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Sample records for nephrotic syndrome due

  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. 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

  3. 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.

  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. 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.

  6. Diagnosis and management of nephrotic syndrome.

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    McCloskey, Oonagh; Maxwell, Alexander P

    2017-02-01

    Nephrotic syndrome is defined by a triad of clinical features: oedema, substantial proteinuria (> 3.5 g/24 hours) and hypoalbuminaemia (syndrome develops following pathological injury to renal glomeruli. This may be a primary problem, with a disease specific to the kidneys, or secondary to a systemic disorder such as diabetes mellitus. The most common cause in children is minimal change glomerulonephritis. In white adults, nephrotic syndrome is most frequently due to membranous nephropathy whereas in populations of African ancestry the most common cause of nephrotic syndrome is focal segmental glomerulosclerosis. Diabetic nephropathy is the most common multisystem disease that can cause nephrotic syndrome. Patients typically present with periorbital oedema (most noticeable in the morning) or dependent pitting oedema (more common later in the day). Proteinuria should be documented by a quantitative measurement e.g. urine protein: creatinine ratio (PCR) or albumin: creatinine ratio (ACR). PCR > 300-350 mg/mmol indicates nephrotic range proteinuria. Urgent referral to a nephrologist (ideally within 2 weeks) is necessary and a renal biopsy is usually performed. This will establish what form of glomerular disease is responsible. Additional tests may be undertaken to assess if nephrotic syndrome is secondary to another disorder e.g. systemic lupus erythematosus or amyloidosis.

  7. Nephrotic and nephritic syndrome in the newborn.

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    Rheault, Michelle N

    2014-09-01

    Glomerular disorders in infancy can include nephrotic and nephritic syndromes. Congenital nephrotic syndrome (CNS) is most commonly caused by single gene mutations in kidney proteins, but may also be due to congenital infections or passive transfer of maternal antibodies that target kidney proteins. Prenatal findings of increased maternal serum α-fetoprotein and enlarged placenta suggest CNS. Neonatal nephritis is rare; its causes may overlap with those of CNS and include primary glomerulonephritis, systemic disease, infections, and transplacental transfer of maternal antibodies. These syndromes in the neonate can cause significant morbidity and mortality, making urgent diagnosis and treatment necessary. Copyright © 2014 Elsevier Inc. All rights reserved.

  8. Nephrotic syndrome associated with meningioma

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    P P Zachariah

    2013-01-01

    Full Text Available A 58-year-old man presented with recurrent frontal meningioma and nephrotic syndrome. Renal biopsy could not be done in view of the rapid neurological deterioration. The patient underwent surgical resection of the tumor. Within 4 weeks, the edema decreased, serum albumin improved, and proteinuria decreased spontaneously. At three months of followup, the patient had attained complete remission of nephrotic state.

  9. Latest research progress on acute nephrotic syndrome

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    Satinder Kakar

    2017-01-01

    Full Text Available Etiology of nephrotic syndrome is somewhat complex in nature. It may range from primary to secondary forms. Nephrotic syndrome patients often need immunosuppressive treatment although it has some side effects and may lead to renal disease which may be acute or severe. This review deals with herbal treatment and other recent approaches for treating symptoms of nephrotic syndrome.

  10. Glucocorticoid pharmacogenetics in pediatric idiopathic nephrotic syndrome.

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    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. 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.

  12. Ethnic Differences in Childhood Nephrotic Syndrome

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    Chanchlani, Rahul; Parekh, Rulan S.

    2016-01-01

    Nephrotic syndrome is a common glomerular disease in children with significant variability in both incidence and steroid responsiveness among various ethnic groups. The average incidence of nephrotic syndrome is 2–16.9 per 100,000 children worldwide. Understanding the variability by ethnicity may point to potential factors leading to nephrotic syndrome, which remains elusive, and may highlight factors accounting for differences in medication response. The emerging role of genetic factors asso...

  13. NEPHROTIC SYNDROME steroid sensitive renal condition

    African Journals Online (AJOL)

    Nephrotic syndrome is primarily a paediatric disorder and is 15 times more common in children than adults. The incidence is 2–3/100,000 children per year, and the majority of affected children will have steroid- sensitive minimal change disease. The characteristic features of nephrotic syndrome are heavy proteinuria.

  14. Case report: nephrotic syndrome associated with a total hydatidiform mole.

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    Komatsuda, A; Nakamoto, Y; Asakura, K; Yasuda, T; Imai, H; Miura, A B

    1992-05-01

    The authors describe a 51-year-old Japanese woman who developed nephrotic syndrome in association with a total hydatidiform mole. The nephrotic syndrome remitted completely following hysterectomy. A renal biopsy performed before the operation showed diffuse mesangial cell proliferation of a moderate degree, and thickened capillary walls with focal and segmental subendothelial deposits, as well as circumferential mesangial interposition. Occasional foci of the mesangiolysis were also observed. Immunofluorescence microscopy revealed granular staining of IgM along the glomerular capillary walls in a fringe pattern. A review of the literature revealed that this patient appears to be the first case of nephrotic syndrome associated with a total mole, although there have been two cases of nephrotic syndrome due to preeclamptic nephropathy associated with a partial or transitional mole.

