODUTION: Steroid resistant idiopathic nephrotic syndrome (SRINS in children is one of the leading causes of progression to chronic kidney disease stage V (CKD V/end stage renal disease (ESRD. OBJECTIVE: The aim of this retrospective study is to evaluate the efficacy of immunosuppressive drugs (IS and to identify risk factors for progression to ESRD in this population. METHODS: Clinical and biochemical variables at presentation, early or late steroid resistance, histological pattern and response to cyclosporine A (CsA and cyclophosfamide (CP were reviewed in 136 children with SRINS. The analyzed outcome was the progression to ESRD. Univariate as well as multivariate Cox-regression analysis were performed. RESULTS: Median age at onset was 5.54 years (0.67-17.22 and median follow up time was 6.1 years (0.25-30.83. Early steroid-resistance was observed in 114 patients and late resistance in 22. Resistance to CP and CsA was 62.9% and 35% respectively. At last follow-up 57 patients reached ESRD. The renal survival rate was 71.5%, 58.4%, 55.3%, 35.6% and 28.5% at 5, 10, 15, 20 and 25 years respectively. Univariate analysis demonstrated that older age at onset, early steroid-resistance, hematuria, hypertension, focal segmental glomerulosclerosis (FSGS, and resistance to IS were risk factors for ESRD. The Cox proportional-hazards regression identified CsAresistance and FSGS as the only predictors for ESRD. CONCLUSION: Our findings showed that CsA-resistance and FSGS were risk factors for ESRD.

  5. The Usefulness of Determining Neutrophil Gelatinase-Associated Lipocalin Concentration Excreted in the Urine in the Evaluation of Cyclosporine A Nephrotoxicity in Children with Nephrotic Syndrome

    Directory of Open Access Journals (Sweden)

    Ewa Gacka

    2016-01-01

    Full Text Available Introduction. The use of cyclosporine (CsA in the treatment of nephrotic syndrome (NS contributed to a significant reduction in the amount of corticosteroids used in therapy and its cumulative side effects. One of the major drawbacks of CsA therapy is its nephrotoxicity. Prolonged CsA treatment protocols require sensitive, easily available, and simple to measure biomarkers of nephrotoxicity. NGAL is an antibacterial peptide, excreted by cells of renal tubules in response to their toxic or inflammatory damage. Aim of the Study. The aim of this study was to assess the suitability of the NGAL concentration in the urine as a potential biomarker of the CsA nephrotoxicity. Material and Methods. The study was performed on a group of 31 children with NS treated with CsA. The control group consisted of 23 children diagnosed with monosyptomatic enuresis. The relationship between NGAL excreted in urine and the time of CsA treatment, concentration of CsA in blood serum, and other biochemical parameters was assessed. Results. The study showed a statistically significant positive correlation between urine NGAL concentration and serum triglycerides concentration and no correlation between C0 CsA concentration and other observed parameters of NS. The duration of treatment had a statistically significant influence on the NGAL to creatinine ratio. Conclusions. NGAL cannot be used alone as a simple CsA nephrotoxicity marker during NS therapy. Statistically significant correlation between NGAL urine concentration and the time of CsA therapy indicates potential benefits of using this biomarker in the monitoring of nephrotoxicity in case of prolonged CsA therapy.

  6. Age and geader in pathological typing of 1116 children with nephrotic syndrome%儿童肾病综合征病理类型与年龄、性别的关系

    Institute of Scientific and Technical Information of China (English)

    任献国; 刘光陵; 夏正坤; 高远赋; 樊忠民; 何旭; 茅松

    2011-01-01

    目的 探讨肾病综合征患儿的肾脏病理和性别、年龄分期的关系.方法 对1 116例经肾穿刺活检明确肾脏病理的原发性肾病综合征患儿临床资料进行回顾性分析.结果 1 116例患儿中男817例,女299例,男女比例2.73:1;平均年龄(7.3 ± 3.3)岁,其中婴幼儿期90例,学龄前期294例,学龄期409例,青春期323例.肾脏病理轻微病变(MCNS)222例,占19.9%;系膜增生性肾小球肾炎(MsPGN)726例,占65.1%;膜增生性肾小球肾炎(MPGN)55例,占4.9%;膜性肾小球肾炎(MN)27例,占2.4%;局灶节段硬化性肾小球肾炎(FSGS)86例,占7.7%.肾脏病理类型在4个年龄分期的分布差异有统计学意义,MCNS患儿以学龄前期最多,MsPGN患儿以学龄期最多,MPGN和MN患儿以青春期最多,FSGS的患儿以学龄期最多.MCNS患儿的男女比例为5.3:1,MsPGN为2.4:1,MPGN为0.96:1,MN为3.5:1,FSGS为2.7:1,肾脏病理类型在性别上的分布差异有统计学意义(P < 0.05).结论 对于无法开展肾活检的医院或有肾穿刺禁忌证的患儿,可根据年龄和性别结合临床检验初步推断肾脏病理变化的轻重,进一步指导治疗、判断预后.%Objective Renal pathological types of 1116 children suffering from primary nephrotic syndrome from March 1992 to March 2010 in Nanjing General Hospital of Nanjing Military Command were reviewed. Relationship between renal pathology and age or gender in these children was analyzed. Methods Renopuncture was performed to 1 116 children with primary nephrotic syndrome to identify pathological types. According to the age, those children were divided into four groups: infancy, pre-school age, school age and adolescence. Different distribution of renal pathological types among age stage and gender were analyzed. Results Of total 1 116 patients, 817 were boys and 299 were girls (M:F=2.73:1). The mean age was (7.3±3.3) yrs. Ninety cases were infants, 294 cases were pre-school age,409 cases were school age, 323 cases were

  7. 汞中毒致大鼠肾病综合征模型的建立%The model of nephrotic syndrome induced by mercury intoxication in rats

    Institute of Scientific and Technical Information of China (English)

    陈子安; 隋昕; 聂志勇; 李万华; 邱泽武; 王永安

    2015-01-01

    Objective The present study aims to establish a model of nephrotic syndrome induced by mercury intoxication, in order to provide stable and scientific platform for clinical relevant treatment.Methods Twenty-four male BN rats were randomly divided into four groups, including three model groups( n=18) and a control group( n=6) .The rats in the three model groups were given respectively mercuric chloride solution by subcutaneous injection of 0.5, 1.0 and 2.0 mg/kg and the control group was given a subcutaneous injection of saline, all the rats were given once every other day. By monitored the urine protein, blood biochemical indicators, mercury pontent, pathological section after injected for 7, 14, 21, 28, 35 days, we identified 1.0 mg/kg was the right dose to establish the model. Another 24 rats were randomly divided into 3 groups, according to the above method the including 18 rats were all injected in 1 mg/kg mercury chloride, the other 6 rats were for the control group.On day 15, 22 and 36 the rats were sacrificed and rat kidneys were sampled to monitor the mercury content in order to observe the accumulation of mercury in the kidney.Results Fourteen days after mercury injection, high proteinuria, low albumin, hyperlipidemia and edema occurred in three model groups, and obvious pathological change of kidney damage was also shown in three model groups.Mercury content in blood and urine showed there was obvious dose -effect relationship.Compared with the control group, the related symptoms were the most prominent and stable with 1mg/kg dose group.There was a typical pathological types with nephrotic syndrome under light microscope with 1 mg/kg dose group and mercury accumulation in kidney tissues after stopped the injection.Conclusion It may be a reliable model of nephrotic syndrome induced by mercury intoxication in BN rats, which are injected subcutaneously with 1 mg/kg mercuric chloride solution once every other day for two weeks.%目的:建立汞中毒致

  8. Mechanisms of hypercoagulability and thromboembolism in pediatric primary nephrotic syndrome%儿童原发性肾病综合征并发高凝状态与血栓栓塞的机制

    Institute of Scientific and Technical Information of China (English)

    张莹

    2012-01-01

    高凝状态和血栓栓塞在原发性肾病综合征(PNS)中发生率很高,且其形成机制颇有争议,包括内皮损伤,血小板活化,凝血功能和抗凝血功能失衡,以及肾病综合征(NS)导致的蛋白尿、低蛋白血症、高胆固醇血症等.近年来,大规模临床病例对照研究进一步阐释了NS患者高凝状态与血栓栓塞的机制.%Patients with the nephrotic syndrome are at increased risk of developing hypercoagulable states and thromboembolism, the most common of which is renal vein thrombosis. There are several unanswered or controversial issues relating to the mechanisms of the hypercoagulability and thromboembolism in the nephrotic syndrome which include endothelial dysfunction, platelet activation, functional disequilibrium between coagulation and anticoagulation, and proteinuria, hypoproteinemia, hypercholesterolemia caused by NS. In the recent years, the mechanisms have been updated by many case-control studies.

  9. The clinical significance of gene polymorphism in children's primary nephrotic syndrome%基因多态性在儿童原发性肾病综合征中的临床意义

    Institute of Scientific and Technical Information of China (English)

    陈娟

    2010-01-01

    儿童原发性肾病综合征(PNS)的发病机制目前尚未完全明确.近年来研究显示,某些基因如血管紧张素转换酶基因、载脂蛋白E基因、血小板活化因子水解酶基因、人类白细胞抗原基因、糖皮质激素受体基因、细胞因子基因等的多态性与PNS的易患性、病理进展、激素疗效、疾病复发及预后明显相关,该文就与PNS有关的基因多态性的研究进展作一综述.%The pathogenesis of children's primary nephrotic syndrome has not been completely clear at present.In the past few years,The polymorphism of these genes,such as angiotensin-converting enzyme gene,apolipoprotein E gene,platelet-activating factor acetylhydrolase gene,human histocompatibility leukocyte antigen gene,glucocorticoid receptor gene and cytokine gene,ect have been discovered that were significantly correlated with susceptibility,pathological progress,steroid response,disease recurrence and prognosis of primary nephrotic syndrome.This article reviewed the research progress of PNS and gene polymorphism.

  10. 糖皮质激素对原发性肾病综合征患者TNFα的影响%Effect of corticosteroids on TNFα level in patients with primary nephrotic syndrome

    Institute of Scientific and Technical Information of China (English)

    刘晓渭; 李冬梅; 李元勋; 赵正辉; 徐月清

    2001-01-01

    目的观察原发性肾病综合征(PNS)患者应用糖皮质激素(以下简称激素)前后血、尿TNFα变化. 方法检测了40例PNS患者激素治疗前及用激素后1,2,4,8 wk血、尿TNFα变化,并与20例正常人血、尿TNFα进行比较. 结果 PNS患者40例治疗前血、尿TNFα明显增高(血正常(68±19)(kU·L-1),PNS (461±362)(kU·L-1),尿正常0/kU·L-1 PNS (202±147)(kU·L-1)(P0.05). CONCLUSION TNFα is involved in invasion and disease progression of primary nephrotic syndrome. The effect and mechanisms of corticosteroids are correlated with the inhibition in the production of TNFα. Plasma and urine levels of TNFα can serve as an index of the effect of corticosteroids on patients with primary nephrotic syndrome.

  11. [A case of advanced esophageal carcinoma with nephrotic syndrome completely responding to chemotherapy of docetaxel, nedaplatin and 5-fluorouracil].

    Science.gov (United States)

    Matsutani, Takeshi; Uchida, Eiji; Yoshida, Hiroshi; Suzuki, Seiji; Maruyama, Hiroshi; Yokoyama, Tadashi; Matsushita, Akira; Hirakata, Atsushi; Kawamoto, Masao; Arai, Hiroki; Umakoshi, Michinobu; Wakabayashi, Hideyuki; Sasajima, Koji

    2011-03-01

    A 78-year-old male was admitted to our hospital because of dysphagia. He had been diagnosed as nephritic syndrome at 30 years of age and had been treated with prednisolone 10 mg/day. Blood examination revealed renal dysfunction; BUN 25 mg/dL, Cr 1. 9 mg/dL, and glomerular filtration rate(GFR)47. 4 mL/min. Endoscopy showed a type 2 tumor at the middle thoracic esophagus, and the biopsy specimen revealed moderately differentiated squamous cell carcinoma pathologically. Computed tomography (CT) of the chest and abdomen showed no metastases at distant regions and lymph nodes. Clinical staging was Stage II (cT2cN0cM0). Because of old age and renal function, we chose chemotherapy using docetaxel, nedaplatin and 5-fluorouracil. The adverse event was grade 2 in leucopenia and grade 1 in inappetence, but the renal function did not progress. Repeated endoscopic examinations after chemotherapy revealed that the esophageal cancer was significantly reduced in size, and no cancer cells were pathologically detected by endoscopic biopsy, resulting in a complete response(CR). This chemotherapy of docetaxel, nedaplatin and 5-fluorouracil might be effective and tolerable for patients with renal dys- function due to nephritic syndrome.

  12. Long versus standard initial steroid therapy for children with the nephrotic syndromeA report from the Southwest Pediatric Nephrology Study Group.

    Science.gov (United States)

    Lande, Marc B; Gullion, Christina; Hogg, Ronald J; Gauthier, Bernard; Shah, Binod; Leonard, Mary B; Bonilla-Felix, Melvin; Nash, Martin; Roy, Shane; Strife, C Frederic; Arbus, Gerald

    2003-04-01

    A retrospective cohort study was conducted by the Southwest Pediatric Nephrology Study Group (SPNSG) to address whether a longer initial course of corticosteroids in patients with idiopathic nephrotic syndrome (INS) provides superior protection against relapse without increased adverse effects. In order to be included in the evaluation, patients with INS must have responded to an initial steroid course, either standard or long regimen as defined here, and completed at least 1 year of follow-up. The standard regimen consisted of prednisone 2.0+/-0.3 mg/kg per day or 60+/-10 mg/m(2) per day for 28+/-4 days, followed by alternate-day prednisone for 4-12 weeks. The long regimen consisted of daily prednisone 2.0+/-0.3 mg/kg per day or 60+/-10 mg/m(2) per day for 42+/-6 days, followed by alternate-day prednisone for 6-14 weeks. The primary outcome measure was relapse of NS within 12 months of discontinuing the initial course of prednisone. There were 151 children who met the criteria for the study; 82 received the standard regimen and 69 the long regimen. The two groups did not differ in age, race, blood pressure, serum albumin, or serum cholesterol prior to the initial steroid course. The cumulative prednisone dose was 49% higher in the long regimen group than in the standard regimen group. Relapse within 12 months was reported in 72.5% of patients who received the long regimen versus 84.1% of those who received the standard regimen. The odds ratio for relapse within 12 months was 0.496 (95% confidence interval 0.22, 1.088), long versus standard regimen. This did not reach statistical significance ( chi(2)=3.058, P=0.08). The odds ratio of experiencing at least one side effect was 3.76, long relative to standard regimen ( n=133, P<0.001). Our data suggest that prolongation of the steroid treatment for the initial episode of steroid-sensitive NS may have a beneficial effect, but at the cost of increased side effects. However, definitive conclusions are limited by the

  13. Minimal change disease: an unusual presentation of marginal zone MALT lymphoma.

    Science.gov (United States)

    Inayat, Faisal; Law, Jason K; Myers-Gurevitch, Patricia M; Seshan, Surya V; Perlman, Alan S

    2016-03-01

    Minimal change disease (MCD) in association with low-grade extra-nodal marginal zone B-cell lymphoma (MZL) of mucosa-associated lymphoid tissue (MALT) (MALT lymphoma) is a rare clinicopathologic entity. We report a 68-year-old male who presented with nephrotic range proteinuria as the first manifestation of underlying MZL, confirmed with standard set of investigations. Being a steroid non-responder, he was treated with rituximab demonstrating a marked response with resolution of proteinuria. However, he relapsed after 3 months. Upon relapse, a combination of rituximab and bendamustine (R-Benda) was initiated achieving sustained resolution of proteinuria. No additional treatment was administered and the proteinuria has remained in remission for over a year.

  14. Class Ⅱ lupus nephritis with nephrotic syndrome%伴肾病综合征Ⅱ型狼疮性肾炎的临床病理特征

    Institute of Scientific and Technical Information of China (English)

    刘瑾; 陈惠萍; 陈浩; 曾彩虹; 刘正钊; 章海涛; 刘志红; 胡伟新

    2012-01-01

    目的:比较临床表现肾病综合征(NS)和非NS的Ⅱ型狼疮性肾炎(LN)患者的临床、病理特征和预后. 方法:回顾性分析经肾活检病理确诊为Ⅱ型LN患者124例,其中女性112例,男性12例,平均年龄29.2±11.4岁,根据尿蛋白定量分为NS组(尿蛋白定量≥3 g/d,血清白蛋白<30 g/L)和非NS组(尿蛋白定量<3g/d,血清白蛋白≥30 g/L,伴或不伴镜下血尿).比较两组患者的临床、免疫学指标、肾脏病理特征及预后. 结果:124例Ⅱ型LN中NS组27例(21.8%),非NS组97例(78.2%),两组患者性别、发病年龄和病程无差异.NS组以肾损害为首发症状(77.8%vs15.5%,P<0.01)及病程中出现急性肾损伤的比例(29.6%vs0,P<0.01)均显著高于非NS组,而皮疹(40.7% vs 69.1%,P<0.01)、发热(14.8%vs62.9%,P<0.01)和关节炎(29.6%vs75.3%,P<0.01)的发生率及血清抗-dsDNA阳性率(29.6%vs52.6%,P<0.05)明显低于非NS组.肾活检病理NS组患者肾小球中重度系膜增生的比例显著低于非NS组(7.4%vs59.8%,P<0.01),电镜下肾小球足细胞足突广泛融合的比例显著高于非NS组(88.9%vs0,P<0.01).NS组和非NS组激素治疗均获得高缓解率(100%vs98.4%,P=0.882),但NS组复发率显著高于非NS组(69.9%vs33.3%,P<0.01),两组分别随访8~125月(中位时间55月)和6~274月(中位时间57月),均未发生终末期肾病.7例复发者重复肾活检,NS组2例病理类型均未转型,非NS组5例均发生转型. 结论:表现为NS的Ⅱ型LN其本质为足细胞病,非NS的Ⅱ型LN为系膜增生性病变,两者临床表现、免疫学异常及病理转型的显著差异表明两者的发生机制不同,狼疮足细胞损伤的机制有待进一步研究.%Objective: To investigate the clinical characteristics, renal histological features and the prognosis in patients of Class Ⅱ LN with nephrotic syndrome. Methodology;One hundred twenty four cases(112 females, 12 males, mean age 29. 22 ± 11

  15. In silico analysis of functional nsSNPs in human TRPC6 gene associated with steroid resistant nephrotic syndrome.

    Science.gov (United States)

    Joshi, Bhoomi B; Koringa, Prakash G; Mistry, Kinnari N; Patel, Amrut K; Gang, Sishir; Joshi, Chaitanya G

    2015-11-01

    The aim of the present study is to identify functional non-synonymous SNPs of TRPC6 gene using various in silico approaches. These SNPs are believed to have a direct impact on protein stability through conformation changes. Transient receptor potential cation channel-6 (TRPC6) is one of the proteins that plays a key role causing focal segmental glomerulosclerosis (FSGS) associated with the steroid-resistant nephritic syndrome (SRNS). Data of TRPC6 was collected from dbSNP and further used to investigate a damaging effect using SIFT, PolyPhen, PROVEAN, and PANTHER. The comparative analysis predicted that two functional SNPs "rs35857503 at position N157T and rs36111323 at position A404V" showed a damaging effect (score of 0.096-1.00).We modeled the 3D structure of TRPC6 using a SWISS-MODEL workspace and validated it via PROCHECK to get a Ramachandran plot (83.0% residues in the most favored region, 12.7% in additionally allowed regions, 2.3% in a generously allowed region and 2.0% were in a disallowed region). QMEAN (0.311) and MUSTER (10.06) scores were under acceptable limits. Putative functional SNPs that may possibly undergo post-translation modifications were also identified in TRPC6 protein. It was found that mutation at N157T can lead to alteration in glycation whereas mutation at A404V was present at a ligand binding site. Additionally, I-Mutant showed a decrease in stability for these nsSNPs upon mutation, thus suggesting that the N157T and A404V variants of TRPC6 could directly or indirectly destabilize the amino acid interactions causing functional deviations of protein to some extent.