  15. The pediatric nephrotic syndrome spectrum: Clinical homogeneity and molecular heterogeneity

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    Schachter, Asher D.

    2004-01-01

    Idiopathic nephrotic syndrome is the most common glomerular disorder of childhood. Recurrence of nephrotic syndrome immediately following renal transplantation is rapid, results in a high rate of graft loss, and represents the most severe form of nephrotic syndrome. This review discusses the molecular heterogeneity of pediatric nephrotic syndrome across the spectrum of disease activity. A schema is offered for a molecular approach to pediatric nephrotic syndrome, including immune-mediated and...

  16. Congenital nephrotic syndrome

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    ... syndrome . It occurs mostly in families of Finnish origin and develops shortly after birth. It is inherited, ... amount of protein leaking into the urine Diuretics ("water pills") to remove excess fluid Nonsteroidal anti-inflammatory ...

  17. 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.

  18. 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.

  19. Neuroblastoma presenting with the nephrotic syndrome.

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    Zheng, H L; Maruyama, T; Matsuda, S; Satomura, K

    1979-08-01

    A case of neuroblastoma in a 4-yr-old boy presenting with the nephrotic syndrome is reported. Nether thrombosis nor stenosis of the renal veins and the inferior vena cava was present. Electron microscopy revealed lumps of subepithelial deposits as well as thickening and tortuosity of the glomerular basement membrane compatible with membranous nephropathy. It is postulated that deposition of neuroblastoma-associated immune complexes on the glomerular basement membrane-was responsible for the development of the nephrotic syndrome in this patient. Five pediatric cases of nephrotic syndrome associated with extrarenal neoplasia were collected from the world literature and are briefly discussed.

  20. Congenital nephrotic syndrome.

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    Begolli, Mirije; Begolli, Ilir; Gojani, Xhenane; Arenliu-Qosaj, Fatime; Berisha, Merita

    2011-01-01

    The aim of this case is to present a case of a two month old female with congenital nephritic syndrome, which is very rare. On admission, the baby showed marked edema and distended abdomen. She was diagnosed and treated with daily albumin infusions, antibiotics, diuretic, gamma globulin replacement, ACEI and NSAIDs. Parents were informed about the nature of the disease, prognosis, and advised for further medical care in a more advanced kidney transplantation centre. This was the first treatment of this condition in the Pediatric Clinic in Kosovo and it presented a challenge for us.

  1. Cytomegalovirus infection can mimic genetic nephrotic syndrome: a case report.

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    Hogan, Julien; Fila, Marc; Baudouin, Véronique; Peuchmaur, Michel; Deschênes, Georges; Niel, Olivier

    2015-09-22

    Nephrotic syndrome is a relatively rare but serious condition in children. Infantile nephrotic syndrome often has a genetic origin; the treatment is then symptomatic, with a poor prognosis, and a rapid evolution to chronic kidney disease. However, non-genetic infantile nephrotic syndrome has been identified. Here we report for the first time in a child a nephrotic syndrome as the sole clinical expression of a cytomegalovirus infection. The patient was 5 months old when he presented with a nephrotic syndrome. An exhaustive genetic testing was conducted and came back negative. A viral work-up only showed a positive cytomegalovirus PCR. Antiviral treatment lead to a complete remission of the nephrotic syndrome, with no requirement for steroid therapy. Renal function remained normal throughout follow-up. Nephrotic syndrome should always be carefully investigated in children. This observation reinforces the connection between viral infections and pediatric nephrotic syndrome, sparking more controversy about an infectious origin to childhood nephrotic disease.

  2. Management of childhood onset nephrotic syndrome.

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    Gipson, Debbie S; Massengill, Susan F; Yao, Lynne; Nagaraj, Shashi; Smoyer, William E; Mahan, John D; Wigfall, Delbert; Miles, Paul; Powell, Leslie; Lin, Jen-Jar; Trachtman, Howard; Greenbaum, Larry A

    2009-08-01

    The therapeutic approach to childhood nephrotic syndrome is based on a series of studies that began with an international collaborative effort sponsored by the International Study of Kidney Disease in Children in 1967. The characteristics of children presenting with nephrotic syndrome have changed over recent decades with greater frequency of the challenging condition focal segmental glomerulosclerosis and a greater prevalence of obesity and diabetes mellitus, which may be resistant to glucocorticoids in the former and exacerbated by long-term glucocorticoid therapy in the latter 2 conditions. The Children's Nephrotic Syndrome Consensus Conference was formed to systematically review the published literature and generate a children's primary nephrotic syndrome guideline for use in educational, therapeutic, and research venues.