  16. 饮食营养干预在肾病综合征患者护理中的疗效评估%Evaluation on the Efficacy of Diet Intervention in Nursing Care of Patients With Nephrotic Syndrome

    Institute of Scientific and Technical Information of China (English)

    陈秀芹

    2015-01-01

    目的:分析肾病综合征患者接受饮食营养干预护理的效果。方法2012~2014年我院接收了60例肾病综合征患者,将这些患者分成对照组和实验组,均有30例,对照组采取常规药物治疗,实验组增加营养干预,对两组的临床护理情况进行比较分析。结果实验组患者接受营养干预后,其尿蛋白水平要比对照组低,两组的该项结果比较存在统计学差异,P<0.05。结论肾病综合征患者接受常规治疗和营养干预能够对患者的尿蛋白、血清蛋白等指标进行改善,提升治疗效果,对患者的身体免疫能力增强起到了很大的提升。%Objective To study the efficacy on patients with nephrotic syndrome under diet nutrition intervention. Methods From 2012 to 2014 in our hospital received 60 cases of patients with nephrotic syndrome,these patients were divided into the control group and the experimental group,in al 30 cases, the control group with conventional drug treatment,the experimental group with nutrition intervention,comparing and analysis of clinical nursing care between two groups. Results After the experimental group received nutritional intervention,level of the urine protein was lower than the control group, there were statistical difference,P<0.05. Conclusion Conventional therapy and nutrition intervention received by patients with nephrotic syndrome can improve their urinary protein,serum protein in patients with other indicators,improve the therapeutic effect,body's immune ability of patients.

  17. Investigation and analysis of subjective quality of life in children with primary nephrotic syndrome%原发性肾病综合征患儿主观生活质量的调查分析

    Institute of Scientific and Technical Information of China (English)

    李君丽; 韦琴

    2013-01-01

    Objective To evaluate the quality of life in children with primary nephrotic syndrome and analyze the influencing factors of the quality of life of the population.Methods The quality of life in children with primary nephrotic syndrome was assessed with Inventory of Subjective Life Quality for Children and Adolescent(ISLQ) edited by CHENG Zao-huo and compared to 55 normal children.The influencing factors were analyzed with multi-linear Logistic regression analysis.Results The total degree of satisfaction,recognition and emotion satisfaction of life of quality in patient children were significantly lower than those in healthy children.The data in each satisfaction degree in patient children were lower than those in healthy children,thereinto,the degree of satisfaction in score of home life,school life,depression practice and anxiety practice,self-cognition in patient children were significantly lower than those in healthy children.Conclusions The quality of life in patient children were significantly lower than that in healthy children,they should accept the comprehensive treatment intervention.%目的 评估原发性肾病综合征(primary nephrotic syndrome,PNS)患儿的生活质量,并分析该人群生活质量的影响因素.方法 采用程灶火等编制的儿少主观生活质量问卷(ISLQ)对58例PNS患儿(病例组)的生活质量进行评定,并与55名正常同龄儿童(对照组)进行对比,采用多元线性回归分析其影响因素.结果 病例组患儿的生活质量总体满意度、认知满意度和情感满意度均明显低于对照组,差异有统计学意义.各维度满意水平均低于对照组,其中家庭生活、学校生活、自我认识、焦虑体验、抑郁体验维度得分比较差异有统计学意义.家庭经济状况影响患儿的生活状况,二者成正相关关系.结论 PNS患儿的生活质量明显低于正常儿童,应对PNS患儿进行综合的干预治疗和护理.

  18. Observation on Efficacy of Astragalus Injection in Treating Diabetic Nephrotic Edema

    Institute of Scientific and Technical Information of China (English)

    胡仲仪; 刘迟; 陈以平; 沈玲妹; 金亚明

    2002-01-01

    @@ At the mid-late stage of diabetic nephropathy massive proteinuria, hypoproteinemia and anasarca would develop which is nephrotic syndrome. Diuretics are often insensitive and treatment is very difficult. From January 1995 to December 1998, the authors adopted intravenous dripping infusion of Astragalus injection (AI) for patients with nephrotic syndrome due to diabetic nephropathy (DN). The control group was treated by Salvia injection (SI). The result was that the group treated by AI showed obvious diuretic and detumescent effect.

  19. Urinary Tract Infections in Children with Primary Nephrotic ...

    African Journals Online (AJOL)

    user1

    BACKGROUND: The occurrence of urinary tract infection. (UTI) in children with Nephrotic syndrome (NS) has been widely reported by various workers, ... 24hours, hypoproteinemia- serum protein. <2g/dl and .... urinary tract from the urethra to the kidney,. AGN affect just the .... with severe protein energy malnutrition in Ilorin.

  20. 原发性肾病综合征合并甲状腺功能异常患者临床资料分析%Analysis of Clinical Data in Primary Nephrotic Syndrome Accompanied with Thyroid Dysfunction

    Institute of Scientific and Technical Information of China (English)

    申万琴; 吴歌; 赵占正; 靳云凤; 赵志红

    2011-01-01

    分析原发性肾病综合征合并甲状腺功能异常患者的临床特征,探讨其临床意叉.对37例原发性肾病综合征合并甲状腺功能异常患者的临床资料进行回顾性分析,治疗前检测甲状腺过氧化物酶抗体(Anti-TPO)、甲状腺球蛋白抗体(Anti-TG),并分别于治疗前后检测血清游离T3(FT3)、血清游离甲状腺激素(FT4)、促甲状腺激素(TSH)、及24小时尿蛋白量、血浆白蛋白,总胆固醇、甘油三酯.37例原发性肾病综合征合并甲状腺功能异常患者中,单纯FT3降低19例,占51.4%,单纯FT4降低7例,占18.9%,FT3降低及TSH升高伴Anti-TPO轻度升高2例,占5.4%;所分析患者中均无甲状腺功能减退的临床表现;血清FT3水平降低与24小时尿蛋白量呈负相关,与血浆白蛋白呈正相关,与总胆固醇、甘油三酯无明显相关性.原发性肾病综合征患者可出现FT3、FT4、TSH异常,且随病情改善而改善;甲状腺功能检测可能有助于判断原发性肾病综合征患者病情及预后.%To investigate the clinical significance, climcal features of patients with primary nephrotic syndrome accompanied with thyroid dysfunction were analysed. Thirty seven patients with primary nephrotic syndrome accompanied with thyroid dysfunction were selected in this retrospective study. Levels of FT3 , FT4 ,TSH, 24h proteinuria quantitative, serum albumin, total cholesterol, triglyceride were detected before and after treatment,while Anti-TPO and Anti-TG were detected before treatment. Decreased level of FT3 was detected in 19 cases(51.4%), decreased level of FT4 was detected in 7 cases (18.9%) and decreased level of FT3 accompanied with increased levels of TSH and Anti-TPO occured in 2 cases( 5.4 % ). None of the patients presented clinical manifestations of hypothyroidism. Serum level of FT3 was positively correlated with serum albumin but negatively correlated with urinary protein. No correlation was found between serum thyroid hormone

  1. 浅析腹水浓缩回输治疗肾病综合征顽固性腹水的临床观察探讨%Analysis of Ascites Reinfusion Treatment of Nephrotic Syndrome Refractory Aascites

    Institute of Scientific and Technical Information of China (English)

    陈涛; 马涛

    2014-01-01

    目的:浅析腹水浓缩回输治疗肾病综合征顽固性腹水的临床观察探讨。方法2000年1月~2004年5月我科利用血液透析机进行腹水浓缩后再回输,治疗肾病综合征顽固性腹水患者32例。结果所有病员在治疗后腹水逐渐减退,尿量增加,双下肢水肿减轻、精神好转,食欲增加,体力增强,未出现发热、心慌及其他症状。讨论本方法由于回输自身蛋白,临床症状改善快,住院时间短,不受住院地点限制,费用降低,能将有限的资金用于更多的治疗上。%Objective To analyze the ascites concentration into the clinical observation to explore for the treatment of nephrotic syndrome with refractory ascites. Methods From January 2000 to May 2004 I col i ascites concentration in blood dialysis machine to go back to lose, after treatment of nephrotic syndrome and 32 patients with refractory ascites. Results Al stents in the treatment of ascites after gradual y decreases, increase urine output, double leg edema relieve, spirit is bet er, increase appetite, physical strength enhancement, the symptoms of fever, flustered and other not.Conclusion This method due to the lost its protein, improve the clinical symptoms rapidly, short hospitalization time, not in place to restrict, reduce the cost, can the limited funds for more treatment.

  2. Padrões morfológicos de lesão glomerular e correlação com achados clinicolaboratoriais de 43 crianças com síndrome nefrótica Morphologic patterns of glomerular lesion and correlation with clinical and laboratory findings of 43 children with nephrotic syndrome

    Directory of Open Access Journals (Sweden)

    Márcia Camegaçava Riyuzo

    2004-10-01

    Full Text Available OBJETIVOS: Avaliar a associação entre os parâmetros clinicolaboratoriais e alteração morfológica de biópsias renais em crianças com síndrome nefrótica. MÉTODOS: Os dados foram obtidos dos prontuários médicos de 43 crianças com síndrome nefrótica submetidas a biópsia renal. RESULTADOS: Vinte e oito pacientes eram do sexo masculino (65,1%, idades entre 1,4 a 12 anos (média de 4,7±3,2. Quarenta e dois pacientes (97,7% apresentaram edema; 83,7%, oligúria e 32,5%, hipertensão arterial. A média de proteinúria foi 15,3g/1,73m²SC/dia e 55,8% apresentaram hematúria microscópica. As biópsias renais mostraram: glomerulonefrite proliferativa mesangial (GNPM em 37,2%, glomeruloesclerose segmentar e focal (GESF em 27,9%, alterações glomerulares mínimas (LM em 25,6%, glomerulonefrite membranoproliferativa (GNMP em 7% e glomerulonefrite membranosa (GNM em 2,3%. Vinte e seis pacientes (60,5% apresentaram resistência ao corticosteróide. Idade, sexo, hipertensão arterial, oligúria, uréia e creatinina séricas não mostraram diferenças estatísticas significativas entre os pacientes com GNPM, GESF e LM. Os pacientes com GNPM e GESF apresentaram maior freqüência de hematúria microscópica (p OBJECTIVES: To evaluate the association between clinical features and laboratory findings with the morphological changes in children with nephrotic syndrome. METHODS: The data were obtained from medical records of 43 children with nephrotic syndrome submitted to renal biopsy. RESULTS: Twenty-eight patients were male (65.1%, aged 1.4-12 years (mean 4.7 ± 3,2. Forty-two patients (97,7% presented edema, 83.7% oliguria and 32.5% hypertension. The mean of proteinuria was 15.3g/1.73m² BSA per day and 55.8% presented microscopic hematuria. Renal biopsies showed: proliferative mesangial glomerulonephritis (PMGN in 37.2%, focal segmental glomerulosclerosis (FSGS in 27.9%, minimal change disease (MCD in 25.6%, membranoproliferative

  3. Meta-analysis of the Efficacy of Bailing Capsules on Primary Nephrotic Syndrome%百令胶囊治疗肾病综合征的Meta分析

    Institute of Scientific and Technical Information of China (English)

    章建军; 谢升阳

    2013-01-01

    目的 对现有的关于百令胶囊对于肾病综合征成人患者的治疗作用的文献进行Meta分析,为临床实践提供依据.方法 通过Pubmed、中国生物医学文献数据库、中国期刊全文数据库等数据库检索,选取相关文献.提取效应尺度指标相关数据进行定量Meta分析或定性描述.结果 共纳入5篇文献.Meta分析结果显示百令胶囊能够降低肾病综合征患者的24h尿蛋白[SMD=-2.35(-3.72,-0.97),P<0.01],提高血清白蛋白水平[SMD=0.94(0.37,1.52),P=0.01],但对于血肌酐[SMD=-0.50(-1.25,0.26),P=0.20]及血尿素氮[SMD=-0.10(-0.36,0.17),P=0.48]的水平没有明显的改善作用.此外,其中有两个研究报道了百令胶囊能够降低血总胆固醇、甘油三酯及低密度脂蛋白.结论 百令胶囊的辅助治疗能够进一步减少肾病综合征患者的蛋白尿,提高血浆白蛋白水平,且可能能够降低患者血脂.但这些结论需要进一步临床试验证实.%OBJECTIVE To perform Meta-analysis of published articles on the efficacy of Bailing capsules for adult patients with primary nephrotic syndrome(PNS) and provide evidence for clinical practice.METHODS We searched Pubmed,CBM,CNKI and other databases to identify eligible articles and extract effect measure-related data to perform Meta-analysis.And qualitative description was also conducted for those unable to be combined.RESULTS Five records were selected.The results of Meta-analysis showed that Bailing capsules could reduce proteinuria [SMD=-2.35(-3.72,-0.97),P<0.01] and improve serum albumin [SMD=0.94 (0.37,1.52),P=0.01] in PNS patients.However,Bailing capsules adjuvant therapy had no effect on serum creatinine [SMD=-0.50(-1.25,0.26),P=0.20] and urea nitrogen level [SMD=-0.10(-0.36,0.17),P=0.48].Additionally,two studies reported that Bailing capsules could lower serum cholesterol,triglyceride and low density lipoprotein.CONCLUSION Addition of Bailing capsules could decrease proteinuria and enhance serum

  4. Increased VLDL in nephrotic patients results from a decreased catabolism while increased LDL results from increased synthesis

    NARCIS (Netherlands)

    de Sain-van der Velden, M; Kaysen, GA; Barrett, HA; Stellaard, F; Gadellaa, MM; Voorbij, HA; Reijngoud, DJ; Rabelink, TJ

    1998-01-01

    Increased very low density lipoprotein (VLDL) in nephrotic patients results from a decreased catabolism while increased low density lipoprotein (LDL) results from increased synthesis. Hyperlipidemias a hallmark of nephrotic syndrome that has been associated with increased risk for ischemic heart dis

  5. Multicenter study on present status of diagnosis and treatment of steroid-resistant nephrotic syndrome in children%激素耐药型肾病综合征患儿诊治现状多中心调研报告

    Institute of Scientific and Technical Information of China (English)

    中华医学会儿科学分会肾脏病学组

    2014-01-01

    Objective To investigate the treatment of multi-center hospitalized children with steroid resistant nephrotic syndrome (SRNS) retrospectively.Method The Subspecialty Group of Nephrology,The Society of Pediatrics,Chinese Medical Association organized 35 hospitals to investigate children with primary SRNS from 2008 to 2011.By filling in the questionnaire,data including age,gender,clinical type,related examination,pathology and treatment in local hospitals were collected,and then statistical analysis was performed.Result A total of 35 hospitals participated in this survey,577 patients were enrolled,their age was from 8 months to 18 years.The male to female ratio was 2∶ 1.School-age children accounted for 33.3% (n =194).Clinical types were mostly simple nephrotic syndrome,which accounted for 60.5% (n =349) and nephritis nephrotic syndrome accounted for 39.5% (n =228).The incidences of different types of NS were significantly different between 2008 and 2011 (x2 =10.751,P =0.001).There were 306 cases of infants who received renal biopsy.Renal pathology showed that 40 of them had mesangial proliferative glomerulonephritis (MsPNG),17 focal segmental glomerulo-sclerosis (FSGS),9 membranous nephropathy (MN),9 minimal change disease (MCD),8 minor lesions,3 endocapillary proliferative glomerulonephritis (EnPGN),2 membrano-proliferative glomerulonephritis (MPGN),2 sclerotic glomerulonephritis and 2 podocyte lesions in 2008.Otherwise there were 53 MsPGN,35 FSGS,25 minor lesions,23 MCD,13 MN,5 MPGN,3 podocyte lesions,2 EnPGN,1 sclerotic glomerulonephritis.Since the evidence-based guidelines had been issued in 2011,209 cases were treated with adequate-dose prednisone therapy > 4 weeks program.Eighty-seven children with nephrotic syndrome whose urinary protein could not be turned negative after 4 weeks' glucocorticoid treatment were treated with high-dose methylprednisolone for 1 course.Thirtyeight of these children got negative urinary protein after the pulse therapy

  6. Genetic Aspects of Nephrotic Syndrome

    DEFF Research Database (Denmark)

    Joshi, Shivani

    SSNS. In Study IV we performed a literature study on published disease causing variants in SRNS and based upon available evidence we developed a practical diagnostic algorithm for genetic evaluation of patients with SRNS. Several gene variants are involved in the pathogenesis of SRNS and genetic...... steroid dependence or become frequent relapsers. Repeated courses of corticosteroid treatment often cause significant associated morbidity. Familial occurrence of SSNS is rare and suggests a potential genetic origin. However, very little data on molecular genetics of familial SSNS is available...... in literature and no causal genes have yet been identified. Genetic aspects of NS bear important implications in therapeutic decisions and genetic counselling in SRNS patients and family members. During the present Ph.D. project we have studied the influence of genetic factors in patients with SRNS and familial...

  7. 卡托普利联合低分子肝素治疗肾病综合征的临床疗效分析%Analysis of nephrotic syndrome treatment by captopril combined with low molecular heparin

    Institute of Scientific and Technical Information of China (English)

    黄恬

    2014-01-01

    Objective To observe the treatment effect of captopril combined low molecular heparin in nephrot-ic syndrome .Methods Sixty and eight patients with nephrotic syndrome were collected from Chongqing cancer hos-pital ,which were randomly divided into control group and observation group .Conventional treatment were used in the control group ,captopril combined with low molecular heparin were added in the observation group .cholesterol , triglyceride ,urine protein ,whole blood viscosity ,hematocrit ,fibrinogen ,hematocrit of pigeon before and after treat-ment were compared .Results Total effective rate of observation group was 92 .1% ,which was significantly higher than the control group′s 83 .3% ,the difference was statistically significant (P<0 .05) .The observation group′s cho-lesterol ,triglyceride ,urine protein ,whole blood viscosity ,hematocrit ,fibrinogen ,hematocrit of pigeon were significant lower than those of the control group (P<0 .05) .Conclusion On the basis of conventional treatment ,captopril and low molecular heparin could significantly improve curative effect ,renal function and high coagulation state on pa-tients with nephrotic syndrome .%目的:观察卡托普利联合低分子肝素治疗肾病综合征的临床效果。方法选择本院收治的68例肾病综合征患者为研究对象。对照组30例患者,给予常规治疗28 d;观察组38例患者,在常规治疗的基础上给予卡托普利联合低分子肝素治疗28 d。观察并比较两组患者治疗前后胆固醇、三酰甘油、肌酐、清蛋白、尿蛋白、全血高切黏度、全血低切黏度、血浆黏度、血浆纤维蛋白原、血细胞比容等指标变化情况。结果观察组治疗总有效率达92.1%,明显高于对照组的83.3%,比较差异有统计学意义( P<0.05)。治疗28 d后,观察组胆固醇、三酰甘油、尿蛋白、全血黏度、血浆黏度、血浆纤维蛋白原、血细胞比容等指标明显低于对照

  8. 肾病综合征的疾病构成:单中心临床研究%The spectrum of nephrotic syndrome from a single-center clinical research

    Institute of Scientific and Technical Information of China (English)

    史晓虎; 李文歌; 杨冠男; 邹古明; 芦建华

    2012-01-01

    目的:探讨肾病综合征(NS)的疾病构成,为临床诊治提供数据.方法:调研中日友好医院肾内科2009年1月~2010年12月住院患者中全部NS患者的临床资料.结果:2年中在住院NS患者共282例,其中男158例,女性124例;患者年龄14~80岁,平均42.5±17.4岁;青年109例,中年119例,老年54例.282例NS患者中,伴高血压、糖尿病、慢性肾功能衰竭、急性衰竭者分别为87例、21例、31例、14例.其中184例进行了肾穿刺活检,青年患者(86例)肾脏病理前3位疾病是:局灶节段性肾小球硬化症(FSGS)24例、微小病变性肾病(MCD)21例、IgA肾病(IgAN)21例;中年患者(81例)肾脏病理前4位疾病分别是:原发性膜性肾病(MN)25例、FSGS 13例、继发性膜性肾病(SMN)12例、MCD 10例;老年患者(17例)肾脏病理前2位是:MN 9例、FSGS3例.结论:NS患者以中青年为主,常合并高血压、糖尿病、肾功能衰竭.青年患者肾脏病理类型以FSGS、MCD和IgAN肾病为主;中年患者以MN、FSGS和SMN为主;老年患者以MN和FSGS为主.%Objective:To investigate the constitute of nephrotic syndrome(NS)to provide data for clinical diag-nosis and treatment.Methods: A total of 282 cases in China-Japan Friendship Hospital from Jan.2009 to Dec. 2010 were enrolled in this prospective study .Their clinical and pathological datas were prospectively analyzed. Results:During this period,there were 282 cases of NS,accounts for 17.2% in total hospitalization.There were 158 males and 124 females,half of them were from cities,The average age of NS patients was 42.5±17.4 years old-Among them, 109 cases were youth(=35 yr),119 cases were middle-aged(36~59yr)and 54 cases the elder (=60yr),Eighty seven of patients with malignant hypertension and 21 cases were diagnosed with diabetes, 31 and 14 cases with chronic renal failure and acute renal failure, respectively .There were 184 cases accepted renal pathological biopsy.There were 86 cases in the youth group

  9. Síndrome nefrótica córtico-sensível e diabetes mellitus tipo 1 de início simultâneo Simultaneous onset of steroid-sensitive nephrotic syndrome and type 1 diabetes

    Directory of Open Access Journals (Sweden)

    Eduardo A. Rego Filho

    2003-11-01

    with steroid-sensitive nephrotic syndrome coexisting with type-1 diabetes mellitus. The interest to this clinical case is due to the unusual association of these diseases, the clinical symptoms and laboratory tests used to confirm diagnosis and the difficulties on corticotherapy. DESCRIPTION: Nephrotic syndrome was diagnosed in a boy (age 3 years and 11 months with generalized edema. Marked weight loss (23 to 16 kg, polyuria, polydipsia and weakness were observed after three weeks of treatment with prednisone 2 mg/kg/day. Diabetic ketoacidosis was confirmed by laboratory tests: hyperglycemia (glucose 657 mg/dl, glycosuria without proteinuria, acidosis and ketonuria. Therapy with insulin and prednisone was started. He was then maintained on a daily dose of NPH insulin. At age 4 years and 1 month a new episode of ketoacidosis without proteinuria occurred in association with a viral infection of the upper airways. At age 4 years and 4 months nephrotic syndrome relapsed, but the child responded well to steroid therapy. There was another relapse three months later, when prednisone treatment was interrupted. This led to the introduction of cyclophosphamide, with good results. Since then, the patient (now 5 years and 6 months old has been taking insulin daily and nephrotic syndrome has not relapsed. Plasma levels of C3 and C4 and renal function are normal. Hematuria is occasionally present. Anti-GAD antibodies (glutamic decarboxilase are normal and anti-islet cell antibodies are positive. HLA antigens: A2; B44; B52; DR4; DR8; DR53. COMMENTS: The simultaneous occurrence of steroid-sensitive nephrotic syndrome and type-1 diabetes mellitus is rare. The literature data, the familiar pattern and studies on HLA antigens are discussed.

  10. Efficacy of leflunomide in treatment of refractory nephrotic syndrome with concurrent infections%来氟米特治疗难治性肾病综合征并发感染的疗效研究

    Institute of Scientific and Technical Information of China (English)

    吴金土

    2012-01-01

    目的 观察来氟米特治疗难治性肾病综合征并发感染的临床疗效,为临床治疗提供依据.方法 选取医院2010年9月-2011年3月56例难治性肾病综合征患者,利用来氟米特进行治疗,观察其临床疗效.结果 56例患者经回访均完全缓解,缓解率为100.0%;其中29例占51.8%连续4年未复发,16例占28.6%连续3年未复发,11例占19.6%连续两年未复发;使用激素治疗共56例,持续缓解时间(5.5±0.7)个月;使用激素+环磷酰胺治疗共56例,持续缓解时间(9.5±0.8)个月;使用激素+吗替麦考酚酯治疗共33例,持续缓解时间(8.0±0.7)个月;使用激素+雷公藤治疗共25例,持续缓解时间(7.5±0.6)个月;使用激素+来氟米特治疗共56例,持续缓解时间(38.5±12.0)个月;使用激素+来氟米特治疗持续缓解时间明显长于其他药物治疗者.结论 来氟米特能够有效减少肾病综合征复发次数,减低感染率,延长难治性肾病综合征的持续缓解时间.%OBJECTIVE To observe the clinical efficacy of leflunomide in treatment of refractory nephrotic syndrome,so as to provide bases for clinical treatment- METHODS A total of 56 patients with refractory nephrotic syndrome from Sep 2010 to Mar 2011 were selected,all the subjects were treated with leflunomide, the clinical efficacy was observed. RESULTS Totally 56 patients were complete remission after a return visit with the remission rate of 100.0%, among which 29 patients (51. 8%) patients were without recurrence for 4 years, 16 patients (28. 6%) patients were without recurrence for 3 years, 11 patients (19. 6%) patients were without recurrence for 2 consecutive years; a total of 56 cases were treated with hormone with the sustained remission time (5. 5±0. 7) months; 56 cases were treated with hormone therapy plus cyclophosphamide with the sustained remission time of (9. 5±0. 8) months) 33 cases were treated with hormone plus mycophenolate mofetil with the sustained

  11. 雷公藤多苷联合泼尼松治疗老年肾病综合征的疗效观察%Effect of tripterygium wilfordii together with prednisone in treatment of elderly primary nephrotic syndrome

    Institute of Scientific and Technical Information of China (English)

    许勇芝; 黄志清; 唐蓉

    2009-01-01

    目的 观察雷公藤多苷联合中小剂量泼尼松治疗老年原发性肾病综合征的疗效.方法 78例老年原发性肾病综合征患者随机分为治疗组(n=42)和对照组(n=36),治疗组接受雷公藤多苷联合中小剂量泼尼松治疗,对照组接受大剂量泼尼松治疗.治疗期间定期监测24 h尿蛋白、血清白蛋白、肾功能、血脂等指标,观察疗效及复发率.结果 治疗6个月后治疗组完全缓解24例,部分缓解12例,无效6例,对照组分别为13例、10例、13例.治疗组总缓解率为85.7%,明显高于对照组的66.7%(P<0.05);治疗组复发率为14.3%,明显低于对照组的36.1%(P<0.05).结论 雷公藤多苷联合泼尼松治疗老年原发性肾病综合征疗效优于单纯应用泼尼松.%Objective To investigate the therapeutic effect of tripterygium wilfordii and prednisone in old patients with primary nephrotic syndrome. Methods 78 elderly primary nephrotic syndrome patients were randomly divided into two groups. 42 patients in treated-group were treated with tripterygium wilfordii and prednisone while 36 patients in control-group were treated with prednisone only. The curative effect,24 hours urinary protein, serum albumin,plasma lipid and renal function were detemined after six months. The responses of the patients were classified as complete remission(CR) ,partial remission(PR) and NO-response. Results After six months treatment, there were 24 patients got to CR,12 patients to PR,and 6 patients remained refractory to treatment group. While there were 13 patients got to CR,10 to PR,and 13 as refractory in the control group. The general effective rate in the treatment group was 85.7% ,which was markedly higher than that of the control group,which is 66. 67% (P<0. 05). The recurrence rate in the treatment group and control group were 14. 3% and 36. 1% respectively (P<0. 05 ). Conclusion The curative effect of tripterygium wilfordii and prednison on elderly primary nephrotic syndrome

  12. Observe the Curative Effect of Refractory Nephrotic Syndrome of Children in Treatment With Chinese Medicine%中药配合治疗小儿难治性肾病综合征的疗效观察

    Institute of Scientific and Technical Information of China (English)

    王晓华

    2015-01-01

    Objective To investigate the clinical efficacy of refractory nephrotic syndromewith chinese medicine treatment of children. Methods 56 patients with refractory nephrotic syndrome were randomly divided into study group and normal group,there were 28 cases. The conventional group received cytotoxic drugs shock therapy,studygroup in the conventional group based on the combined treatment of traditional chinese medicine,after 6 months of treatment,and adverse reaction were compared between the two groups of clinical efficacy.ResultsThe two groups after treatment,urine protein, cholesterol level were improved,but the study group improved amplitude better than routine group,and the total efficiency rate was higher than the conventional group,the significant difference has statistics significance(P<0.05); the study group the incidence of adverse reaction is lower than that of routine group,significant difference has statistics significance(P<0.05).Conclusion For the treatment of children with refractory nephrotic syndrome in conventional shock treatment on the basis of,the use of chinese medicine treatment can obviously improve the symptoms in children, improve the clinical efficacy,with low incidence of adverse reactions.%目的:探讨中药配合治疗小儿难治性肾病综合征的临床疗效。方法将我院收治的56例难治性肾病综合征患儿随机分为研究组和常规组,均为28例。常规组给予细胞毒类药物冲击治疗,研究组在常规组基础上联合中药治疗,治疗6个月后,对比两组临床疗效和不良反应情况。结果通过治疗后,两组胆固醇、尿蛋白定量水平均得到改善,但研究组改善幅度优于常规组,且总有效率也明显高于常规组,差异显著有统计意义(P<0.05);研究组不良反应发生率低于常规组,差异有统计意义(P<0.05)。结论对于小儿难治性肾病综合征的治疗在常规冲击治疗基础上,加用中药治疗可明显改

  13. [Severe hypercalcemia as a complication of intensive treatment for osteoporosis due to steroid therpay in 17-year-old girl with the nephrotic syndrome].

    Science.gov (United States)

    Ksiazek, Ewelina; Majewski, Marek; Borzecka, Halina; Sikora, Przemysław; Bieniaś, Beata; Zajaczkowska, Małgorzata

    2008-01-01

    In the article, 17-year-old girl with iatrogenic severe hipercalcemia was presented. The girl was treated since the age of 12 years for steroid-sensitive minimal change disease. Due to steroid therapy osteoporosis developed and intensive treatment with active form of vitamin D and high doses of calcium was started. She was admitted to our clinic in severe general state with abdominal pain, vomiting, dehydration, muscle weakness, hypertension and mental confusion. Severe hipercalcemia with nephrocalcinosis was diagnosed. The history revealed that the girl had increased the doses of drugs intentionally. The authors emphasized the need for careful monitoring of prophylaxis and treatment for osteoporosis due to steroid therapy.

  14. The Evolving Role of Rituximab in Adult Minimal Change Glomerulopathy.

    Science.gov (United States)

    Brown, Landon C; Jobson, Meghan A; Payan Schober, Fernanda; Chang, Emily H; Falk, Ronald J; Nachman, Patrick H; Pendergraft, William F

    2017-01-01

    Minimal-change glomerulopathy is defined histologically by the presence of normal glomeruli on light microscopy and diffuse podocyte effacement on electron microscopy. Although effective in children, corticosteroid treatment in adults is more variable and time to response can be prolonged. Data to support rituximab use in adults with corticosteroid-dependent or resistant minimal-change glomerulopathy are limited. Here, we describe the clinical course of adults with corticosteroid-dependent or -resistant minimal-change glomerulopathy who received rituximab. Demographic and clinical data were collected and analyzed from all adult patients with native kidney, biopsy-proven, minimal-change glomerulopathy who were administered rituximab between 2009 and 2014 and cared for at the UNC Kidney Center. Ten patients with corticosteroid-resistant (n = 5) or corticosteroid-dependent (n = 5) idiopathic minimal-change glomerulopathy were treated with rituximab between 2009 and 2014. Rituximab treatment induced remission in all 10 patients with a median time to remission of 2 months. The median time from rituximab to corticosteroid discontinuation was 3.5 months. The median remission time was 29 months and follow-up time was 39.5 months. No serious adverse events attributable to rituximab were observed. Rituximab induced remission in all patients with corticosteroid-dependent or -resistant minimal-change glomerulopathy, and may hold great therapeutic potential with good efficacy and minimal toxicity. Mounting evidence implies that a well-conducted randomized controlled clinical trial using rituximab in adults with minimal-change glomerulopathy in both corticosteroid-resistant and corticosteroid-dependent patients is warranted. © 2017 S. Karger AG, Basel.

  15. 频复发肾病综合征患儿家庭功能状况调查%Survey of family function condition of children with frequently relapsing nephrotic syndrome

    Institute of Scientific and Technical Information of China (English)

    吕林华; 李智英; 刘晓红

    2016-01-01

    [目的]探讨频复发肾病综合征患儿家庭功能现状及其影响因素。[方法]采用家庭功能评定量表(FAD)及自行设计的一般资料问卷,对广州市某三级甲等医院180例频复发肾病综合征住院患儿的家长进行调查。[结果]与广州市普通儿童家庭相比,频复发肾病综合征患儿的家庭功能在角色、行为控制、沟通及总体功能方面水平偏低(P<0.05),问题解决能力优于普通儿童家庭(P<0.05);主要照顾者为母亲的家庭情感介入功能较好(F=4.653,P=0.018),照顾者职业对问题解决功能存在影响(F=3.107,P=0.044),家庭居住地在城市者其家庭角色功能优于农村家庭(t=-3.114,P=0.004),确诊时间对家庭问题解决(F=3.587,P=0.042)及行为控制(F=5.506,P=0.010)均存在影响。[结论]频复发肾病综合征患儿家庭功能存在一定的功能障碍,主要照顾者、照顾者职业、家庭居住地、确诊时间均为家庭功能的影响因素。在护理工作中应当根据家庭及患儿情况,为家庭成员提供针对性的健康教育及心理护理。%Objective:To probe into the family function status quo of children with frequently relapsing nephrotic syndrome and its influencing factors.Methods:A total of 180 parents of children with frequently relapsing ne-phrotic syndrome from a third grade A hospital in Guangzhou were analyzed by using the Family Assessment Device(FAD)and self designed general information questionnaires.Results:Comparing with the families of normal children in Guangzhou,the family function of children with frequently relapsing nephrotic syndrome showed a low level in dimensions of role,behavior control,communication and overall function(P<0.05);but the problem solving ability showed a higher level than normal children's families(P<0.05).Emotional involve-ment showed a better function while mothers acting as the main caregivers for children(F=4.653,P=0.018), the job of caregivers affected the function of

  16. Multiple-factor analysis of proteinuria elimination time in children with primary nephrotic syndrome%儿童原发性肾病综合征尿蛋白转阴时间的多因素分析

    Institute of Scientific and Technical Information of China (English)

    元君辉; 王启林

    2014-01-01

    目的:分析儿童原发性肾病综合征激素治疗后尿蛋白转阴时间的影响因素。方法146例原发性肾病综合征患儿行激素治疗后,可评价疗效136例。按照尿蛋白转阴时间分为A组(尿蛋白转阴时间≤1周)和B组(1周1周的危险因素,与患儿的不良预后相关,临床应予以高度重视。%Objective To analyze the influencing factors of proteinuria elimination time after hormone therapy in chil-dren with primary nephrotic syndrome (PNS). Methods 146 cases of children with PNS were treated by hormone, and a-mong them 136 patients were evaluated for efficacy and tolerability. The patients were divided into two groups accord-ing to proteinuria elimination time. The proteinuria elimination time≤1 week of patients were in group A, while 1 week 1 week in children with PNS, which also relate to the worse prognosis and need to be paid more atten-tion in clinic.

  17. The Study on the Relationship between Serum Vascular Endothelial Growth Factor and Proteinuria in Adriamycin-induced Nephrotic Rats

    Institute of Scientific and Technical Information of China (English)

    ZHU; Zhonghua(

    2001-01-01

    [1]Carmeliet P Collen D.Molecular analysis of blood vessel formation and disease.Am J Physiol 1997 273 :H2091[2]Gleade J M Ebert B L Firth J D.Regulation of angiogenic growth factor expression by hypoxia transition metals and chelating agents.Am J Physiol 1995 268:C1362[3]Uchida K Uchida S.Glomerular endothelial cells in culture express and secrete vascular endothelial growth factor.AmJPhysiol 1994 266:F240[4]Simon M Grone H J Johren O.Expression of vascular endotheial growth factor and its receptors in human renal ontogenetis and adult kidney.Am J Physiol 1995 268:F81[5]Bailey E Bottomley M J.Vascular endothelial growth factor mRNA expression in minimal change membranous and diabetic nephrothy demonstrated by non-isotopic in situ hybridization.J Clin Pathol 1999 52:735[6]Nicholas J A Webb N J Waston C J.Circulation vascular endotheial growth factor is not increased during relapses of steroid-sensitive nephrotic syndrome.Kidney Int 1999 55:1063[7]Shulman K Rosen S Tognazzi K.Expression of VPF is altered in many glomerulous disease.J Am Soc Nephrol 1996 7:661

  18. Rituximab in treatment of children with frequently relapsed nephrotic syndrome%利妥昔单抗在小儿频复发肾病综合征中的应用

    Institute of Scientific and Technical Information of China (English)

    都娟; 黄建萍; 王硕; 赵晓艳; 肖丽丽

    2014-01-01

    Objective To explore the effect and safety of rituximab (RTX) in children with frequently relapsed nephrotic syndrome.Methods Twelve children (8 male and 4 female) with frequently relapse nephrotic syndrome were treated with RTX.They all showed resistance to various immunosuppressive agents and relapsed when steroid was reduced at 1.5-2.0 mg/kg.All immunosuppressive agents were stopped.Steroid was prescribed at doses of 2 mg/(kg · d),followed by alternate-day dosing (2 mg/kg) after proteinuria was negative for 5 days,and then tapered by 5 mg every 2 weeks until to low dose (< 0.5 mg/kg on alternate day) or discontinued.RTX was administered at a dose of 375 mg/m2 once every week for 3 weeks.Results The patients were followed up for 4-19 months [median (7.79 ± 3.00) months].Proteinuria turned negative on 1-14 d after first RTX treatment.Mean steroid dosages were significantly reduced than before treatment [alternate-day dosing (1.83 ± 1.02) mg/kg vs alternate-day dosing (0.34 ± 0.16) mg/kg,t =3.78,P =0.002].Gingival hyperplasia and Cushing appearance were significantly improved and steroid-induced glaucoma disappeared.The height was significantly increased compared with before treatment [(2.21 ± 1.40) cm/year vs (8.27 ±2.10) cm/year].Six cases had no relapse during follow-up.Another 6 cases relapsed in 5.5-19.0 months.All patients showed CD19 + B lymphocytes depletion after RTX treatment.However,CD19 + recovery could be seen in 5.5-12.0 months.Serum immunoglobulin did not significantly change.None of the patients was found with adverse events.Conclusions RTX can significantly reduce the dosage of steroid or even stop medication in children free of immunosuppressive agents,maintain remission for (7.79 ± 3.00) months,significantly improve the adverse reaction induced by steroid and immune-suppression.Thus,for children with frequently relapsed nephritic syndrome/steroid-dependent nephrotic syndrome who show resistance to immunosuppressive therapies

  19. 新生儿弥漫性系膜硬化型先天性肾病综合征一例报告并文献复习%To report of a case of congenital nephrotic syndrome of diffuse mesangial sclerosis and review literature

    Institute of Scientific and Technical Information of China (English)

    关则想; 徐玲玲; 莫樱; 蒋小云

    2012-01-01

    Objective: To report a case of congenital nephrotic syndrome whose renal pathology was diffuse mesangial sclerosis ( DMS) , in order to improve the recognition of the disease. Methods; To collect the clinical manifestations characteristic imaging of a case with congenital nephrotic syndrome of DMS, and to review and summarize the etiology, clinical manifestations, pathological features and prognosis of DMS. Results: She had early onset of clinical features of nephrotic syndrome, soon become renal insufficiency, progress to end - stage renal disease, and her renal biopsy pathology was diffuse mesangial sclerosis. She died two months later. Conclusions ; DMS manifested congenital nephrotic syndrome and often comprised renal insufficiency earlier, then progressing to renal failure. Renal biopsy is way to diagnose DMS. The disease has a poor prognosis, with no special treatment at present.%目的 报道一例新生儿弥漫性系膜硬化型先天性肾病综合征,以提高对该病的认识.方法 收集一例于2010年10月在中山大学附属第一医院儿科住院确诊为先天性肾病综合征弥漫性系膜硬化型的患儿临床资料,并复习文献,总结弥漫性系膜硬化型先天性肾病综合征的病因、临床表现、病理特点及预后.结果 患儿生后即有水肿、大量蛋白尿等肾病综合征表现,并很快出现肾功能不全,进展至肾功能衰竭,肾活检病理符合弥漫性系膜硬化型,2个多月后死亡.结论 先天性肾病综合征弥漫性系膜硬化型的临床表现为出生时或幼儿期内出现肾病综合征的特征,起病时可已有肾功能不全,进行性肾功能减退,确诊须肾穿病理,本病预后差,目前无特殊治疗.