  3. Immunology of idiopathic nephrotic syndrome.

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    Colucci, Manuela; Corpetti, Giorgia; Emma, Francesco; Vivarelli, Marina

    2018-04-01

    The pathogenesis of idiopathic nephrotic syndrome (INS) is as yet unknown, but several lines of evidence indicate that the immune system may play a crucial pathogenic role in non-genetic INS. The most important of these are, first, the effectiveness of therapy based on immunosuppression and, second, a vast body of data derived both from experimental models and from patient studies that implicate T cells and more recently B cells as major players in INS pathogenesis. However, recent findings also suggest a direct role of podocytes as drivers of the disease process, and the interplay between the glomerulus and the immune system is still being elucidated. In this review we provide an overview of current knowledge on the role of different components of the immune system in determining disease. Advances in our understanding of the pathogenesis of INS may help drive new, more tailored therapeutic approaches.

  4. Cardiac Findings in Congenital Nephrotic Syndrome

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    Shamsi Ghaffari

    2011-08-01

    Full Text Available Introduction: Congenital nephrotic syndrome is a severe debilitating problem associated with extra renal manifestation such as diverse cardiac findings. Methods: During four years 6 cases diagnosed as congenital nephritic syndrome in base of definitive criteria their documents reviewed and echocardiographic evaluation has been done for all with or with-out cardiac sign or symptoms, results gathered and expressed as incidence. Results: All cases have some grades of structural or functional defects from simple form like as tricus-pid regurgitation to complex defects. It may be run in consecutively in siblings of a family from non consanguine parents. Conclusion: Pulmonary stenosis may occur in all parts subvalvualr, valvular and peripheral parts of pulmonary artery, left ventricular hypertrophy and mitral regurgitation observed in some, moderate tricuspid regurgitation observed in half of cases due to pulmonary hypertension or right ventricular hypertrophy due to pulmonary stenosis.

  5. Cardiac Findings in Congenital Nephrotic Syndrome

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    Malaki, Majid; Ghaffari, Shamsi; Ghaffari, Samad; Rastkar, Bahaman; Goldust, Mohammad; Malaki, Pouya; Sahar, Behnam

    2011-01-01

    Introduction Congenital nephrotic syndrome is a severe debilitating problem associated with extra renal manifestation such as diverse cardiac findings. Methods During four years, 6 cases diagnosed as congenital nephritic syndrome in base of definitive criteria their documents reviewed and echocardiographic evaluation has been done for all with or without cardiac sign or symptoms, results gathered and expressed as incidence. Results All cases have some grades of structural or functional defects from simple form like as tricuspid regurgitation to complex defects. It may be run in consecutively in siblings of a family from non consanguine parents. Conclusion Pulmonary stenosis may occur in all parts subvalvualr, valvular and peripheral parts of pulmonary artery, left ventricular hypertrophy and mitral regurgitation observed in some, moderate tricuspid regurgitation observed in half of cases due to pulmonary hypertension or right ventricular hypertrophy due to pulmonary stenosis. PMID:24250960

  6. Urinary indices during relapse of childhood nephrotic syndrome.

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    Iyengar, A A; Kamath, N; Vasudevan, A; Phadke, K D

    2011-07-01

    Sodium retention is the hallmark of idiopathic nephrotic syndrome (INS). Sodium retention could be secondary to activation of renin-angiotensin-aldosterone axis or due to an intrinsic activation of Na(+)K(+) ATPase in the cortical collecting duct. Urine potassium/urine potassium + urine sodium (UK(+)/UK(+) + UNa(+)) is a surrogate marker for aldosterone activity and can be useful in differentiating primary sodium retention from secondary sodium retention in children with INS. This was a cross-sectional study of children with INS, presenting to our center from June 2007 to June 2008. Children were categorized into those with steroid responsive and steroid nonresponsive nephrotic syndrome. One hundred and thirty-four children with nephrotic syndrome were analyzed. The FeNa(+) was significantly lower during relapse than in remission but no such difference was observed with UK(+)/UK(+) + UNa(+). The values of FeNa(+) and UK(+)/UK(+) + UNa(+) across various categories of nephrotic syndrome were similar. Correlating FeNa(+) and UK(+)/UK(+) + UNa(+) with cut-off of 0.5 and 60%, respectively, we found 50% of steroid responsive children and 36% of steroid nonresponders having a corresponding UK(+)/UK(+) + UNa(+) of nephrotic syndrome.

  7. Rituximab for childhood refractory nephrotic syndrome.

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    Iijima, Kazumoto

    2011-10-01

    Several therapies including immunosuppressive agents have been shown to be effective and safe for frequently relapsing nephrotic syndrome/steroid-dependent nephrotic syndrome (FRNS/SDNS) and steroid-resistant nephrotic syndrome in children. It is evident, however, that a substantial number of children are still refractory to treatment despite these therapies. Rituximab is a chimeric monoclonal antibody, which inhibits CD20-mediated B-cell proliferation and differentiation. It was first introduced for the treatment of B-cell non-Hodgkin's lymphoma and was subsequently administered to patients with autoimmune diseases, such as rheumatoid arthritis, lupus erythematosus, or immunocomplex glomerulonephritis. Recently, a number of case reports and non-controlled clinical trials have suggested that rituximab may be effective for children with refractory nephrotic syndrome. Controlled prospective trials, however, are required to establish the value of rituximab in refractory nephrotic syndrome. The purpose of the present study was therefore to evaluate the efficacy and safety of rituximab in childhood-onset refractory nephrotic syndrome. The Research Group of Childhood-onset Refractory Nephrotic Syndrome (RCRNS) conducted a randomized, double-blind, placebo-controlled, multi-center clinical trial (RCRNS-01) and an open-label, multi-center, pharmacokinetic clinical trial (RCRNS-02). These two trials were investigator-initiated, registration-directed clinical trials designed to apply Ministry of Health, Labour and Welfare approval for the use of rituximab for childhood-onset refractory FRNS/SDNS in Japan. RCRNS-01 could be the first study to clarify whether rituximab is effective and safe for childhood-onset refractory FRNS/SDNS. © 2011 The Author. Pediatrics International © 2011 Japan Pediatric Society.

  8. [Physiopathology and treatment of nephrotic syndrome complications].

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    Ducloux, Didier

    2011-02-01

    The nephrotic syndrome is defined by a urinary protein excretion exceeding 3g per day, associated with hypoalbuminaemia (syndrome are multiple, essentially dominated by sodium retention and oedema formation. The oedema physiopathology is related to both increased capillary permeability and primary activation of the Na/K pump in the collect duct. Other complications of the nephrotic syndrome include thromboembolic complications, dyslipidaemia, and infections. The treatment of these complications represents an important part of the general management of the nephritic syndrome. Copyright © 2010 Association Société de néphrologie. Published by Elsevier SAS. All rights reserved.

  9. Rituximab for nephrotic syndrome in children.

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    Iijima, Kazumoto; Sako, Mayumi; Nozu, Kandai

    2017-04-01

    Idiopathic nephrotic syndrome is the most common chronic glomerular disease in children. At least 20 % of children with this syndrome show frequent relapses and/or steroid dependence during or after immunosuppressive therapies, a condition defined as complicated frequently relapsing/steroid-dependent nephrotic syndrome (FRNS/SDNS). Approximately 1-3 % of children with idiopathic nephrotic syndrome are resistant to steroids and all immunosuppressive agents, a condition defined as refractory steroid-resistant nephrotic syndrome (SRNS); these SRNS children have a high risk of end-stage renal failure. Rituximab, a chimeric anti-CD20 monoclonal antibody, has been shown to be effective for patients with complicated FRNS/SDNS and refractory SRNS. This review describes the recent results of rituximab treatment applied to pediatric nephrotic syndrome, as well as those of our recent study, a multicenter, double-blind, randomized, placebo-controlled trial of rituximab for childhood-onset complicated FRNS/SDNS (RCRNS01). The overall efficacy and safety of rituximab for this disease are discussed.

  10. Rare case of nephrotic syndrome: Schimke syndrome.

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    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.

  11. [Thromboembolic complications of childhood nephrotic syndrome].

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    Gargah, Tahar; Abidi, Kamal; Nourchene, Khelil; Zarrouk, Chokri; Lakhoua, Mohamed R

    2012-02-01

    Vascular thrombosis in the childhood nephrotic syndrome is a rare event. It poses major diagnostic and therapeutic problems. The prognosis depends on early diagnosis and on precocious anticoagulation. The risk of extension of thrombosis and pulmonary embolism is real. To consider the major thrombotic events associated with childhood nephrotic syndrome and to establish an appropriate preventive approach based on objective clinical and laboratory parameters. This is a retrospective study of all cases of patients suffering from idiopathic nephrotic syndrome, during a period of 20 years, starting from January 1990 to December 2009. We selected six patients with vascular thrombosis. The diagnosis was confirmed by appropriate radiological investigation. Six cases of vascular thrombosis were identified among 260 cases of nephrotic syndrome collected during the period study. Patients are divided into five boys and one girl. The mean age was 13.3 years. The localisation of thrombosis is venous in all cases. One patient presented a massively fatal pulmonary embolism. Sinovenous thrombosis has been objectified in 3 patients who were all treated with a good therapeutic response. vascular thrombosis remains one of the most serious complications of nephrotic syndrome in children. Early diagnosis and precocious anticoagulation are essential for preventing the extension of thrombosis.

  12. Ethnic differences in childhood nephrotic syndrome

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    Rahul eChanchlani

    2016-04-01

    Full Text Available Nephrotic syndrome is a common glomerular disease in children with significant variability in both incidence and steroid responsiveness among various ethnic groups. The average incidence of nephrotic syndrome is 2-16.9 per 100,000 children worldwide. Understanding the variability by ethnicity may point to potential factors leading to nephrotic syndrome, which remains elusive, and may highlight factors accounting for differences in medication response. The emerging role of genetic factors associated with steroid responsive and steroid resistant forms of nephrotic syndrome within an ethnic group can provide insight into potential biological mechanisms leading to disease. For example, among African Americans, the risk variants in APOL1 are associated with a more than 10-fold increase in risk of focal segmental glomerulosclerosis and high-risk carriers have a 2-fold greater risk of progression to end stage renal disease. Ongoing collaborative studies should consider capturing data on self-reported ethnicity to understand differences in incidence and outcomes. In the future, the availability of whole genome data will provide an excellent opportunity for new clinical and translational research in childhood nephrotic syndrome and lead to better understanding of the disease.