  20. 探讨尿N-乙酰-β-D-氨基葡萄糖苷酶检测对原发性肾病综合征的临床价值%Discusses the urine N-acetyl-β-D-amino grape glycosidase to test the application value of the primary nephrotic syndrome

    Institute of Scientific and Technical Information of China (English)

    刘红; 伊力夏提; 岳华; 陆晨; 刘颖; 赵红娟

    2013-01-01

    Objectives To investigate the clinical value of urine NAG in Primary nephrotic syndrome patients.Methods 52 patients with primary nephrotic syndrome were enrolled who were in-patients,dated from December 2008 to May 2011,in patients before and after the use of hormone by colorimetric method to detect the urine NAG,and the detection results compare and statistical analysis.Results Primary nephrotic syndrome patients use hormone therapy,the detection of positive urine NAG for 9.62%,urinary protein qualitative results for (-) ~ +,a statistically significant difference(P < 0.05).Conclusions Urine NAG enzyme detect can as a primary nephrotic syndrome sensitive indexes,the curative judgment and delay the progress of kidney disease has an important clinical significance.%目的 探讨尿N-乙酰-β-D-氨基葡萄糖苷酶(N-acetyl-β-D-glucosa minidase,NAG)检测对原发性肾病综合征患者的临床价值.方法 选择2008年12月~2011年5月在新疆维吾尔自治区人民医院肾病科住院的原发性肾病综合征患者52例为病例组,对患者使用激素前、后采用对硝基苯酚(PNP)比色法检测尿NAG,并对检测结果进行比较及统计学分析.结果 原发性肾病综合征患者使用激素治疗后,检测尿NAG阳性率为9.62%,尿蛋白定性结果为(-)~+,差异具有统计学意义(P<0.05).结论 尿NAG酶检测可以作为原发性肾病综合征的敏感指标,对疗效判断及延缓肾脏疾病的进展具有重要的临床意义.

  1. A patient with minimal change disease and acute focal tubulointerstitial nephritis due to traditional medicine: a case report and small literature review.

    Science.gov (United States)

    Lee, Keun-Hyeun; Jeong, Han-Sol; Rhee, Harin

    2014-01-01

    Gongjin-dan (GJD) is a traditional formula that is widely used in Korea and China, and it has been used from 1345 AD in China to improve the circulation between the kidneys and the heart and to prevent all diseases. However, its adverse effects have not yet been reported. We present a patient with minimal change disease and focal tubulointerstitial nephritis associated with GJD. A 72-year-old man visited the clinic for generalized edema 20 days after starting GJD. His serum albumin level was low and nephrotic-range proteinuria was detected. A kidney biopsy showed minimal change disease and acute tubulointerstitial nephritis. After stopping GJD, a spontaneous complete remission was achieved. We discuss the possible pathogenesis of GJD-induced minimal change disease and review the adverse effects of GJD's ingredients and traditional Chinese medicines that can induce proteinuria. We report a new adverse effect of GJD, which might induce increased IL-13 production and an allergic response, leading to minimal change disease and focal tubulointerstitial nephritis.

  2. 小儿肾病综合征相关骨代谢异常及防治%Prevention and treatment of nephrotic syndrome associated bone metabolic abnormality in children

    Institute of Scientific and Technical Information of China (English)

    徐海霞; 姚勇

    2015-01-01

    Metabolic bone disease in nephrotic syndrome(NS) are increasingly being renal physician's attention.As calcium binding protein and VitD binding protein losing with a large number of protenuria, the bone metabolic biochemical abnormalities had happened at the beginning of the onset of the nephrotic syndrome, and is further exacerbated by therapeutic high-dose or long course of glucocorticoids (GC) application.The main mechanism of the glucocorticoid-induced osteoporosis (GIOP) is for GC to inhibit the activity of osteoblasts and promote apoptosis of osteoblasts and formation of osteoclasts, resulting in secondary hyperparathyroidism,leading to increasing the risk of osteoporosis,slow growth and fracture,seriously harm to children's physical and mental health.The biomarkers of bone transform can prompt the NS with bone metabolic abnormalities early;vertebral body bone dual-energy X-ray absorptiometry bone mineral density detection is the best method and position to determine GIOP.As the most commonly used and effective means to prevent and control metabolic bone disease, calcium supplements and VitD were always taken when GC was treated for NS, even the dosage of GC was very low.So far, it is still lack of guideline of prevention and treatment of bone metabolic abnormalities in NS in children.%肾病综合征(NS)时的代谢性骨病正日益受到儿肾科医师的关注.在NS起病之初因钙结合蛋白、维生素D结合蛋白自尿中丢失,骨代谢生化异常即已发生.治疗性糖皮质激素(GC)大剂量、长疗程的应用则进一步加剧了骨代谢异常,其主要机制为GC抑制成骨细胞的活性、促进成骨细胞的凋亡以及促进破骨细胞的生成、引起继发性甲状旁腺功能亢进,导致骨质疏松、生长迟缓、骨折风险增加,严重危及儿童身心健康.骨转换的生物标志物可以早期反映NS患儿骨代谢的异常;椎体骨双能X线吸收法骨密度检测是判断GC相关性骨质疏松症的最佳

  3. Effect of two prophylactic bolus vitamin D dosing regimens (1000 IU/day vs. 400 IU/day) on bone mineral content in new-onset and infrequently-relapsing nephrotic syndrome: a randomised clinical trial.

    Science.gov (United States)

    Muske, Sravani; Krishnamurthy, Sriram; Kamalanathan, Sadish Kumar; Rajappa, Medha; Harichandrakumar, K T; Sivamurukan, Palanisamy

    2017-05-03

    To examine the efficacy of two vitamin D dosages (1000 vs. 400 IU/day) for osteoprotection in children with new-onset and infrequently-relapsing nephrotic syndrome (IFRNS) receiving corticosteroids. This parallel-group, open label, randomised clinical trial enrolled 92 children with new-onset nephrotic syndrome (NS) (n = 28) or IFRNS (n = 64) to receive 1000 IU/day (Group A, n = 46) or 400 IU/day (Group B, n = 46) vitamin D (administered as a single bolus initial supplemental dose) by block randomisation in a 1:1 allocation ratio. In Group A, vitamin D (cholecalciferol in a Calcirol® sachet) was administered in a single stat dose of 84,000 IU on Day 1 of steroid therapy (for new-onset NS), calculated for a period of 12 weeks@1000 IU/day) and 42,000 IU on Day 1 of steroid therapy (for IFRNS, calculated for a period of 6 weeks@1000 IU/day). In Group B, vitamin D (cholecalciferol in a Calcirol® sachet) was administered as a single stat dose of 33,600 IU on Day 1 of steroid therapy (for new-onset NS, calculated for a period of 12 weeks@400 IU/day) and 16,800 IU on Day 1 of steroid therapy (for IFRNS, calculated for a period of 6 weeks@400 IU/day). The proportionate change in bone mineral content (BMC) was analysed in both groups after vitamin D supplementation. Of the 92 children enrolled, 84 (n = 42 new onset, n = 42 IFRNS) completed the study and were included in the final analysis. Baseline characteristics including initial BMC, bone mineral density, cumulative prednisolone dosage and serum 25-hydroxycholecalciferol levels were comparable in the two groups. There was a greater median proportionate change in BMC in the children who received 1000 IU/day vitamin D (3.25%, IQR -1.2 to 12.4) than in those who received 400 IU/day vitamin D (1.2%, IQR -2.5 to 3.8, p = 0.048). The difference in proportionate change in BMC was only statistically significant in the combined new-onset and IFRNS, but not for IFRNS alone. There was a greater

  4. The clinical study on evidence - based nursing in patients with nephrotic syndrome%循证护理在肾病综合征护理中的临床应用

    Institute of Scientific and Technical Information of China (English)

    胡晓萍; 王亚华; 熊迎春

    2015-01-01

    目的:探讨循证护理在肾病综合征护理中的作用。方法:将入组患者随机分为两组,对照组采用传统护理方法进行护理干预。实验组依据循证护理干预法,制订出一套系统有效的护理措施。结果:循证护理能够改善患者的生活习惯,提高护理满意度(P <0.05)。结论:循证护理在肾病综合征中具有积极的护理作用,可以改善患者生活习惯,提高护理满意度。%Objective:To investigate the effect of evidence - based nursing care in thesyndrome of kidney. Methods:The patients were randomly divided into two groups,the control group used the traditional nursing care intervention. The experimen-tal group on the basis of evidence - based nursing intervention,to develop a set of system and effective nursing measures. Re-sults:the evidence based nursing can improve the patients living habits,improve nursing satisfaction(P < 0. 05). Conclusion:Evidence - based nursing haspositive effect on nephrotic syndrome nursing,improve the living habits of patients,improve nurs-ing satisfaction.

  5. The impact of pediatric nephrotic syndrome on parents' health-related quality of life and family functioning: an assessment made by the PedsQL 4.0 family impact module.

    Science.gov (United States)

    Mishra, Kirtisudha; Ramachandran, Smita; Firdaus, Saima; Rath, Bimbadhar

    2015-03-01

    The multi-dimensional impact on the quality of life (QOL) of families of children with the nephrotic syndrome (NS) has not been studied sufficiently in the literature. We aimed to study this aspect and the predictors of poor QOL among Indian families having children with NS. A cross-sectional study was conducted to compare the parents of children with chronic NS on treatment for at least one year with parents of a matched healthy control group. The parents of both groups were asked to complete the standard self-administered multi-dimensional questionnaire of Pediatric Quality of Life Inventory 4 (PedsQL TM ) Family Impact Module (FIM). Descriptive and analytical statistics were performed to compare scores between the two groups. Possible predictors of poor outcome in each of the summary scales among the cases were assessed by both univariate and multivariate analysis. The parents of 61 cases and 72 controls completed the PedsQL TM FIM questionnaire. The scores in each of the categories, namely FIM Total Scale Score, Parent HRQOL Summary Score, Family Functioning Summary Score and eight individual domains, were found to be significantly higher among controls. Female gender of the affected child was an independent risk factor for poor Family Functioning Summary Score. Also, presence of serious complications during the course of the disease independently predicted poor Total FIM and Parent HRQOL Summary Scores. Even a relatively benign and potentially curable chronic disorder in children, like the NS, can disturb the QOL of parents in multiple domains of functioning.

  6. Effectiveness and nephrotoxicity of a 2-year medium dose of cyclosporine in pediatric patients with steroid-dependent nephrotic syndrome: determination of the need for follow-up kidney biopsy.

    Science.gov (United States)

    Kuroyanagi, Yoshiyuki; Gotoh, Yoshimitsu; Kasahara, Katsuaki; Nagano, China; Fujita, Naoya; Yamakawa, Satoshi; Yamamoto, Masaki; Takeda, Asami; Uemura, Osamu

    2017-07-11

    High dose of cyclosporine (CyA) for ≥2 years in children with steroid-dependent nephrotic syndrome (SDNS) increases the risk for nephropathy. Considering this, risk can be lowered with lower doses of CyA; we evaluated the effects of a medium dose of CyA, with target serum level, C2, of 450 ng/ml, over a 2-year period of observation, to determine the need for follow-up kidney biopsy. We retrospectively evaluated C2 levels in 38 patients (17 males, 5.2 ± 2.9 years old) with SDNS at treatment initiation, at 6, 12 and 18 months during treatment, and at the time of kidney biopsy, 2-year after treatment initiation. Fifteen patients were also treated with mizoribine or mycophenolate mofetil. A number of relapses-per-patient-per-year, relative to SDNS onset and initiation of CyA treatment, were evaluated. Serum levels of total protein, albumin and total cholesterol, blood urea nitrogen level, and the estimated glomerular filtration rate were measured at treatment initiation and at 1- and 2-year post-treatment initiation. Only one very mild case of CyA-associated nephrotoxicity was identified based on biopsy results at 2-year post-treatment initiation. C2 concentrations were maintained at 422.2 ± 133.5 ng/ml and the number of relapses decreased from 3.0 relapses-per-patient-per-year prior to CyA treatment to 0.47 relapses-per-patient-per-year after CyA treatment. No effects of the treatment on the estimated glomerular filtration rate were noted. A 2-year treatment with a medium dose of cyclosporine A with or without other immunosuppressive agents is relatively safe with regard to the development of cyclosporine A nephrotoxicity.

  7. 呈肾病综合征的乙型肝炎病毒相关性肾炎临床病理特点%Clinicopathological Features of Hepatitis B Virus-associated Glomerulonephritis Patients with Nephrotic Syndrome

    Institute of Scientific and Technical Information of China (English)

    刘波; 庄永泽; 余英豪

    2013-01-01

    目的:探讨呈肾病综合征(NS)的乙型肝炎病毒相关性肾炎(hepatitis B virus-associated glomerulonephritis,HBV-GN)的临床病理特点.方法:回顾性分析呈NS且肾穿组织内小动脉≥5条的HBV-GN患者152例的临床病理资料,光镜下观察其肾内小动脉病变,比较伴与不伴肾内小动脉病变患者的临床病理资料.结果:平均年龄26.3岁,男:女为3.6:1;伴高血压者36例(23.7%)、贫血52例(34.2%)、肾衰竭者33例(21.7%)、肝功能异常者20例(13.2%).乙肝大三阳者87例(57.2%);血清和肾组织内HBV-DNA载量呈正相关(P<0.01).病理表现以膜性肾性(MN)、系膜增生性肾炎(MsPGN)、膜增生性肾炎(MPGN)为主;未成年组与成年组MN、MPGN所占比例不同(P<0.05).伴有肾内小动脉病变者84例(55.2%),与无肾内小动脉病变者相比,其高血压发生率较高(P<0.05),24 h尿蛋白定量显著较高(P<0.01),贫血、肾间质纤维化的发生率显著较高(P<0.01).结论:呈NS的HBV-GN患者绝大多数为HBV携带者且伴有病毒复制,病理以MN、MsPGN及MPGN为主,半数以上伴有肾内小动脉病变,伴有肾内小血管病变者高血压、贫血及肾小管间质纤维化发生率较高,蛋白尿更严重,为预后不良的病理学指标.%Objective:To Investigate the clinicopathological features of hepatitis B virus -associated glomerulonephritis patients with nephrotic syndrome. Methods:The clinical and pathological data of 152 cases with nephrotic syndrome and renal biopsy tissue small artery cross - sectional ≥ 5 of HBV - GN were analyzed retrospectively. Small intrarenal arterial lesions( SRAL ) were observed, and the clinical and pathological data in patients with or without SRAL were compared. Results: Among them, there was a 3. 6:1 predominance of males, mean age was 26. 3 years,36( 23. 7% ) patients broken out with hypertension, 52( 34. 2% ) with anemia, 33( 21. 7% ) suffered renal dysfunction, 20( 13. 2% ) liver function were impaired, 87

  8. Analysis of risk factors in primary nephrotic syndrome patients with urinary tract infection%原发性肾病综合征患者尿路感染危险因素分析

    Institute of Scientific and Technical Information of China (English)

    郝向勤; 郝亚宁

    2014-01-01

    ObjectiveTo investigate the risk factors in primary nephrotic syndrome (PNS) patients with urinary tract infections (UTI) and provide clinical reference for the prevention of urinary tract infection. MethodFrom February 2007 to October 2013, the clinical data of 570 cases with PNS combined with 154 cases of UTI were retrospectively analyzed. Through the comparative analysis of two groups and the statistical analysis of related factors (including gender, age, complications, hormone dosage and immunosuppressor) in 154 cases of PNS combined with UTI, in order to ifnd the relations between various factors and primary nephrotic syndrome of urinary tract infections.Result154 cases were urinary tract infection in 570 cases with PNS, and the incidence of infection was 27.0%; compared with patients with no UTI complication, the content of the urine protein, serum protein, lipoprotein (a) [Lp(a)], IgG and low density lipoprotein (LDL) in patients with PNS combined with UTI had signiifcant changes (P0.05). The results of single factor showed that the incidence of urinary tract infection was not related with age. In different stages of age, the incidence of children was greater than seniors and young adults. Two groups had signiifcant difference (P<0.05). Patients can improve the incidence of UTI (P<0.05) by the use of immunosuppressor and high doses of hormones and other complications.ConclusionLow serum proteinemia, low IgG emesis emia, high proteinuria, high LDL and (or) Lp(a) may indicate the incidence of primary nephrotic syndrome combined with urinary tract infections. Age, complications, with or without the use of immunosuppressor and concentration of hormone may be risk factors of causing urinary tract infection.%目的:探讨原发性肾病综合征(PNS)患者并发尿路感染(UTI)的危险因素,为诊断和治疗PNS并发UTI提供临床参考依据。方法对2007年2月至2013年10月本院收治的570例PNS患者中并发UTI的154例患者(试验

  9. 灯盏细辛联合低分子肝素治疗肾病综合征的临床疗效观察%Clinical Effect of Herba Erigerontis Breviscapi Combined with Low Molecular Heparin on Nephrotic Syndrome

    Institute of Scientific and Technical Information of China (English)

    廖家贤; 韦秋敏; 卢荣羡; 韦东艳

    2012-01-01

    Objective To explore the clinical effect of Herba Erigerontis Breviscapi combined with low molecular heparin on nephrotic syndrome. Methods We performed a retrospective analysis on the clinical data of 78 patients with primary nephrotic syndrome patients in our hospital from January 2009 to December 2010. The patients were randomly divided into two groups: treatment group and control group, with 39 cases in each group. Patients in the control group were given conventional treatment. Patients in the treatment group were given Herba Erigerontis Breviscapi combined with low molecular heparin besides conventional treatment. The treatment period lasted for about two weeks. The levels of urine protein, serum triglyceride (TG) , total cholesterol (Choi) , serum albumin (ALB) and activation of blood coagulation enzyme time (APTT) , fibrinogen (FIB) of patients in two groups were tested and the clinical effect was compared between two groups. Results The curative effect of the treatment group was better than the control group, with the effective rate of 92.3% and 71.8% (P <0.05). Compared with the control group, the treatment group, there was a signinicant increase of 24 h urine protein, and a significant increase of serum albumin, serum triglyceride and total cholesterol in patients in treatment group. The active site blood coagulation time was significantly longer and the fibrinogen (FIB) was reduced in treatment group than control group. Conclusion Herba Erigerontis Breviscapi combined with low molecular heparin can improve the levels of urinary albumin, decrease the blood lipids levels, improve the high coagulative state and improve the serum albumin levels of primary nephrotic syndrome patients, so has good therapeutic effect.%目的 探讨灯盏细辛联合低分子肝素治疗肾病综合征的临床疗效.方法 回顾性分析2009年1月至2010年12月在河池市中医院治疗的78例原发性肾病综合征患者的临床资料,随机分为2组,对照组39

  10. The Diagnosis of Minimal Change Disease in Diabetic Nephropathy

    OpenAIRE

    M. Barry Stokes

    2005-01-01

    Proteinuria is common in diabetic patients and usually reflects the presence of diabetic glomerulosclerosis. This paper reviews the differential diagnosis of proteinuria in diabetic patients and discusses the role of renal biopsy examination in identification and management of minimal change disease in this cohort. Identification of nondiabetic glomerular disease requires careful correlation of clinical history and renal biopsy findings and may have important implications for prognosis and th...