  13. Ethnic Differences in Childhood Nephrotic Syndrome.

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    Chanchlani, Rahul; Parekh, Rulan S

    2016-01-01

    Nephrotic syndrome is a common glomerular disease in children with significant variability in both incidence and steroid responsiveness among various ethnic groups. The average incidence of nephrotic syndrome is 2-16.9 per 100,000 children worldwide. Understanding the variability by ethnicity may point to potential factors leading to nephrotic syndrome, which remains elusive, and may highlight factors accounting for differences in medication response. The emerging role of genetic factors associated with steroid responsive and steroid-resistant forms of nephrotic syndrome within an ethnic group can provide insight into potential biological mechanisms leading to disease. For example, among African-Americans, the risk variants in APOL1 are associated with a more than 10-fold increase in risk of focal segmental glomerulosclerosis and high-risk carriers have a twofold greater risk of progression to end-stage renal disease. Ongoing collaborative studies should consider capturing data on self-reported ethnicity to understand differences in incidence and outcomes. In the future, the availability of whole-genome data will provide an excellent opportunity for new clinical and translational research in childhood nephrotic syndrome and lead to a better understanding of the disease.

  14. Dyslipidaemia in nephrotic syndrome: mechanisms and treatment

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    Agrawal, Shipra; Zaritsky, Joshua J.; Fornoni, Alessia; Smoyer, William E.

    2018-01-01

    Nephrotic syndrome is a highly prevalent disease that is associated with high morbidity despite notable advances in its treatment. Many of the complications of nephrotic syndrome, including the increased risk of atherosclerosis and thromboembolism, can be linked to dysregulated lipid metabolism and dyslipidaemia. These abnormalities include elevated plasma levels of cholesterol, triglycerides and the apolipoprotein B containing lipoproteins VLDL and IDL; decreased lipoprotein lipase activity in the endothelium, muscle and adipose tissues; decreased hepatic lipase activity; and increased levels of the enzyme PCSK9. In addition, there is an increase in the plasma levels of immature HDL particles and reduced cholesterol efflux. Studies from the past few years have markedly improved our understanding of the molecular pathogenesis of nephrotic syndrome associated dyslipidaemia, and also heightened our awareness of the associated exacerbated risks of cardiovascular complications, progressive kidney disease and thromboembolism. Despite the absence of clear guidelines regarding treatment, various strategies are being increasingly utilized, including statins, bile acid sequestrants, fibrates, nicotinic acid and ezetimibe, as well as lipid apheresis, which seem to also induce partial or complete clinical remission of nephrotic syndrome in a substantial percentage of patients. Future potential treatments will likely also include inhibition of PCSK9 using recently developed anti PCSK9 monoclonal antibodies and small inhibitory RNAs, as well as targeting newly identified molecular regulators of lipid metabolism that are dysregulated in nephrotic syndrome. PMID:29176657

  15. Nephrotic syndrome in infants and children: pathophysiology and management.

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    Downie, Mallory L; Gallibois, Claire; Parekh, Rulan S; Noone, Damien G

    2017-11-01

    Nephrotic syndrome is defined by nephrotic-range proteinuria (≥40 mg/m 2 /hour or urine protein/creatinine ratio ≥200 mg/mL or 3+ protein on urine dipstick), hypoalbuminaemia (nephrotic syndrome of childhood, and includes a brief overview of the congenital forms.

  16. AKI in Children Hospitalized with Nephrotic Syndrome.

    Science.gov (United States)

    Rheault, Michelle N; Zhang, Lei; Selewski, David T; Kallash, Mahmoud; Tran, Cheryl L; Seamon, Meredith; Katsoufis, Chryso; Ashoor, Isa; Hernandez, Joel; Supe-Markovina, Katarina; D'Alessandri-Silva, Cynthia; DeJesus-Gonzalez, Nilka; Vasylyeva, Tetyana L; Formeck, Cassandra; Woll, Christopher; Gbadegesin, Rasheed; Geier, Pavel; Devarajan, Prasad; Carpenter, Shannon L; Kerlin, Bryce A; Smoyer, William E

    2015-12-07

    Children with nephrotic syndrome can develop life-threatening complications, including infection and thrombosis. While AKI is associated with adverse outcomes in hospitalized children, little is known about the epidemiology of AKI in children with nephrotic syndrome. The main objectives of this study were to determine the incidence, epidemiology, and hospital outcomes associated with AKI in a modern cohort of children hospitalized with nephrotic syndrome. Records of children with nephrotic syndrome admitted to 17 pediatric nephrology centers across North America from 2010 to 2012 were reviewed. AKI was classified using the pediatric RIFLE definition. AKI occurred in 58.6% of 336 children and 50.9% of 615 hospitalizations (27.3% in stage R, 17.2% in stage I, and 6.3% in stage F). After adjustment for race, sex, age at admission, and clinical diagnosis, infection (odds ratio, 2.24; 95% confidence interval, 1.37 to 3.65; P=0.001), nephrotoxic medication exposure (odds ratio, 1.35; 95% confidence interval, 1.11 to 1.64; P=0.002), days of nephrotoxic medication exposure (odds ratio, 1.10; 95% confidence interval, 1.05 to 1.15; Pchildren with nephrotic syndrome. Nephrotoxic medication exposure was common in this population, and each additional nephrotoxic medication received during a hospitalization was associated with 38% higher risk of AKI. AKI was associated with longer hospital stay after adjustment for race, sex, age at admission, clinical diagnosis, and infection (difference, 0.45 [log]days; 95% confidence interval, 0.36 to 0.53 [log]days; Pchildren hospitalized with nephrotic syndrome and should be deemed the third major complication of nephrotic syndrome in children in addition to infection and venous thromboembolism. Risk factors for AKI include steroid-resistant nephrotic syndrome, infection, and nephrotoxic medication exposure. Children with AKI have longer hospital lengths of stay and increased need for intensive care unit admission. Copyright © 2015 by the