  11. Lithium-induced minimal change disease and acute kidney injury

    OpenAIRE

    Parul Tandon; Natalie Wong; Zaltzman, Jeffrey S.

    2015-01-01

    Context: Lithium carbonate is a psychiatric medication commonly used in the treatment of bipolar disorder. It has been implicated in inducing nephrogenic diabetes inspidus, chronic tubulointerstitial nephropathy, and acute tubular necrosis. We describe a case of lithium-induced minimal change disease (MCD) and acute kidney injury (AKI). Case Report: A 32-year-old female with a medical history of bipolar disorder treated with chronic lithium therapy presented with anasarca, fatigue, and tremor...

  12. The detection of aberrantly glycosylated serum IgA1 in minimal change nephrotic syndrome with mesangial IgA deposition patients%伴IgA沉积的微小病变肾病综合征患者血清IgA1糖基化缺陷的检测

    Institute of Scientific and Technical Information of China (English)

    陈玮; 燕宇; 陈幸; 陈欢; 王梅

    2012-01-01

    目的 通过对伴IgA沉积的微小病变肾病综合征(MCNS-IgA)患者血清中IgA1分子糖基化缺陷程度的检测,探讨MCNS-IgA的可能的病理分类归属. 方法 选择北京大学人民医院肾内科MCNS-IgA患者10例,微小病变肾病综合症(MCNS)患者10例、大量蛋白尿IgAN (H-IgAN)患者10例为对照.用双抗体夹心ELISA法检测各组患者血清IgA1的相对浓度,用黑木凝集素检测IgA1分子的α2,6唾液酸水平,花生凝集素检测IgA1分子的半乳糖水平,蜗牛凝集素检测IgA1分子的N-乙酰氨基半乳糖水平,计算经血清IgA1浓度校正的各糖基水平.观察MCNS-IgA组患者血清IgA1分子糖基化缺陷情况,并且与H-IgAN及MCNS组进行比较.结果 与MCNS组相比,MCNS-IgA肾病患者血清IgA1的α2,6唾液酸(1.232±0.250比较1.379±0.623,P=0.455)、半乳糖缺失(0.204±0.053 vs 0.229±0.088,P=0.454)水平及N-乙酰氨基半乳糖暴露(0.191±0.039 vs0.205±0.068,P=0.626)水半无明显差异.但其血清IgA1分子α2,6唾液酸缺失(1.232±0.250vs 0.756±0.243,P=0.015)及N-乙酰氨基半乳糖的暴露(0.191±0.039比0.258±0.066,P=0.025)显著低于H-IgAN组,半乳糖缺失少,但未达统计学差异(0.204±0.053比0.139±0.038,P=0.052). 结论 MCNS-IgA组患者血清IgA1糖基化水平上显示了与IgA肾病不同的特点,提示其可能是微小病变伴IgA分子的非特异性沉积.%Objective To test the different degree of glycosylation of serum IgAl among MCNS-IgA, H-IgAN, MCNS, in order to investigate the relationship between MCNS-IgA and IgAN. Methods Sera from 30 patients were collected. 10 of them were MCNS-IgA, 10 patients were H-IgA and the rest were MCNS used as controls. Biotinylated lectins were used in ELISAto examine different glycans on serum IgAl molecules. The a 2,6 sialic acid was detected by elderberry bark lectin (SNA), the exposure of terminal galactose (Gal) and N-acetylgalactosamine (GalNAc) were detected by arachis hypogaea (PNA) and Helix asperas agglutinin (HAA), respectively. The IgAl glycans levels, corrected by IgAl concentrations, were compared between patients and controls. Results Reduced terminal a 2,6 sialic acid (1.232 ± 0.250 vs 0.756 ± 0.243, P=0.015), and increased exposure of N-acetylgalactosamine (0.191 ± 0.039 vs 0.258 ± 0.066, P=0.025) were demonstrated in serum IgAl from patients with H-IgAN as compared in that from MCNS-IgA. However, MNCS and MCNS-IgA had no significant differences among the levels of glycosylation (P > 0.05). Conclusion MCNS-IgA shows the characteristics of IgA nephropathy in serum IgAl glycosylation level, suggesting the presence of MCD with non-specific mesangial IgA molecule deposition.

  13. The role of fibroblast browth factor-2 in children with minimal change nephrotic syndrome%成纤维细胞生长因子-2检测在儿童微小病变型肾病中的意义

    Institute of Scientific and Technical Information of China (English)

    孙利; 徐虹; 张秀荣; 郭慕依

    2005-01-01

    目的观察成纤维细胞生长因子-2(FGF-2)在微小病变肾病患儿的肾组织、血和尿中的表达;探讨FGF-2的作用.方法收集1999年6月至2003年6月复旦大学附属儿科医院50例微小病变型肾病(MCNS)患儿的临床资料,用免疫组化法检测所有患儿肾组织中的FGF-2沉积,同时用酶联免疫吸附试验检测20例MCNS患儿不同时点(发病期、缓解期、未缓解期和复发期)的血、尿FGF-2质量浓度.结果 (1)FGF-2的分布:在MCNS患儿的肾组织中,FGF-2主要分布在肾小球足细胞和肾小管上皮细胞的胞浆中,且有不同程度的表达.(2)肾小球的FGF-2表达:频复发组的肾小球FGF-2明显高于非频复发组(P<0.001);肾小球FGF-2与尿液中的转铁蛋白(TRF)、白蛋白(Alb)和视黄醇结合蛋白(RBP)呈正相关(P<0.05).(3)肾小管的FGF-2表达:肾小管FGF-2与尿液中的TRF呈正相关(P<0.001).肾活检时尿液FGF-2的质量浓度与肾小管FGF-2呈正相关(P<0.005).(4)血、尿FGF-2的变化:尿液FGF-2的质量浓度在缓解期明显低于初发期;复发期比初发期更高;未缓解期亦比初发期高(P<0.05).血FGF-2质量浓度各期无明显差异.结论 MCNS患儿的肾组织中,FGF-2主要表达于肾小球足细胞和肾小管上皮细胞的胞浆中.肾小球FGF-2的分泌与临床出现MCNS频复发有关.尿FGF-2质量浓度主要反映肾小管FGF-2的分泌,其动态变化可监测MCNS病情的发展.

  14. 微小病变性肾病合并门静脉脾静脉肠系膜上静脉血栓1例并文献复习%Minimal change disease of nephrotic syndrome complicated with portal,splenic and superior mesenteric vein thrombosis

    Institute of Scientific and Technical Information of China (English)

    王军; 田百玲; 范秋灵

    2009-01-01

    原发性肾病综合征(NS)由多种疾病组成,主要包括微小病变性肾病、局灶节段性肾小球硬化、膜性肾病、IgA肾病、膜增生性肾小球肾炎等。NS易形成静脉血栓,多为肾静脉血栓、下肢静脉血栓等,但合并门静脉、脾静脉、肠系膜上静脉血栓形成十分罕见。现将1例NS合并门静脉、脾静脉、肠系膜上静脉血栓形成的病例报道如下。

  15. Hemostatic problems and thromboembolic complications in nephrotic children.

    Science.gov (United States)

    Citak, A; Emre, S; Sâirin, A; Bilge, I; Nayir, A

    2000-02-01

    A hypercoagulable state and the risk of thromboembolism in both arterial and venous circulation is a relatively frequent and serious feature of nephrotic syndrome (NS) in children and adults. The aim of this study was to evaluate the coagulation states of children with NS before and after corticosteroid (CS) therapy and to compare the results with a healthy control group. The first group consisted of 49 nephrotic children (30 boys and 19 girls) with a mean age of 6. 5+/-4.9 years (range 1-16 years). The control group included 17 healthy children (9 boys and 8 girls). At the time of admission, all patients were evaluated for the presence of clinical thromboembolism, hematological and biochemical indicators of a hypercoagulative state, and renal disease. This was repeated after CS treatment. Deep vein thrombosis was observed in 2 nephrotic patients who had very low plasma antithrombin III (AT III) levels and fibrinogen levels above 750 mg/dl. Thus, the prevalence of thromboembolism was 4% in our pediatric nephrotic population. The mean AT III level of the study group was 68.2+/-23.4% at the onset of the disease, which was significantly lower than the level of the control group (84.0+/-7. 6%). Plasma AT III levels increased to 74.4+/-15.3% after CS treatment, which correlated with the serum albumin levels. However, there was no correlation with urinary protein excretion. Protein C levels were higher than controls during all stages of the disease in both steroid-responsive and -unresponsive patients. The mean protein S level was similar in both groups. Plasma fibrinogen and cholesterol levels were significantly higher in the study group but decreased to within normal limits with remission. Our study suggests that thromboembolic complications are not infrequent in children with NS, and may be related to low plasma AT III and albumin and high fibrinogen and cholesterol levels.

  16. 黄芪注射液对儿童原发性肾病综合征高凝状态的影响%Effect of Astragalus Inj ection on Hypercoagulation State in Children with Primary Nephrotic Syndrome

    Institute of Scientific and Technical Information of China (English)

    陈文波; 张经; 吴学典

    2014-01-01

    目的:探讨黄芪注射液对儿童原发性肾病综合征(primary nephrotic syndrome,PNS)高凝状态的影响。方法将41例PNS患儿随机分为治疗组21例、对照组20例。2组均给予泼尼松片1.5~2 mg·kg-1·d-1口服治疗,在常规激素治疗基础上治疗组给予黄芪注射液1 mL · kg-1· d-1静脉滴注治疗,对照组给予阿司匹林1~3 mg· kg-1· d-1口服治疗。15 d 为1个疗程,2组均治疗2个疗程。结果2组治疗后血生化指标血尿素氮(BUN)、肌酐清除率(Ccr)、血肌酐(Scr)、血浆清蛋白(A1b)、总胆固醇(TC)、三酰甘油(TG),24 h尿蛋白定量及血液高凝状态指标血纤维蛋白原降解产物(FDP)、血浆纤维蛋白原(FIB)浓度、血 D-二聚体(D-D)较治疗前均显著改善(P<0.01),且治疗组改善效果明显优于对照组,差异有统计学意义(P<0.05);2组凝血酶原时间(PT)指标治疗前后比较差异无统计学意义(P>0.05)。结论黄芪注射液对PNS患儿高凝状态有显著疗效,同时可降低蛋白尿,保护肾功能。%Objective To investigate the effect of astragalus injection on hypercoagulation state in children with primary nephrotic syndrome(PNS).Methods Forty-one children were randomly divided into treatment group(n=21)and control group(n=20).All patients were treated with o-ral prednisone tablets (1 .5-2 mg · kg-1 · d-1 ).In addition,treatment group and control group were given two 15-day cycles of intravenous infusion of astragalus injection(1 mL·kg-1·d-1) and oral aspirin(1-3 mg·kg-1 ·d-1 ),respectively.Results Blood urea nitrogen,creatinine clear-ance rate,serum creatinine,plasma albumin,total cholesterol,triacylglycerol,24-hour protein ex-cretion,fibrinogen degradation products,plasma fibrinogen and D-dimer were significantly im-proved after treatment in both group(P0.05).Conclusion Astragalus injection is effec-tive for hypercoagulation state

  17. Clinicopathological features of cosmetics mercury poisoning-related nephrotic syndrome: A case report%1例化妆品汞中毒相关肾病综合征临床病理分析

    Institute of Scientific and Technical Information of China (English)

    李红艳; 魏日胞; 王远大; 杨勇

    2011-01-01

    目的 探讨化妆品汞中毒相关肾病的临床病理特点及治疗方法.方法 解放军总医院肾科2010年1 1月收治的1例女性化妆品汞中毒病例,分析其临床病理特点及对驱汞治疗的反应.结果 该病例用含汞美白化妆品半年,临床表现为肾病综合征,血压及肾功能正常,血清自身抗体系列正常.病理特点表现为膜性肾病Ⅰ期,患者未用免疫抑制剂,驱汞治疗效果较好,尿蛋白逐渐减少,全身症状改善.结论 本例化妆品汞中毒相关肾病表现为肾病综合征,病理为膜性肾病Ⅰ期,对驱汞治疗反应较好.%Objective To study the clinicopathological features of cosmetics mercury poisoning-related nephropathy and its treatment methods. Methods Clinicopathological features of a female patient with cosmetics mercury poisoning-related nephropathy admitted to Department of Nephrology, Chinese PL A General Hospital, in November 2010 and her response to antimercurialism were analyzed. Results The patient developed nephritic syndrome after using whiten cosmetics that contains mercury for half a year. Her blood pressure, renal function, and serum autoantibody were normal. Pathologic examination showed stage 1 membranous nephropathy. The patient did receive any immunosuppressive agent. After antimercurialism, her urinary protein gradually decreased and systemic symptoms improved. Conclusion Cosmetics mercury-related nephropathy is manifested as nephrotic syndrome (which was diagnosed as stage I membranous nephropathy in this patient who had a rather good response to antimercurialism).

  18. Plasma alpha(2) macroglobulin is increased in nephrotic patients as a result of increased synthesis alone

    NARCIS (Netherlands)

    Sain-van der Velden, MGM; Rabelink, TJ; Reijngoud, DJ; Gadellaa, MM; Voorbij, HAM; Stellaard, F; Kaysen, GA

    1998-01-01

    Background. alpha(2) Macroglobulin (alpha(2)M), a protease inhibitor, is often increased in plasma of patients with the nephrotic syndrome. Although it has been speculated that synthesis is increased, no direct measurements have been performed. Methods. alpha(2)M synthesis in both normal subjects (N

  19. Minimal change disease caused by mercury-containing cosmetics: 7 cases report%七例化妆品汞中毒相关肾小球微小病变病报告

    Institute of Scientific and Technical Information of China (English)

    王艳艳; 谌贻璞; 王国勤; 董鸿瑞; 孙丽君; 王长江; 唐艳玲; 程虹

    2013-01-01

    目的 探讨化妆品汞中毒相关微小病变病(MCD)的临床病理表现及治疗方法.方法 回顾性分析首都医科大学附属北京安贞医院肾内科确诊的7例化妆品汞中毒相关MCD患者的临床及病理资料.结果 7例患者均为女性,平均年龄37.2岁,均曾间断使用美白祛斑化妆品.7例患者尿汞均明显升高,为正常值的2.2~ 59.1倍.其中2例患者化妆品的汞检测,汞浓度为国家规定化妆品汞限量的1000多倍.7例患者均表现为肾病综合征,肾组织病理检查符合MCD诊断.经停用含汞化妆品,并予糖皮质激素及驱汞治疗,7例患者肾病综合征均在应用激素后1~2个月完全缓解.结论 对非少年儿童及老年的MCD女性患者,要注意询问美白祛斑化妆品的应用史,检测尿和血汞浓度,以早期发现化妆品汞中毒相关MCD.%Objective To enhance the understanding of mercury poisoning related minimal change disease (MP-MCD).Methods A retrospective analysis about the clinical,laboratory and pathological manifestations,diagnosis,treatment and outcome of 7 patients with MP-MCD caused by skin-lightening cosmetics were conducted.Results All of the 7 patients were female with mean age 37.2 years.One patient had intermittently used skin-lightening cosmetics for 15 years and the other 6 patients continuously used the cosmetics for 2 ~ 6 months.Urine mercury levels of the 7 patients were significantly increased,reaching to 2.2 ~ 59.1 times of the normal value.The mercury content of 2 cosmetics used by patients was 1200 to 1560 times the mercury limit in cosmetics set by the government in 1987.Seven patients all presented as nephrotic syndrome,and all had MCD on renal biopsy.After the diagnosis of MP-MCD was confirmed,all the patients stopped using the mercurycontain cosmetics and accepted corticosteroid and chelation therapy.The nephrotic syndrome underwent complete remission after 1 ~ 2 months of therapy.Urine mercury levels in the 5 patients

  20. Exploration of the relationship between the influence of prednison on cell apoptosis of the peripheralblood mononuclear cell in children with primary nephrotic syndrome%激素治疗与小儿肾病综合征外周血单个核细胞凋亡的关系

    Institute of Scientific and Technical Information of China (English)

    刘振翔; 杜华; 黄欣秋; 周微雅

    2003-01-01

    AIM: To explore the influence of prednisone on cell apoptosis of the peripheral blood mononuclear cell (PBMC) in children with primary nephrotic syndrome (PNS).METHODS: According to the results of prednison after treatment,we divided 50 cases into two groups. 37 cases were steroid-responsive and 13 were not responsive. Taking blood from the two groups before and after prednison treatment (in the 2nd,4th,6th and 8th weeks), we calculated the apoptosis rate of PBMC. RESULTS: There was no significant differences in the apoptosis rate.of PBMC between the two groups and the normal control before treatment. The apoptosis rate of PBMC increased obviously after the patients were treated in the 2nd, 4th, 6th weeks,and the highest was in the second week (P<0.05,P<0.01),in comparison with that before the treatment. During the eighth week, the apoptosis rate of PBMC was obviously higher in the steroid-unresponsive than that of steroid-response (P<0.05). CONCLUSIONS: The cell apoptosis may be involved in the regulations during the process of treatment. Some disorders of cell apoptosis may be responsible for the steroid-unresponsiveness in children with nephrotic syndrome.

  1. Proteinuria predicts relapse in adolescent and adult minimal change disease

    Directory of Open Access Journals (Sweden)

    Cristiane Bitencourt Dias

    2012-11-01

    Full Text Available OBJECTIVE: This study sought to outline the clinical and laboratory characteristics of minimal change disease in adolescents and adults and establish the clinical and laboratory characteristics of relapsing and non-relapsing patients. METHODS: We retrospectively evaluated patients with confirmed diagnoses of minimal change disease by renal biopsy from 1979 to 2009; the patients were aged >13 years and had minimum 1-year follow-ups. RESULTS: Sixty-three patients with a median age (at diagnosis of 34 (23-49 years were studied, including 23 males and 40 females. At diagnosis, eight (12.7% patients presented with microscopic hematuria, 17 (27% with hypertension and 17 (27% with acute kidney injury. After the initial treatment, 55 (87.3% patients showed complete remission, six (9.5% showed partial remission and two (3.1% were nonresponders. Disease relapse was observed in 34 (54% patients who were initial responders (n = 61. In a comparison between the relapsing patients (n = 34 and the non-relapsing patients (n = 27, only proteinuria at diagnosis showed any significant difference (8.8 (7.1-12.0 vs. 6.0 (3.6-7.3 g/day, respectively, p = 0.001. Proteinuria greater than 7 g/day at the initial screening was associated with relapsing disease. CONCLUSIONS: In conclusion, minimal change disease in adults may sometimes present concurrently with hematuria, hypertension, and acute kidney injury. The relapsing pattern in our patients was associated with basal proteinuria over 7 g/day.

  2. Minimal change disease versus IgA nephropathy.

    Science.gov (United States)

    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.

  3. Minimal change disease versus IgA nephropathy

    Directory of Open Access Journals (Sweden)

    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.

  4. Relationship of Interleukin-18 and Interleukin-18 Antibody in Adrianlycin-Induced Model of Minimal Change Nephritic Syndrome%白细胞介素-18和白细胞介素-18抗体与大鼠微小病变型多柔比星肾病的关系

    Institute of Scientific and Technical Information of China (English)

    冯晓慧; 贾春梅; 王冬梅

    2011-01-01

    目的 探讨IL-18和IL-18抗体(IL-18 Ab)与大鼠微小病变型多柔比星肾病的关系.方法 将30只Wistar大鼠随机分为3组:正常对照组、非治疗组、IL-18Ab治疗组.尾静脉注射多柔比星(6.5 mg·kg)造成微小病变型肾病综合征动物模型.其中IL-18Ab治疗组腹腔注射IL-18Ab(每只10 μg),非治疗组注射等量9 g·L盐水,正常对照组大鼠予尾静脉注射等量9 g·L盐水.分别于第1、14、28及42天检测其24 h尿蛋白水平,第42天心脏取血测其血清总胆固醇(TC)、三酰甘油(TG)、总蛋白(TP)、清蛋白(Alb)、BuN、SCr、IL-18、干扰素-γ(IFN-γ)及TNF-α水平,取其肾组织进行免疫组织化学分析IL-18、IFN-γ、TNF-α的表达及光、电镜病理形态.结果 24 h尿蛋白定量:非治疗组和IL-18Ab治疗组第14天开始增加,第28天达高峰;第14、28、42天非治疗组和IL-18Ab治疗组均高于正常对照组(P<0.05).肾组织光、电镜病理形态:3组光镜无明显改变,电镜:正常对照组结构正常,非治疗组病变明显,IL-18Ab治疗组病变轻微.结论 IL-18介导IFN-γ、TNF-α的产生,可能参与微小病变型大鼠多柔比星肾病蛋白尿的形成,且IL-18Ab对其有部分治疗作用.%Objective To investigate the relationship of interleukin - 18( IL - 18) and IL - 18 antibody ( IL - 18Ab) in the adriamycin (ADR) - induced model of minimal change nephritic syndrome(MCNS).Methods Thirty rats were divided randomly into 3 groups: normal control group,non - treatment group and treatment group.Wistar rats were induced to the model of MCNS by single injection of ADR(6.5 mg·kg- 1 ) though tail vena.The rats in non - treatment group and normal control group were induced by injection of equal volume of physiological saline solution.Urine protein was measured on the 1st,14th ,28th and 42nd day.Serum total cholesterol ( TC ), triacylglycerol (TG), serum total protein (TP), albumin ( Alb ), urea nitrogen ( BUN ), creatinine (SCr), IL - 18, interferon

  5. Efficacy and safety of low-dose chlorambucil in nephrotic patients with idiopathic membranous nephropathy.

    Science.gov (United States)

    Idasiak-Piechocka, Ilona; Oko, Andrzej; Łochyńska-Bielecka, Katarzyna; Skrobańska, Beata

    2009-01-01

    This observational study aimed to evaluate the results of treatment with low-dose chlorambucil in combination with corticosteroids in patients with idiopathic membranous nephropathy (iMGN) and nephrotic syndrome. Thirty-two patients with nephrotic syndrome and biopsy-proven iMGN were included in the study. At presentation, 9 patients were found to be in stage 1, 13 patients in stage 2 and 10 patients in stage 3 chronic kidney disease. In all patients, i.v. methylprednisolone pulses (500 mg/day for 3 days) were administered, followed by oral prednisone at an initial dose of 1 mg/kg per day, tapered gradually after 8 weeks to the maintenance dose of 5 mg/day after 6 months, and chlorambucil 2 mg twice daily for 6 months. Complete remission of nephrotic syndrome was obtained in 14 patients (47.3%) and partial remission in 16 patients (50%). Two patients relapsed after 1 year of treatment. We did not record any severe side effects in treated patients, except glucose intolerance in 4 subjects on high corticosteroid doses. Immunosuppressive treatment with corticosteroids and low-dose chlorambucil seems to be effective and well tolerated in nephrotic patients with iMGN. (c) 2009 S. Karger AG, Basel.

  6. The Italian Society for Pediatric Nephrology (SINePe) consensus document on the management of nephrotic syndrome in children: Part I - Diagnosis and treatment of the first episode and the first relapse.

    Science.gov (United States)

    Pasini, Andrea; Benetti, Elisa; Conti, Giovanni; Ghio, Luciana; Lepore, Marta; Massella, Laura; Molino, Daniela; Peruzzi, Licia; Emma, Francesco; Fede, Carmelo; Trivelli, Antonella; Maringhini, Silvio; Materassi, Marco; Messina, Giovanni; Montini, Giovanni; Murer, Luisa; Pecoraro, Carmine; Pennesi, Marco

    2017-04-21

    This consensus document is aimed at providing an updated, multidisciplinary overview on the diagnosis and treatment of pediatric nephrotic syndrome (NS) at first presentation. It is the first consensus document of its kind to be produced by all the pediatric nephrology centres in Italy, in line with what is already present in other countries such as France, Germany and the USA. It is based on the current knowledge surrounding the symptomatic and steroid treatment of NS, with a view to providing the basis for a separate consensus document on the treatment of relapses. NS is one of the most common pediatric glomerular diseases, with an incidence of around 2-7 cases per 100000 children per year. Corticosteroids are the mainstay of treatment, but the optimal therapeutic regimen for managing childhood idiopathic NS is still under debate. In Italy, shared treatment guidelines were lacking and, consequently, the choice of steroid regimen was based on the clinical expertise of each individual unit. On the basis of the 2015 Cochrane systematic review, KDIGO Guidelines and more recent data from the literature, this working group, with the contribution of all the pediatric nephrology centres in Italy and on the behalf of the Italian Society of Pediatric Nephrology, has produced a shared steroid protocol that will be useful for National Health System hospitals and pediatricians. Investigations at initial presentation and the principal causes of NS to be screened are suggested. In the early phase of the disease, symptomatic treatment is also important as many severe complications can occur which are either directly related to the pathophysiology of the underlying NS or to the steroid treatment itself. To date, very few studies have been published on the prophylaxis and treatment of these early complications, while recommendations are either lacking or conflicting. This consensus provides indications for the prevention, early recognition and treatment of these complications

  7. Analysis of prognosis of patients with nephrotic syndrome%肾病综合征的临床治疗预后分析

    Institute of Scientific and Technical Information of China (English)

    贺建军

    2014-01-01

    Objective:To discuss the prognosis of patients with nephritic syndrome treated by combination of Chinese traditional and western medicine .Methods:60 adults with nephritic syndrome were divided randomly into observation group and control group with 30 pa-tients each group.All the patients were treated by hormonotherapy .Observation group were treated by self -made Chinese medicine .The course was 4 weeks.Results:The total effective rates of observation group and control group were 93.3%and 73.3%respectively.The difference between two groups was obviously (P<0.05).The SCr and BUN in two groups were obviously reduced after treatment .The differences between before and after treatment in each group were obviously .The differences of SCr and BUN between two groups after treatment were obviously(P<0.05).Conclusion:Combination of Chinese traditional and western medicine for patients with nephritic syn -drome could improve the renal function and prognosis .%目的:探讨肾病综合征的临床中西药结合治疗预后。方法:肾病综合征成人患者60例根据随机抽签法分为观察组与对照组各30例,所有患者都采用激素治疗,在此基础上观察组加用自拟中药汤治疗,疗程为4周。结果:观察组与对照组总有效率分别为93.3%和73.3%,2组对比差异明显(P<0.05)。治疗后2组的SCr和BUN值都明显下降,与治疗前对比差异明显,同时上述值在治疗后的组间对比差异明显(P<0.05)。结论:肾病综合征在临床上采用中西药结合治疗能改善肾功能,提高预后疗效。

  8. Minimal-Change Disease Secondary to Borrelia burgdorferi Infection.

    Science.gov (United States)

    Kwiatkowska, Ewa; Gołembiewska, Edyta; Ciechanowski, Kazimierz; Kędzierska, Karolina

    2012-01-01

    Lyme borreliosis is a chronic illness caused by tick-transmitted spirochete Borrelia burgdorferi. Borreliosis can be extremely threatening if it is not diagnosed and treated in early stages. Kidneys are not typically involved in the disease. However, in infected dogs, Lyme nephritis is present in 5-10% of cases. It is associated with rapidly progressing renal failure. Histopathological examination shows mesangial proliferative glomerulonephritis with diffuse tubular necrosis, (Dambach et al. (1997)). In available literature, there were reports of human's glomerulonephritis associated with Borrelia burgdorferi infection. These cases refer to membranous and mesangial proliferative glomerulonephritis (Kirmizis and Chatzidimitriou (2010), Zachäus (2008), and Kirmizis et al. (2004)). In this paper, we present the case of minimal-change disease (MCD) as a result of Borrelia burgdorferi infection.

  9. Minimal-Change Disease Secondary to Borrelia burgdorferi Infection

    Directory of Open Access Journals (Sweden)

    Ewa Kwiatkowska

    2012-01-01

    Full Text Available Lyme borreliosis is a chronic illness caused by tick-transmitted spirochete Borrelia burgdorferi. Borreliosis can be extremely threatening if it is not diagnosed and treated in early stages. Kidneys are not typically involved in the disease. However, in infected dogs, Lyme nephritis is present in 5–10% of cases. It is associated with rapidly progressing renal failure. Histopathological examination shows mesangial proliferative glomerulonephritis with diffuse tubular necrosis, (Dambach et al. (1997. In available literature, there were reports of human's glomerulonephritis associated with Borrelia burgdorferi infection. These cases refer to membranous and mesangial proliferative glomerulonephritis (Kirmizis and Chatzidimitriou (2010, Zachäus (2008, and Kirmizis et al. (2004. In this paper, we present the case of minimal-change disease (MCD as a result of Borrelia burgdorferi infection.

  10. Clinical significance of determining urinary podocalyxin level in children with primary nephrotic syndrome%检测原发性肾病综合征患儿尿足细胞标志蛋白的临床意义

    Institute of Scientific and Technical Information of China (English)

    刘涛; 张碧丽; 李莉

    2012-01-01

    Objective To study the clinical significance of urinary podoeyte marker protein podocalyxin (PCX) in the diagnosis of primary nephrotic syndrome (PNS) in children and the evaluation of disease severity. Methods PCX levels in the first morning urine were measured by turhidimetric immunoassay (TIA) in 175 children, including 53 children with acute PNS[36 cases of simple nephrotic syndrome (NS) and 17 cases of nephritic NS], 56 children with PNS in the remission stage (relapsed: 42 cases) and 66 healthy children ( control group). Twenty-four hour urinary protein was measured in the 53 children with acute PNS. The optimal operating points for the diagnosis of acute PNS and nephritic NS were determined using the receiver-operating characteristic curve ( ROC carve). Results Significant increasd levels of urinary PCX were found in children with acute PNS compared with those in the remission stage and the control group ( P < 0.01 ). A positive correlation was found between urinary PCX and 24 hour urinary protein in children with acute PNS (r = 0.39,P<0.01). In children with acute PNS, urinary PCX levels were significantly higher in the nephritic NS group than in the simple NS group (P < 0.05). In children in the remission stage, a significant increase in levels of urinary PCX was found in children who had relapsed compared with those who had not (P <0.05). The area under the ROC curve for the diagnosis of acute PNS and nephritic NS was 0.915 and 0.784 respectively. The optimal operating point for the diagnosis of acute PNS and nephritic NS was 7.97 and 10. 2S ng/mL respectively, with a sensitivity and specificity of 81.1% and 93.4% respectively for acut? PNS and of 94.1% and52.8% respectively for nephritic NS. Conclusions The quantitative detection of urinary PCX is useful in the evaluation of podoeyte dynamic changes. It is helpful in the diagnosis of acute PNS and in the differentiation of nephritic NS and simple NS.%目的 探讨检测尿液中足细胞标志蛋

  11. Effect observation of application of low molecular heparin in the treatment of children with primary nephrotic syndrome and hypercoagulative state%应用低分子肝素治疗儿童原发性肾病综合征高凝状态的效果观察

    Institute of Scientific and Technical Information of China (English)

    曲春霞

    2015-01-01

    Objective:To explore the treatment effect of low molecular heparin in the treatment of children with primary nephrotic syndrome and hypercoagulative state.Methods:38 patients with primary nephrotic syndrome and hypercoagulative state were selected.They were randomly divided into the control group and the treatment group with 19 cases in each group.The control group was treated with oral dipyridamole,and the treatment group was given subcutaneous injection of low molecular weight heparin on the basis of the control group,then we compared the effect of two groups.Results:At 1 week and 2 weeks after treatment, compared with before treatment,the plasma levels of FB and DD of the two groups were significantly decreased(P<0.05);complete remission time of urinary protein in the treatment group was significantly lower than that of the control group(P<0.05).Conclusion:The treatment effect of low molecular heparin in the treatment of children with primary nephrotic syndrome and hypercoagulative state was significant.%目的:探讨低分子肝素对儿童原发性肾病综合征并发高凝状态时的治疗效果。方法:收治合并高凝状态的原发性肾病综合征患儿38例,随机分为对照组和治疗组,各19例。对照组口服双嘧达莫,治疗组在对照组基础上加皮下注射低分子肝素钙,比较两组效果。结果:治疗1周及2周时两组血浆FB和DD均较治疗前有明显下降(P<0.05),治疗组患儿尿蛋白完全缓解时间明显低于对照组(P<0.05)。结论:低分子肝素对儿童原发性肾病综合征并发高凝状态时的治疗效果显著。

  12. Clinical Pharmacists'Practice in Pharmaceutical Care for One Nephrotic Syndrome Patient with Gastrointestinal Infection%临床药师参与1例肾病综合征并发胃肠道感染患者的药学监护

    Institute of Scientific and Technical Information of China (English)

    唐景财; 阎敏

    2016-01-01

    OBJECTIVE:To explore the role of clinical pharmacists in clinical rational drug use for nephrotic syndrome patients . METHODS: To introduce the treatment process of one nephrotic syndrome patient with gastrointestinal infection participated by the clinical pharmacists .RESULTS & CONCLUSIONS: Proper drugs were selected through the discussion of clinical pharmacists and clinicians , the medication scheme was adjusted according to the patient's conditions and clinical efficacy of drugs , and necessary pharmaceutical care was provided for the patient . Meanwhile , the patient's urinary protein decreased without any complications like thrombus , and the gastrointestinal tract infection had been cured .Therefore , clinical pharmacists‘ participation in the formulation of the related treatment regimen can improve patient's compliance and clinical treatment efficacy and also promote medical quality .The clinical pharmacists play an important role in the rational drug use for nephrotic syndrome patients .%目的:探讨临床药师在肾病综合征患者合理用药中的作用。方法:介绍临床药师参与1例肾病综合征并发胃肠道感染患者的治疗过程。结果与结论:经临床药师与医师讨论并选择合适的药物,根据患者病情变化及药物疗效及时调整用药方案,可为患者提供必要的药学服务。结果,该患者尿蛋白减少,未出现血栓等并发症,胃肠道感染治愈。由此表明,临床药师参与患者有关治疗方案的制订,可提高患者的用药依从性和临床治疗效果,医疗质量提升,临床药师在肾病综合征患者合理用药中发挥了重要作用。

  13. Treatment of primary nephrotic syndrome with glucocorticoid and alfacalcidol%泼尼松联合阿法骨化醇治疗原发性肾病综合征的临床观察

    Institute of Scientific and Technical Information of China (English)

    徐鹏; 张骅; 陈星华

    2011-01-01

    目的 观察泼尼松联合阿法骨化醇治疗原发性肾病综合征的临床疗效.方法 选择2008年1月-2009年10月住院及门诊治疗的原发性肾病综合征患者56例,随机分为2组:C组28例常规泼尼松治疗,T组28例常规泼尼松联合阿法骨化醇(0.5 μg/d)治疗,比较2组治疗前后临床症状的改善,血清钙、磷、甲状旁腺激素(PTH)、25-(OH)2D3以及1,25-(OH)2D3水平以及尿蛋白定量变化.结果 治疗3、6及12个月后,T组缓解率均明显高于C组(P<0.05).治疗6个月,C组患者24 h尿蛋白下降(P<0.05),血钙、血磷、血25-(OH)2D3及1,25-(OH)2D3、PTH水平稍有改善,但差异无统计学意义(P﹥0.05);T组血钙、25-(OH)2D3及1,25-(OH)2D3水平上升(P<0.05),24 h尿蛋白、PTH水平下降(P<0.05),且与C组治疗后相比,差异具有统计学意义(P<0.05),而血磷水平无明显变化(P>0.05).结论 泼尼松联合阿法骨化醇治疗原发性肾病综合征疗效明显.%Objective To investigate the effect of prednisone combined with alfacalcidol in patients with primary nephrotic syndrome( PNS ).Methods 56 hospitalized patients of PNS were chosen from January, 2008 to October, 2009.They were randomly allocated into two groups: 28 patients ( Group C ) were treated with regular prednisone, and other 28 patients ( Group T ) were treated with regular prednisone combined with alfacalcidol ( 0.5 μg/d ).The level of serum calcium, phosphonium, parathyroid hormone ( PTH ), 25-( OH )2 D3, 1,25-( OH )2 D3 and proteinuria were compared before and after the therapy in each group.Results 3 months, 6 months and 12 months after treatment, the effect in T group was better than C group( P < 0.05 ).The levels of calcium,25-( OH )2 D3 and 1,25-( OH )2 D3 were decreased( all P < 0.05 ), the level of PTH was increased in Group C and Group T.After prednisone treatment, the above parameters did not change obviously in Group C.But with the treatment of prednisone combined with alfacalcidol, the levels

  14. 儿童原发性肾病综合征并发尿路感染的临床分析%Primary Nephrotic Syndrome Complicated by Urinary Tract Infection in Children

    Institute of Scientific and Technical Information of China (English)

    陈文波; 许文峰; 苏永智

    2014-01-01

    目的:研究小儿原发性肾病综合征(primary nephrotic syndrome,PNS)并发尿路感染(urinary tract infec-tion,UTI)的临床特征,分析其危险因素和对抗生素的敏感情况,为儿童 PNS 并发 UTI 的诊断和治疗提供参考依据。方法收集62例 PNS 并发 UTI 患儿(UTI 组)的临床资料,分析其实验数据特点,并与同期60例符合 PNS诊断标准的非 UTI 患儿(非 UTI 组)进行对比。结果62例并发 UTI 的 PNS 患儿中,有症状者14例(占22.5%),无症状性菌尿者48例(占77.5%)。UTI 组患儿24 h 尿蛋白定量、血肌酐(Scr)等指标水平均显著高于非 UTI 组(P <0.01),而血浆清蛋白(Alb)、免疫球蛋白 IgG(IgG)等指标水平均显著低于非 UTI 组(均 P <0.01);UTI 组患儿中激素联合免疫抑制剂治疗患儿28例(45.1%),显著高于非 UTI 组[13例(21.6%)](P <0.05)。致病菌以革兰阴性杆菌为主(75.00%),大肠杆菌为优势菌群,其他依次为肺炎克雷伯杆菌、变形杆菌等,氨苄青霉素、哌拉西林等抗生素已被大多数病原菌耐受。结论PNS 合并 UTI 临床过程隐匿,大量蛋白尿、低 IgG 血症、低 Alb 水平、肾功能不全、使用免疫抑制剂是导致 PNS 易发生 UTI 的因素。应对并发 UTI 的易感因素进行早期干预,治疗时应根据病原学和药敏结果用药。%Objective To study the clinical features,risk factors and sensitivity to antibiotics of primary nephrotic syndrome(PNS)complicated by urinary tract infection(UTI)in children,and to provide reference basis for the diagnosis and treatment of PNS complicated by UTI.Methods Clinical data of 62 children with PNS and UTI(UTI group)were collected to analyze the labora-tory features,and were compared with data of 60 non-UTI children who met diagnostic criteria for PNS(non-UTI group).Results Among the 62 children with PNS and UTI,14 (22