  17. Congenital nephrotic syndrome. Gallium-67 imaging

    International Nuclear Information System (INIS)

    Trepashko, D.W.; Gelfand, M.J.; Pan, C.C.

    1988-01-01

    Congenital nephrotic syndrome is a rare disorder. Heavy proteinuria, hypoalbuminemia, and edema occur during the first 3 months of life. Initial cases were reported from Finland and sporadic cases have occurred elsewhere. Finnish cases demonstrated an autosomal recessive inheritance pattern; currently, Finnish and non-Finnish types are recognized. The clinical course consists of failure to thrive, frequent infections, declining renal function, and early death by age 4 years from sepsis or uremia. Recently renal transplantation has improved the prognosis of patients with this disease. An abnormal Ga-67 scan in a case of congenital nephrotic syndrome is presented

  18. Nephrotic syndrome: a rare cause of acute coronary syndrome in a child

    International Nuclear Information System (INIS)

    Khan, J.A.; Shamsi, F.

    2012-01-01

    Patients with nephrotic syndrome are at risk of developing thrombosis in both veins and arteries. Various manifestations in different organs have been reported. Thrombi in heart seen, associated with multi organ thrombosis have been reported on autopsy earlier, but only once in a living patient with nephrotic syndrome. Here, we report a 13 years old boy with steroid-resistant nephrotic syndrome, who developed an asymptomatic but potentially hazardous large intracardiac thrombus. The child developed nephrotic syndrome at the age of 9 years and had multiple recurrences. At the age of 13 years, he developed myocardial infarction (MI) due to embolism from a large intracardiac thrombus. Later on, he was treated with heparin and warfarin anticoagulation. (author)

  19. Idiopathic nephrotic syndrome in South African children.

    African Journals Online (AJOL)

    Abstract. Background: Different histo-pathological types and treatment response patterns of Idiopathic nephrotic syndrome (INS) have been associated with differences in ethnicity and geographical location. Objective: To provide an update on the steroid response and renal histo-pathological pattern in children treated for ...

  20. Childhood Nephrotic Syndrome in Ilorin | Adedoyin | Nigerian ...

    African Journals Online (AJOL)

    A cohort of 17 children (12M:5F), aged 3-16 years, with nephrotic syndrome seen at the University of Ilorin Teaching Hospital, Ilorin, between January 1995 and December 1998 were studied. Renal biopsies were performed in eight patients. Five (63 per cent) of the biopsies showed focal mesangial proliferative ...

  1. Ofatumumab for the treatment of childhood nephrotic syndrome.

    Science.gov (United States)

    Wang, Chia-Shi; Liverman, Rochelle Schmidt; Garro, Rouba; George, Roshan Punnoose; Glumova, Anastacia; Karp, Alana; Jernigan, Stephanie; Warshaw, Barry

    2017-05-01

    Ofatumumab is a humanized anti-CD20 monoclonal antibody that has recently garnered interest as a potential therapeutic agent for nephrotic syndrome. We report our center's experience in administering ofatumumab to five pediatric patients with idiopathic nephrotic syndrome. Between March 2015 and November 2016, five patients were treated with ofatumumab. One patient had post-transplant recurrent focal segmental glomerulosclerosis (FSGS) which had been resistant to plasmapheresis and numerous immunosuppressive agents. Four patients had nephrotic syndrome in their native kidneys, one with initial steroid-resistant disease and the others with subsequent development of steroid resistance. Two of the patients were treated with a desensitization protocol after experiencing hypersensitivity reactions to ofatumumab. One patient did not complete ofatumumab treatment due to infusion reactions. Of the four remaining patients, three achieved complete remission after treatment, and one achieved partial remission. One of the patients achieving complete remission represents the first reported case of successful treatment of post-transplant recurrent FSGS using ofatumumab. Two patients who received ofatumumab with our desensitization protocol were able to complete their treatments after initially experiencing hypersensitivity reactions. Ofatumumab may be an effective treatment for refractory childhood nephrotic syndrome and post-transplant recurrent FSGS. A desensitization protocol may be helpful to address hypersensitivity reactions.

  2. NEPHROTIC SYNDROME: PAST, PRESENT AND FUTURE

    Directory of Open Access Journals (Sweden)

    M. S. Ignatova

    2017-01-01

    Full Text Available This literature review is focused to change our ideas about the etiology, pathogenesis and treatment tactics of the nephrotic syndrome  in recent decades. The change in the treatment outcomes of the primary nephrotic syndrome in connection with the emergence of new  therapy technologies, is shown. Features of the course, examination and therapy of congenital and infantile nephrotic syndrome and  the possibility of the debut of a nephrotic syndrome associated with various gene mutations and at an older age are presented. Principal differences in diagnostic and therapeutic approaches are accentuated depending on the cause of the development of the disease.  Modern syndromological and pathogenetic methods of therapy of primary nephrotic syndrome are presented, and the immediate opportunities for the introduction of new treatment technologies based on the use of monoclonal antibodies, are shown.

  3. HOMOCYSTEINE AND KIDNEY'S FUNCTIONAL STATUS OF THE CHILDREN WITH THE NEPHROTIC SYNDROME

    Directory of Open Access Journals (Sweden)

    L.T. Faizova

    2008-01-01

    Full Text Available The authors presented the data on the homo cysteine serum concentration in the children with nephrotic syndrome. They established the high rate of hyper homo cysteinemia in the nephritic syndrome, but the degree of the homocysteine concentration increase is largely due to the kidney's functional status rather than the activity of the disease. They also discuss the pathogenetic role of hyper homo cysteinemia in the event of the chronic nephritic pathology.Key words: homo cysteine, nephrotic syndrome, children.

  4. A new 'tac' for childhood nephrotic syndrome.

    Science.gov (United States)

    Becknell, Brian; Greenbaum, Larry A; Smoyer, William E

    2012-11-01

    Calcineurin inhibitors (CNIs) are frequent first-line agents in children with steroid-resistant nephrotic syndrome (SRNS). However, limited randomized controlled trial (RCT) data are available comparing CNIs with alternative therapies. Gulati and colleagues report their experience with tacrolimus versus cyclophosphamide in childhood SRNS. Their results establish clear superiority of tacrolimus over cyclophosphamide and give further proof that RCTs in childhood SRNS are both feasible and vital for improving the standard of care.

  5. 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

  6. Childhood nephrotic syndrome--current and future therapies.

    Science.gov (United States)

    Greenbaum, Larry A; Benndorf, Rainer; Smoyer, William E

    2012-06-12

    The introduction of corticosteroids more than 50 years ago dramatically improved the prognosis of children with nephrotic syndrome. Corticosteroids remain the standard initial treatment for children with this disease, but a considerable proportion of patients do not respond and are therefore at risk of progressing to end-stage renal disease. Because of this risk, new therapeutic strategies are needed for steroid-resistant nephrotic syndrome. These strategies have historically focused on identifying effective alternative immunosuppressive agents, such as ciclosporin and tacrolimus, yet evidence now indicates that nephrotic syndrome results from podocyte dysfunction. Even conventional immunosuppressive agents, such as glucocorticoids and ciclosporin, directly affect podocyte structure and function, challenging the 'immune theory' of the pathogenesis of childhood nephrotic syndrome in which disease is caused by T cells. This Review summarizes the currently available treatments for childhood nephrotic syndrome, and discusses selected novel pathways in podocytes that could be targeted for the development of next-generation treatments for children with this syndrome.

  7. Cellulitis as complication of nephrotic syndrome in a pediatric patient

    Science.gov (United States)

    Siregar, R. S.; Daulay, K. R.; Siregar, B.; Ramayani, O. R.; Eyanoer, P. C.

    2018-03-01

    Nephrotic syndrome is a chronic disease that may act as a risk for other major infection in skin, respiratory and urinary tract, while also increasingthe chance for other diseases, like peritonitis, meningitis, and cellulitis. Cellulitis is often caused by Streptococcus β-hemolytic, Staphylococcus aureus, and Escherichia coli. The clinical features of cellulitis marked with redness rash and well-defined borders, pain pressure and swelling. Hypoalbuminemia which occurs due to proteinuria occurred in this patient acts as a risk factor for cellulitis. It has been reported the case of cellulitis as one of the complications of the nephrotic syndrome in the pediatric patient. The treatment has been given to the patient such as antibiotics and supportive therapy and also planned albumin substitution.

  8. Nephrotic Syndrome in Children: From Bench to Treatment

    Directory of Open Access Journals (Sweden)

    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.

  9. Nephrotic Syndrome After Hematopoietic Stem Cell Transplant: Outcomes in Iran.

    Science.gov (United States)