  15. 桂西地区壮族人肾素-血管紧张素系统基因多态与原发性肾病综合征的相关分析%Correlation analysis between rennin-angiotensin system gene polymorphism and primary nephrotic syndrome in Zhuang population of West of Guangxi Zhuang Autonomous Region

    Institute of Scientific and Technical Information of China (English)

    尤燕舞; 林栩; 王洁; 杨发奋

    2012-01-01

    Objective To analysis the correlation between three key genes of angiotensin II type 1 receptor (AT,R) gene Al 166C polymorphism, angiotensin I converting enzyme (ACE) gene insertion/deletion (I/D) polymorphism and angiotensinogen (AGT) gene M235T polymorphism in rennin-angiotensin system (RAS) and primary nephrotic syndrome in Zhuang population of West of Guangxi Zhuang Autonomous Region. Methods 112 cases of patients with primary nephrotic syndrome in Zhuang population of West of Guangxi Zhuang Autonomous Region (nephropathy group) and 150 cases of normal controls (control group) were selected for case-control study. Polymerase chain reaction-restriction fragment length polymorphism technique, direct polymerase chain reaction technique and DNA sequencing method were used to detect the RAS ACE gene I/D polymorphism, AT(R gene A1166C polymorphism and AGT gene M235T polymorphism. The distributions of 3 genes polymorphism in the two group were statistically compared. Results The ACE gene I/D polymorphism in the nephropathy group was different from the normal control group, D genotype and D allele in the nephropathy group were accounted for a significant advantage (P 0.05). Conclusion D allele of ACE I/D polymorphism is one of the predisposing factors of primary nephrotic syndrome in Zhuang population of West of Guangxi Zhuang Autonomous Region. This study has not found the relevance between AT,R gene A1166C polymorphisms, AGT gene M235T polymorphisms and primary nephrotic syndrome.%目的 分析桂西地区壮族人肾素-血管紧张素系统中血管紧张素Ⅱ1型受体(AT1R)基因A1166C多态、血管紧张素Ⅰ转化酶(ACE)基因插入/缺失(I/D)多态及血管紧张素原(AGT)基因M235T多态与原发性肾病综合征的相关性.方法 选取112例桂西地区壮族原发性肾病综合征患者(肾病组)及150例正常对照者(对照组)进行病例对照研究,采用聚合酶链反应-限制性片段长度多态性技术、直接聚合酶链反应

  16. Relationship between epstein-barr virus and children primary nephrotic syndrome%EB病毒感染与儿童原发性肾病综合征关系的研究

    Institute of Scientific and Technical Information of China (English)

    李素娥; 唐骏; 吴乐天

    2014-01-01

    Objective To explore the relationship between epstein-barr virus (EBV)infection and children primary nephrotic syndrome. Methods Blood samples and renal biopsy tissue of 66 children with PNS were analyzed as PNS group,the EBV DNA、VCA antibodies (VCA-IgM、VCA-IgG),atypical lympho-cyte were detected.Samples of 68 children without kidney disease were enrolled as control group. The differ-ence between two groups about EBV DNA,VCA-IgM、VCA-IgG,atypical lymphocyte in blood were com-pared. EBV DNA、hemic-renal EBV DNA in renal tissue were also compared between simple type and nephrit-ic type of PNS. Results The positive rates of VCA-IgM and VCA-IgG in PNS group were higher than those of control group (P<0.05). And compared with simple type of PNS,the positive rates of VCA-IgMand VCA-IgG in nephritic type of PNS were significantly higher (P<0.05 ). The positive rate of hemic-renal EBV DNA expression in nephritic type of PNS was also higher than that of simple type of PNS (P<0.05 ). Conclusion There may be some relations between EBV and children PNS,especially nephritic type of PNS.%目的:探讨EB病毒感染与儿童原发性肾病综合征(PNS)的关系。方法收集66例PNS患儿血液和肾组织标本行EB病毒DNA和EB病毒壳抗原抗体VCA-IgM、VCA-IgG以及异形淋巴细胞检测,并将同期住院的68例非肾脏疾病患儿作为对照,比较两组患儿血EB病毒DNA、VCA-IgM、VCA-IgG以及异形淋巴细胞的差异,并比较单纯型及肾炎型PNS患儿的肾EB病毒DNA、血-肾EB病毒DNA表达差异。结果 PNS 患儿血 VCA-IgM及 VCA-IgG 的阳性率高于对照组(P 均<0.05)。与单纯型 PNS 相对比,肾炎型 PNS 患儿血 VCA-IgM及 VCA-IgG 的阳性率更高(P 均<0.05);且肾炎型PNS患儿血、肾组织均有EB病毒DNA表达者的阳性率高于单纯型PNS患儿(P<0.05)。结论 EB病毒与儿童PNS可能有一定关联,特别是与肾炎型PNS。

  17. Molecular study of idiopathic nephrotic syndrome

    OpenAIRE

    Bullich Vilanova, Gemma

    2016-01-01

    Aquesta tesi és una contribució al coneixement de les bases moleculars de la síndrome nefròtica idiopàtica concretament, la nefropatia membranosa idiopàtica i la síndrome nefròtica córtico-resistent (SNCR) o glomeruloesclerosis segmentària i focal (GESF). La primera part d'aquesta tesi presenta l'associació de determinades variants genètiques tan amb el risc de desenvolupar nefropatia membranosa idiopàtica com amb el curs clínic de la malaltia, en població espanyola. Els nostres resultats mos...

  18. 我院肾病综合征超说明书用药分析%Analysis of Off-label Drug Use in Nephrotic Syndrome in Our Hospital

    Institute of Scientific and Technical Information of China (English)

    黄婧; 于西全; 陈威

    2016-01-01

    Objective:To analyze the rationality of off-label drug use in nephritic syndrome prescriptions to provide scientific basis for clinical rational drug use and further regulate the off-label drug use in our hospital. Methods:Totally 1 908 outpatient prescriptions of nephritic syndrome selected from our hospital during November 2014 to April 2015 were analyzed, and all the off-label drugs were listed . The rationality of the off-label drug use was analyzed and evaluated by searching the related kidney disease guidelines and litera-tures. Results:The off-label drug use of tacrolimus capsules, mycophenolate mofetil capsules, cyclophosphamide for injection, ciclos-porin soft capsules, dipyridamole tablets, hydroxychloroquine sulfate tablets and warfarin sodium tablets were recommended by domes-tic and overseas guidelines with better evidence of evidence-based medicines. Tripterygium glycosides tablets and leflunomide tablets were supported by the literatures on clinical studies at home and abroad. The above 9 kinds of off-label drugs were rational drug use. Lumbrokinase enteric-coated capsules, salvia miltiorrhiza and ligustrazine injection and pidotimod dispersible tablets were reported only by a handful of journals. Bacteria lysate capsules and boric acid powder had no related information support, which belonged to the em-pirical prescriptions of physicians. The above 5 kinds of off-label drugs were not rational drug use. Conclusion:It is a widely existing phenomenon that the medication in nephrotic syndrome is beyond the instruction, the most of off-label drug use are reasonable, and cli-nicians should prescribe medicines carefully. Our hospital needs to further standardize the management of off-label drug use supported by higher evidence in order to improve the level of clinical rational drug use and the reasonable rate of prescriptions.%目的::分析我院治疗肾病综合征超说明书用药的合理性,为临床合理用药提供科学依据,并进一

  19. 原发性肾病综合征并发急性肾损伤患者血清及肾组织中NGAL的表达及意义%Expression of NGAL in serum and renal tissues of patients with primary nephrotic syndrome and acute kidney injury

    Institute of Scientific and Technical Information of China (English)

    刘静; 李绍梅; 薛雯; 温文龙; 杨林; 杨万霞; 王建荣

    2012-01-01

    目的:检测原发性肾病综合征(PNS)患者血清及肾组织中中性粒细胞明胶酶相关脂质运载蛋白(NGAL)的水平,探讨PNS合并急性肾损伤(AKI)时患者血清及肾组织中NGAL浓度的变化.方法:72例PNS患者根据病理结果分为:(1)PNS合并急性肾小管坏死(ATN)组 15例,其中微小病变(MCD)合并ATN 10例,系膜增生(MsPGN)合并ATN 5例;(2)PNS不合并ATN组 57例,其中MCD组 24例,膜性肾病(MN)组23例和MsPGN 组10例.15例健康体检者的血液及5例正常肾组织作为正常对照组.采用ELISA检测血清NGAL的水平;采用免疫组化法检测肾组织中NGAL的表达.结果:(1)PNS患者血清NGAL水平及肾组织中NGAL表达明显高于正常对照组(P0.05);血清中NGAL与肾组织NGAL的表达呈正相关(P<0.01).结论:血清NGAL可能作为判断原发性肾病综合征并发急性肾损伤的早期、无创、敏感指标.%AIM; To observe the changes of neutrophil gelatinase -associated lipocalin (NGAL) level in ser-um and renal tissues of the patients with primary nephrotic syndrome (PNS) and acute kidney injury (AKI). METH-ODS : Seventy - two PNS patients were selected in the study and divided into 2 groups according to the pathological results of renal biopsy. The patients in PNS + AKI group included 15 cases of PNS with acute tubular necrosis ( ATN) , in which there were 10 cases of minimal change disease ( MCD) with ATN and 5 cases of mesangial proliferative glomerulonephritis ( MsPGN) with ATN. The patients in PNS alone group included 57 cases of PNS without ATN. According to the pathologi-cal types, they were divided into MCD group (24 cases) , membranous nephropathy (MN) group (23 cases) and MsPGN group (10 cases). Serum samples from 15 healthy persons and 5 cases of normal renal tissues were used as controls. The serum levels of NGAL were detected by ELISA. The distribution and expression of NGAL in the renal tissues were observed by immunohistochemical method. RESULTS: ( 1) The serum

  20. Long-term effects of cyclosporine A in children with steroid-resistant idiopathic nephrotic syndrome and outcomes of the patients%长疗程环孢素A治疗儿童激素耐药型肾病综合征的疗效和预后

    Institute of Scientific and Technical Information of China (English)

    曹琦; 黄文彦; 徐虹; 周利军

    2008-01-01

    Objective To observe the effects of long-term cyclosparine A(CsA)treatment in 20 children with steroid-resistant idiopathic nephrotic syndrome(SRNS)and analyse the relevant influencing factom of CsA therapy.Methods Twenty children with SRNS received CsA therapy for 2 years between February 2001 and October 2006 in the Department of Nephmlogy.The mean age of children at initiation of CsA therapy was 5.5 years(30 months to 12 years).The initial renal histology showed minimal change (MCNS)in 15 patients,focal segmental glomerulosclerosis(FSGS)in 4 patients and mesangial proliferative with a trough level of 40-70 μg/L wfs applied to mainrain remission for 1 year.Liver function,serum albumin,serum cholesterol,serum creatinine,urinary NAG/Cr,24 h urinary protein excretion and CsA whole blood trough level of the patients were monitoted every one or three months.Results (1)Complete remission(proteinuria≤0.1 g/d or negative by dipstick for 3 consecutive days),partial remission of 100μg/L)were observed in 65%,20% and 15%,respectively.Eleven patients who had complete remission discontinued CsA,in 5(45%)patients the disease relapsed,and resumption of CsA thempy was foilowed by remission in th.ree of them.(2)MCNS showed a 93%response to CsA therapy while non-MCNS showed a 60% response,but the difference was not significant(P>0.05).(3)Hypertrichosis,gingival hyperplasia and hypertension occurred in 75%,25%and 10%of the patients,respectively.Two patients were found to have renal impairment(>30% rise of serum creatinine)and recovered in 2 weeks. Four patients showed a rise of urinary NAG/Cr.The central nervous system adverse event occurred in 2 cages.Post-thempy biopsies performed in 3 patients(2 with FSGS and one with MCNS)did not show any relevant tubulointerstitial fibrosis.Two patients with FSGs ofthe twenty cases progressed into end-stage renal failure.Conclusions CsA treatment was confirmed to be effective in children with SRNS.Rehal fibrosis was rare in patients

  1. Expression of L-FABP in serum, urine and renal tissues in the patients with primary nephrotic syndrome%原发性肾病综合征患者血清、尿肝型脂肪酸结合蛋白的变化及其在肾组织表达的意义

    Institute of Scientific and Technical Information of China (English)

    施婧; 尹忠诚; 唐敏; 杨晶; 张颖; 李胜开

    2014-01-01

    Objective By detecting liver-type fatty acid binding protein (L-FABP) levels in serum, urine and L-FABP expression in renal tissues in patients with primary nephrotic syndrome ( PNS), to investigate its relationship with pathological type and PNS with acute kidney injury (AKI).Methods 45 patients with PNS were divided into 2 groups according to whether they had acute tubular necrosis ( ATN) .There were 37 cases of PNS without ATN and AKI ( PNS without AKI group ) .According to the pathological types , they were further divided into mesangial proliferative glomerolonephritis (MsPGN) group (13 cases), minimal change disease (MCD) group (5 cases), focal segmental glo-merulosclerosis (FSGS) group (9 cases), and membranous nephropathy (MN) group (10 cases).There were 8 cases of PNS with ATN and AKI (PNS with AKI group ) , in which all cases were MCD .Serum and urine of 10 healthy subject who received routine physical checkup and 10 normal renal tissues located far from renal tumor in patients with nephritic tumor served as control groups .The levels of L-FABP in serum and urine were detected by enzyme -linked immunosor-bent assay ( ELISA) .Immunohistochemical staining was used to detect the expression of L -FABP in renal tissues .Re-sults ①The levels of L-FABP in serum and urine and expression of L -FABP in renal tissues in patients with PNS were significantly higher than those of the control group (P0.05).②The serum and urine levels of L-FABP and the expression of L -FABP in renal tissures were enhanced in the PNS with AKI group compared with the PNS without AKI group (P<0.05).③The receiver operator characteristic curve′s area under the curve (ROC-AUC) of serum, urine L-FABP and serum creatinine (SCr) for diagnosis of AKI were 0.910, 0.973, 0.812, respectively.④The serum and urine levels of L -FABP were positively correlated with SCr , blood urea nitrogen (BUN), 24 h urine pro-tein (24hUpro) and expression of L -FABP in renal tissures (r=0.331~0.764, P<0

  2. PLA2R antibody levels and clinical outcome in patients with membranous nephropathy and non-nephrotic range proteinuria under treatment with inhibitors of the renin-angiotensin system.

    Directory of Open Access Journals (Sweden)

    Elion Hoxha

    Full Text Available Patients with primary membranous nephropathy (MN who experience spontaneous remission of proteinuria generally have an excellent outcome without need of immunosuppressive therapy. It is, however, unclear whether non-nephrotic proteinuria at the time of diagnosis is also associated with good prognosis since a reasonable number of these patients develop nephrotic syndrome despite blockade of the renin-angiotensin system. No clinical or laboratory parameters are available, which allow the assessment of risk for development of nephrotic proteinuria. Phospholipase A2 Receptor antibodies (PLA2R-Ab play a prominent role in the pathogenesis of primary MN and are associated with persistence of nephrotic proteinuria. In this study we analysed whether PLA2R-Ab levels might predict development of nephrotic syndrome and the clinical outcome in 33 patients with biopsy-proven primary MN and non-nephrotic proteinuria under treatment with blockers of the renin-angiotensin system. PLA2R-Ab levels, proteinuria and serum creatinine were measured every three months. Nephrotic-range proteinuria developed in 18 (55% patients. At study start (1.2±1.5 months after renal biopsy and time of diagnosis, 16 (48% patients were positive for PLA2R-Ab. A multivariate analysis showed that PLA2R-Ab levels were associated with an increased risk for development of nephrotic proteinuria (HR = 3.66; 95%CI: 1.39-9.64; p = 0.009. Immunosuppressive therapy was initiated more frequently in PLA2R-Ab positive patients (13 of 16 patients, 81% compared to PLA2R-Ab negative patients (2 of 17 patients, 12%. PLA2R-Ab levels are associated with higher risk for development of nephrotic-range proteinuria in this cohort of non-nephrotic patients at the time of diagnosis and should be closely monitored in the clinical management.

  3. PLA2R antibody levels and clinical outcome in patients with membranous nephropathy and non-nephrotic range proteinuria under treatment with inhibitors of the renin-angiotensin system.

    Science.gov (United States)

    Hoxha, Elion; Harendza, Sigrid; Pinnschmidt, Hans; Panzer, Ulf; Stahl, Rolf A K

    2014-01-01

    Patients with primary membranous nephropathy (MN) who experience spontaneous remission of proteinuria generally have an excellent outcome without need of immunosuppressive therapy. It is, however, unclear whether non-nephrotic proteinuria at the time of diagnosis is also associated with good prognosis since a reasonable number of these patients develop nephrotic syndrome despite blockade of the renin-angiotensin system. No clinical or laboratory parameters are available, which allow the assessment of risk for development of nephrotic proteinuria. Phospholipase A2 Receptor antibodies (PLA2R-Ab) play a prominent role in the pathogenesis of primary MN and are associated with persistence of nephrotic proteinuria. In this study we analysed whether PLA2R-Ab levels might predict development of nephrotic syndrome and the clinical outcome in 33 patients with biopsy-proven primary MN and non-nephrotic proteinuria under treatment with blockers of the renin-angiotensin system. PLA2R-Ab levels, proteinuria and serum creatinine were measured every three months. Nephrotic-range proteinuria developed in 18 (55%) patients. At study start (1.2±1.5 months after renal biopsy and time of diagnosis), 16 (48%) patients were positive for PLA2R-Ab. A multivariate analysis showed that PLA2R-Ab levels were associated with an increased risk for development of nephrotic proteinuria (HR = 3.66; 95%CI: 1.39-9.64; p = 0.009). Immunosuppressive therapy was initiated more frequently in PLA2R-Ab positive patients (13 of 16 patients, 81%) compared to PLA2R-Ab negative patients (2 of 17 patients, 12%). PLA2R-Ab levels are associated with higher risk for development of nephrotic-range proteinuria in this cohort of non-nephrotic patients at the time of diagnosis and should be closely monitored in the clinical management.

  4. PLA2R Antibody Levels and Clinical Outcome in Patients with Membranous Nephropathy and Non-Nephrotic Range Proteinuria under Treatment with Inhibitors of the Renin-Angiotensin System

    Science.gov (United States)

    Hoxha, Elion; Harendza, Sigrid; Pinnschmidt, Hans; Panzer, Ulf; Stahl, Rolf A. K.

    2014-01-01

    Patients with primary membranous nephropathy (MN) who experience spontaneous remission of proteinuria generally have an excellent outcome without need of immunosuppressive therapy. It is, however, unclear whether non-nephrotic proteinuria at the time of diagnosis is also associated with good prognosis since a reasonable number of these patients develop nephrotic syndrome despite blockade of the renin-angiotensin system. No clinical or laboratory parameters are available, which allow the assessment of risk for development of nephrotic proteinuria. Phospholipase A2 Receptor antibodies (PLA2R-Ab) play a prominent role in the pathogenesis of primary MN and are associated with persistence of nephrotic proteinuria. In this study we analysed whether PLA2R-Ab levels might predict development of nephrotic syndrome and the clinical outcome in 33 patients with biopsy-proven primary MN and non-nephrotic proteinuria under treatment with blockers of the renin-angiotensin system. PLA2R-Ab levels, proteinuria and serum creatinine were measured every three months. Nephrotic-range proteinuria developed in 18 (55%) patients. At study start (1.2±1.5 months after renal biopsy and time of diagnosis), 16 (48%) patients were positive for PLA2R-Ab. A multivariate analysis showed that PLA2R-Ab levels were associated with an increased risk for development of nephrotic proteinuria (HR = 3.66; 95%CI: 1.39–9.64; p = 0.009). Immunosuppressive therapy was initiated more frequently in PLA2R-Ab positive patients (13 of 16 patients, 81%) compared to PLA2R-Ab negative patients (2 of 17 patients, 12%). PLA2R-Ab levels are associated with higher risk for development of nephrotic-range proteinuria in this cohort of non-nephrotic patients at the time of diagnosis and should be closely monitored in the clinical management. PMID:25313791

  5. Analysis of frequent relapses related factors of primary nephrotic syndrome in children%儿童原发性肾病综合征频反复相关因素分析

    Institute of Scientific and Technical Information of China (English)

    范树颖; 张碧丽; 王文红; 张暄

    2011-01-01

    目的 通过对原发性肾病综合征(PNS)患儿临床资料统计分析,探讨小儿PNS频反复的影响因素.方法 收集2007年1月至2010年2月于我科诊断为PNS的患儿住院及门诊随访病历,对获得的完整临床资料进行分析.结果 245例激素敏感型肾病综合征患儿中,非频反复202例(82.4%),频反复43例(17.6%).单因素分析结果中,首次反复距初治缓解时间<3个月、起病时血总蛋白、胆固醇水平、血清IgE水平在频反复及非频反复两组间差异有统计学意义(P<0.05).多因素非条件logistic回归分析中,首次反复距初治缓解时间<3个月及血清IgE水平升高(>358IU/ml)为频反复的危险因素(P<0.05).引起反复的诱因中,以上呼吸道感染最多见,共194次(65.1%),有诱因的反复发生在1~3月份最多,占30.4%.结论 频反复在正规激素治疗的PNS中所占比例达17.6%,存在冬春季复发高峰的季节性特点.起病时血总蛋白<40g/L、血胆固醇水平>10mmol/L可作为预测PNS易出现频反复的指标.感染是肾病综合征反复的主要诱因,不规则治疗、特应性体质导致反复亦应引起重视.%Objective To explore the influencing factors of frequent relapse primary nephritic syndrome (PNS) in children according to the statistical analysis of their clinical data and laboratory examinations. Method We collected in-hospital and out-patient medical records of children who were diagnosed PNS from Jan. 2007 to Feb. 2010 and whole clinical data were analyzed. Results In 245 cases of children with steroid sensitine nephrotic syndrome, non-frequent replapse (NFR) accounted for 82.4% and frequent relapse (FR) accounted for 17.6%. In univariate analysis, period of first relapse between initial treatment remission within 3 months , level of serum total protein and cholesterol and IgE at onset had statistical significance in FR children and NFR children, which were significant predictors of FR. The logistic

  6. Fulminant ulcerative colitis associated with steroid-resistant minimal change disease and psoriasis: A case report

    OpenAIRE

    Lok, Ka-Ho; Hung, Hiu-Gong; Yip, Wai-Man; Li, Kin-Kong; Li, Kam-Fu; Szeto, Ming-Leung

    2007-01-01

    A 43-year-old Chinese patient with a history of psoriasis developed fulminant ulcerative colitis after immunosuppressive therapy for steroid-resistant minimal change disease was stopped. Minimal change disease in association with inflammatory bowel disease is a rare condition. We here report a case showing an association between ulcerative colitis, minimal change disease, and psoriasis. The possible pathological link between 3 diseases is discussed.

  7. Fulminant ulcerative colitis associated with steroid-resistant minimal change disease and psoriasis: a case report.

    Science.gov (United States)

    Lok, Ka-Ho; Hung, Hiu-Gong; Yip, Wai-Man; Li, Kin-Kong; Li, Kam-Fu; Szeto, Ming-Leung

    2007-08-07

    A 43-year-old Chinese patient with a history of psoriasis developed fulminant ulcerative colitis after immunosuppressive therapy for steroid-resistant minimal change disease was stopped. Minimal change disease in association with inflammatory bowel disease is a rare condition. We here report a case showing an association between ulcerative colitis, minimal change disease, and psoriasis. The possible pathological link between 3 diseases is discussed.

  8. Fulminant ulcerative colitis associated with steroid-resistant minimal change disease and psoriasis: A case report

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    A 43-year-old Chinese patient with a history of psoriasis developed fulminant ulcerative colitis after immunosuppressive therapy for steroid-resistant minimal change disease was stopped. Minimal change disease in association with inflammatory bowel disease is a rare condition. We here report a case showing an association between ulcerative colitis, minimal change disease,and psoriasis. The possible pathological link between 3 diseases is discussed.

  9. Clinical significance of retinol-binding protein detection for diagnosis of nephrotic syndrome in children%视黄醇结合蛋白检测在肾病综合征患儿诊断中的临床意义

    Institute of Scientific and Technical Information of China (English)

    李浩军; 孟秀荣; 董晓妮

    2014-01-01

    Objective To explore the clinical significance of retinol‐binding protein detection for diagnosis of nephrotic syndrome in children .Methods A total of 60 cases of children with nephrotic syndrome were enrolled in the experimental group and divided into two groups (33 cases for the simple type nephrotic syndrome group and 27 cases for the nephritic type nephrotic syndrome group) .And other 30 cases of healthy children were selected as the healthy control group .The levels of retinol‐binding protein ,urea and creatinine were detected and analyzed .Results The levels of serum retinol‐binding protein ,urea and creatinine were higher in two experimental groups than those in healthy control group ,and in the two experimental groups the positive detectable rate of serum retinol‐binding protein was higher than that of urea and creatinine ,all with significant difference(P< 0 .05) .The levels of serum retinol‐binding protein detected after treatment were evidently higher than those detected before treatment(P<0 .05) ,and a certain correlation was found between levels of serum retinol‐binding protein and the clinical feature (r=0 .799 3 ,P<0 .05) .The diagnostic efficiency of retinol‐binding protein was the highest ,followed by urea and creatinine .Conclu‐sion The retinol binding protein detection could be with positive clinical value for the clinical diagnosis and thera‐peutic morniteration of children with nephrotic syndrome .%目的:探讨视黄醇结合蛋白检测在肾病综合征患儿诊断中的临床意义。方法选择2013年1~12月在涿州市中医院确诊为肾病综合征的患儿60例为试验组(单纯型肾病综合征组33例,肾炎型肾病综合征组27例),另选择30名健康儿童设为健康对照组,分别测定其血清视黄醇结合蛋白、尿素和肌酐水平并进行比较分析。结果试验组两组患儿的血清视黄醇结合蛋白、尿素和肌酐浓度水平均高于健康对照组,且试验组

  10. 小剂量多巴胺联合酚妥拉明治疗原发性肾病综合征中重度水肿%Treatment of primary nephrotic syndrome in children with severe edema by phentolamine associated with low-dose dopamine

    Institute of Scientific and Technical Information of China (English)

    银宏伟

    2015-01-01

    Objective To investigate the clinical effects of application of low-dose dopamine combined with phentolamine on primary nephritic syndrome in children with severe edema.Methods One hundred cases of children with primary nephrotic syndrome were randomly divided into observation group and control group,with 50 cases in each group,low-dose dopamine combined phentolamine treatment were used in the observation group; furosemide was used in the control group.The urine volume,edema subsided,fade time and incidence of adverse reactions of the two groups were compared.Results Urine of children in the observation group was significantly higher than that in the control group,edema subsided significantly shorter than that in the control group,the incidence of adverse events was significantly lower than that in the control group,the differences between the two groups were statistically significant (P < 0.05).Conclusions Treatment of primary nephrotic syndrome in children with severe edema by phentolamine associated with low-dose dopamine,can increase urine volume,rapidly swelled and with low side effect,the clinical results are good,should be widely applied.%目的 探讨小剂量多巴胺联合酚妥拉明对原发性肾病综合征中重度水肿的临床疗效.方法 将100例原发性肾病综合征患者随机分为观察组和对照组,每组50例,观察组采用小剂量多巴胺与酚妥拉明联合治疗,对照组采用呋塞米治疗,观察对比两组患者尿量、水肿消退程度、消退时间及不良反应发生率.结果 观察组尿量显著多于对照组,患儿水肿的消退时间显著短于对照组,不良反应发生率显著低于对照组,两组比较差异有统计学意义(P<0.05).结论 采用小剂量多巴胺联合酚妥拉明治疗原发性肾病综合征患儿中重度水肿可增加尿量,迅速消肿且不良反应小,临床效果良好,值得临床推广应用.

  11. Characteristics of Mood Status in School-Aged Children with Nephrotic Syndrome and Their Primary Caregivers%学龄期肾病综合征患儿及其主要照顾者的心境现状

    Institute of Scientific and Technical Information of China (English)

    张大华; 张凡; 贾玉静; 张臻

    2012-01-01

    Objective To investigate the prevalence and characteristics of mood status in children with nephrotic syndrome ( NS ) and their primary caregivers. The correlative factors of the mood status of school - aged children with NS and their primary caregivers were analyzed. Methods There were 43 cases with NS. General information from enrolled subjects was collected by using self - designed questionnaires used in this study. Screening for Child Anxiety Related Emotional Disorders (SCARED) , Depression Self- Rating Scale for Children ( DSRSC), Self - rating Anxiety Scale (SAS) and Self - rating Depression Scale (SDS) were performed on the subjects. Statistical analysis was performed by using SPSS 14.0 software. Results 1. The total positive rates were 32.6% (14/43 cases) and 44.2% (19/43 cases) in children with NS and their primary caregivers, respectively. The scores of anxiety and depression in children with NS and their primary caregivers were higher than those in the general population,and there was significant statistical difference between the primary caregivers and the general population. 2. There was no correlation between sex, age, mothers' educational level, family finance status, family residence, disease relapses during the past 6 months, the primary caregivers' type, the primary caregivers' mood status and the mood status in children with NS. There was correlation among mood status of the primary caregivers, the primary caregivers' type, family residence, and disease relapses during the past 6 months. Conclusions Medical staff should pay attention to the mood status in children with NS and their primary caregivers. It should be recognized and treated as early as possible. The life quality of patients and their family might be improved through individual intervention.%目的 探讨学龄期肾病综合征(NS)患儿及其主要照顾者的心境现状,并分析影响学龄期NS患儿及其主要照顾者心境现状的相关因素.方法 NS患儿43例.采用

  12. Association between angiotensin-converting enzyme 2 gene polymorphisms and childhood primary nephrotic syndrome%ACE2基因多态性与儿童原发性肾病综合征的相关性研究

    Institute of Scientific and Technical Information of China (English)

    邱明瑜; 谢琴芳; 王丽娜; 于力

    2015-01-01

    ObjectiveAngiotensin-converting enzyme 2 (ACE2) gene polymorphisms have been shown to be implicated in hypertension, diabetic nephropathy, and other diseases. However, it remains unclear whether ACE2 gene polymorphisms are involved in the development of primary nephrotic syndrome (PNS) in children. The aim of this study was to assess the association between A9570G polymorphisms of ACE2 gene and PNS in a group of Han children in Guangdong Province, China.MethodsThe genotype distribution and allele frequency of ACE2 gene A9570G in 66 children with PNS and 60 healthy subjects (control group) were analyzed by polymerase chain reaction and restriction fragment length polymorphism.ResultsAllele frequency and genotype distribution showed no signiifcant difference between the PNS and control groups whether in female or in male children (P>0.05). The PNS group was classiifed into the glucocorticoid-sensitive and glucocorticoid-resistant subgroups according to glucocorticoid treatment response. Subgroup analysis revealed that in female children, the frequency of GG genotype was 17% in the glucocorticoid-sensitive group vs 45% in the glucocorticoid-sensitive group (P=0.018); the frequency of G allele was 31% in the glucocorticoid-sensitive group vs 61% in the glucocorticoid-resistant group (P=0.023). In male children, the frequency of G genotype/G allele was 36% in the glucocorticoid-sensitive group vs 64% in the glucocorticoid-resistant group (P=0.017).ConclusionsThere is no clear association between ACE2 gene A9570G polymorphisms and childhood PNS, but ACE2 gene A9570G polymorphisms might be associated with glucocorticoid treatment response in children with PNS. The G allele might be a genetic susceptibility factor of glucocorticoid resistance in children with PNS.%目的:血管紧张素转换酶2(ACE2)基因多态性与高血压病、糖尿病肾病等多种疾病相关,是否参与儿童原发性肾病综合征(PNS)的发病尚不明确,该研

  13. Efficacy and impact on urinary protein of mycophenolate mofetil on primary nephrotic syndrome%霉酚酸酯治疗原发性肾病综合征的临床疗效及对尿蛋白的影响

    Institute of Scientific and Technical Information of China (English)

    崔玉波

    2014-01-01

    目的 研究霉酚酸酯(MMF)治疗原发性肾病综合征的疗效及对尿蛋白的影响.方法 选取原发性肾病综合征患者136例,采用随机数字表法分为MMF组和对照组各68例,对照组行常规治疗,MMF组加用霉酚酸酯治疗.两组均连续治疗6个月后进行疗效评价.结果 MMF组总缓解率(82.8%)显著高于对照组(60.6%)(x2=10.86,P<0.05);两组BUN、血肌酐(Scr)、24h尿蛋白定量均有不同程度降低,MMF组降低更为显著,各项差异具有统计学意义(t =2.50、4.59、11.12,均P<0.05).两组血清白蛋白均上升,但MMF组升高更明显,差异具有统计学意义(t =3.09,P<0.05).结论 霉酚酸酯对原发性肾病综合征及尿蛋白具有疗效显著、副反应小、安全可靠等优点,值得临床推广使用.%Objective To study the effect of mycophenolate mofetil(MMF) on primary nephrotic syndrome and the impact on urinary protein.Methods According to the digital table,136 cases of nephrotic syndrome were randomly divided into MMF group and control group,68 cases in the control group were dealed with conventional treatment,MMF group was treated with MMF.After 6 months,the treatment effects of both groups were evaluated.Results The overall response rate of MMF group was 82.8%,higher than that of control group 60.6% with statistically significant difference (x2 =10.86,P < 0.05),;Blood urea nitrogen(BUN),serum creatinine(Scr),24 h urinary protein of two groups had varying degree of reduction,the indexes of MMF group decreased even more significant (t =2.50,4.59,11.12,all P < 0.05).Serum albumin of the two groups had risen,but MMF group increased more significantly (t =3.09,P < 0.05).Conclusion The advantages of MMF are significant effect,reduction in side effects,safe and reliable on nephrotic syndrome and urinary protein,which is worthy of clinical use.

  14. 自我效能对肾病综合征患者饮食自我管理水平的影响%Study on the effect of self-efficacy on the level of dietary self-management of patients with nephrotic syndrome

    Institute of Scientific and Technical Information of China (English)

    林剑珊; 黄燕林

    2010-01-01

    Objective To study the effect of self-efficacy on the level of dietary self-management of patients with nephrotic syndrome. Methods Data collected from 128 patients with nephrotic syndrome measured by Self-Management Scale and General Self-Efficacy Scale. 65 patients in the experimental group were given self-efficacy training, and the other 63 patients in the control group were given clinical general nursing. After 6months, data were collected again. Results Before intervention, two groups had no significant difference both in self-management and self-efficacy( P > 0.05 ). After intervention, two groups had significant differences both in self-management and self-efficacy ( P < 0.05 ). There was correlation between the scores of self-efficacy and the level of dietary self-management. Conclusions The higher the scores of self-efficacy, the better the level of dietary self-management the patients with nephrotic syndrome got. The self-efficacy training had more advantages in improving the patients'dietary self-management level than clinical general nursing.%目的 探讨自我效能对肾病综合征患者饮食自我管理水平的影响.方法 将128例肾病综合征患者分为实验组(n=65)和对照组(n=63),实验组给予自我效能训练,对照组给予常规指导,干预前后采用肾病综合征患者自我管理行为问卷和一般自我效能感量表对f两组患者进行调查评估.结果 干预前两组患者在饮食自我管理、自我效能的得分情况方面的比较均无统计学意义(P>0.05);干预后实验组患者在饮食自我管理水平方面高于对照组,差异统计学意义(P<0.05);干预后两组患者在自我效能得分情况方面的比较差异有统计学意义(P<0.05);患者自我效能得分值与因子饮食自我管理得分值呈正相关.结论 肾病综合征患者自我效能越高,其饮食自我管理水平就越高.自我效能训练在提高肾病综合征患者饮食自我管理水平方面比传统的常规指导更具有优势.

  15. 甲泼尼龙与泼尼松治疗肾病综合征合并乙型肝炎的疗效及安全性比较%The comparison of the efficacy and safety of methylprednisolone and prednisone in the treatment of nephrotic syndrome complicated with hepatitis B

    Institute of Scientific and Technical Information of China (English)

    王敬

    2012-01-01

    目的 比较甲泼尼龙与泼尼松治疗肾病综合征合并乙型肝炎的临床疗效及安全性.方法 选取肾病综合征合并乙型肝炎患者66例,随机分为对照组和观察组,每组33例.对照组于清晨顿服泼尼松,观察组顿服甲泼尼龙.比较两组患者的临床疗效及肝损害不良反应的发生情况.结果 治疗后两组患者的各指标均比治疗前明显改善(均P<0.05);观察组的总有效率(93.94%)高于对照组(84.85%)(x2=1.44,P>0.05);观察组HBV-DNA转阴率显著高于对照组(x2=4.69,P<0.05);观察组患者肝损害作用显著低于对照组(t =5.62,5.13,P<0.05).结论 甲泼尼龙与泼尼松均能有较治疗肾病综合征合并乙型肝炎,但甲泼尼龙的效果优于泼尼松,对患者肝损害作用较轻.%Objective To compare the clinical efficacy and side effects of liver damage of the methylprednisolone (methylprednisolone tablets ) and prednisone treating nephrotic syndrome compicated with hepatitis B.Methods 66 patients with nephrotic syndrome complicated with hepatitis B were randomly divided into control group and observation group,and each group had 33 cases.The control group was given prednisone in the morning,while the observation group was received methylprednisolone.The clinical efficacy and side effects of liver damage were compared between the two groups.Results After the treatment,the biochemical index significantly improved compared with the pre-treatment( all P < 0.05 ) ;The total effective rate of observation group was 93.94% higher than 84.85% of the control group( x2 =1.44,P > 0.05 ).However,there was no significant difference between the two groups( P >0.05) ;The observation group was significantly higher than the control group in negative rate of HBV-DNA (x2 =4.69,P < 0.05 ) ;The side effects of liver damage in the observation group were lower than the control group (t =5.62,5.13,P < 0.05 ).Conclusion Methylprednisolone and prednisone for

  16. Clinical Observation of Sequential Therapy with Dexamethasone and Prednisone in Treating Adult Primary Nephrotic Syndrome%地塞米松泼尼松序贯疗法治疗成人原发性肾病综合征临床观察

    Institute of Scientific and Technical Information of China (English)

    张万超; 蒲月英; 王程; 左松; 欧阳治

    2013-01-01

    Objective To explore the effect of sequential therapy using dexamethasone and prednisone in treating a-dult primary nephrotic syndrome. Methods 64 patients suffered with primary nephrotic syndrome were randomly divided into 3 groups, which were treated respectively by sequential therapy with dexamethasone and prednisone (observational group, 22 cases), standard treatment with prednisone administrated orally (control group A, 21 cases), and sequential therapy with methylprednisolone and prednisone (control group B, 21 cases). Regression time of edema, disappearing time of urine protein, relieving time of the symptoms, therapeutic effect, as well as incidence of adverse effects were recorded and compared between the different groups. Results Regression time of edema and disappearing time of urine protein of observational group were significantly less than those of control group A (P0. 05). Therapeutic effects between the 3 groups exhibited no statistical differences (P>0. 05). As for relieving time of the symptoms, it was statistically shorter for observational group as compared with control group A (P0, 05), And no differences in incidence of adverse effects were found among the three groups. Conclusion As compared with standard prednisone treatment in tre