    Saddadi, Fereshteh; Alidadi, Ali; Hakemi, Monir; Bahar, Babak

    2017-02-01

    Patients undergoing hematopoietic stem cell transplant have an elevated incidence of acute renal failure. However, the incidence of nephritic syndrome due to graft-versus-host disease is growing and is independently associated with chronic renal disease after this procedure. We conducted a prospective study to examine the risk of chronic kidney disease in glomerulopathy patients following hematopoietic stem cell transplant with a follow-up of 10 years. In our follow-up of 14 patients (4 men and 10 women) who were diagnosed with nephrotic syndrome after hematopoietic stem cell transplant, in 10 patients (71%), biopsy showed membranous nephropathy associated with graft-versus-host disease. The remaining 4 patients had focal segmental glomerulosclerosis, membranoproliferative glomerulonephritis, or minimal change disease. All patients were treated with angiotensin receptor blockers, cyclosporine (Neoral), and prednisolone. During follow-up, 6 patients (43%) had heavy proteinuria and a rise in serum creatinine, and 1 patient (7%) needed hemodialysis. Eleven patients (79%) achieved complete remission of nephrotic syndrome, 5 (36%) remained hypertensive, and 3 (21%) did not respond to therapy.. The early diagnosis of nephrotic syndrome should be considered after hematopoietic stem cell transplant, and therapeutic outcome measures should be in place in advance. If this is done, we found that patients' response to treatment can be optimal, and their renal function and overall survival can improve.

  10. Cerebral venous thrombosis in a child with nephrotic syndrome ...

    African Journals Online (AJOL)

    Cerebral venous thrombosis (CVT), a rare and life threatening complication of nephrotic syndrome, has a variable and non-specific presentation, posing diagnostic challenges. We describe a case of CVT in a Sierra Leonean child with nephrotic syndrome who was successfully treated for the condition despite the resource ...

  11. 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

  12. Superior sagittal sinus thrombosis: a rare complication of nephrotic syndrome.

    Directory of Open Access Journals (Sweden)

    Tullu M

    1999-10-01

    Full Text Available A two and half year-old-male child, known case of steroid responsive nephrotic syndrome presented with fever and vomiting of acute onset. He was diagnosed to have superior sagittal sinus thrombosis on a contrast computerised tomographic scan of brain. Recovery was complete without anticoagulant therapy. Superior sagittal sinus thrombosis is an extremely rare complication of nephrotic syndrome.

  13. Nephrotic syndrome in Namibian children | van Buuren | South ...

    African Journals Online (AJOL)

    The aim of this study was to document the clinical, pathological and outcome features of nephrotic syndrome in Namibian children. Subjects. 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.

  14. Childhood nephrotic syndrome as seen at the Federal Medical ...

    African Journals Online (AJOL)

    Background: Nephrotic syndrome still remains one of the commonest indications for paediatric renal consult worldwide. The Nephrology unit in our centre is a young one. This study reviews the cases of nephrotic syndrome we managed over a three year period. Aim and Objective: To document its prevalence among other ...

  15. 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.

  16. 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.

  17. 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.

  18. 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.

  19. Renal biopsy in childhood nephrotic syndrome

    International Nuclear Information System (INIS)

    Hafeez, F.; Rasool, F.; Hamid, T.

    2002-01-01

    Objective: To study the histopathological pattern in childhood nephrotic syndrome who underwent biopsy, and the safety of the procedure. Design: A prospective study. Patients and Duration of Study: Nephrology Department, The Children Hospital and the Institute of Child Health, Lahore form July 1999 to June 2001. Subjects and method: Ultrasound guided percutaneous renal biopsy was done in nephrotics, who were (a) steroid resistant (SR), (b) wit signs of steroid toxicity (c) deranged renal functions, hypocomplementemia, gross haematuria (d) signs of systemic disease. Patients with congenital nephrosis were not included. The biopsy material was examined under light microscope after staining with H and E and PAS. Results: Thirty-five patients with male to female ratio of 2.5:1 in the age range of 1-1/2 years 14 years (mean 6.9 years) underwent renal biopsy. Fifteen (42.9)% were SRNS (Steroid resistant nephritic syndrome), while 7 (20%) had signs of steroid toxicity and deranged renal functions each. Gross haematuria and signs suggestive of systemic disease were present in 3 (8.6%) Patients in each group. The most predominant histopathology was measangioproliferative in 12 (34.2%) followed by MPGN (Membranoproliferative glomerulonephritis) in 7 (20%), MCNS (Minimal change nephrotic syndrome) in 6 (17.1%), membranous nephropathy in 3 (8.85%), FSGS (Focal segmental glomeurlosclerosis) and focal proliferative GN (Glomerulonephritis) was seen in 1 (2.85%) each. Among the steroid resistant patients the most common histopathology was mesangioproliferative GN in 7 (46.6%) followed by MCNS and FSGS in 2 (13.2%) each. Overall complication rate was 17.2% in 6 with gross haematuria and flank pain seen in 2 (5.7%), while small perirenal haematoma and urinary retention occurred in 1 (2.85%) patients in each group. Conclusion: Percutaneous renal biopsy is a safe procedure with minimal risk of complications, it helps the nephrologist to find out the underlying histopathology for the

  20. Use of high-dose prednisolone to overcome rifampicin-induced corticosteroid non-responsiveness in childhood nephrotic syndrome

    Directory of Open Access Journals (Sweden)

    H Barman

    2016-01-01

    Full Text Available Inducing remission in nephrotic children on anti-tubercular therapy is difficult due to the increased metabolism of prednisolone induced by rifampicin. We report a child with nephrotic syndrome treated successfully with an increased dose of steroids without discontinuing anti-tubercular therapy.

  1. Use of high-dose prednisolone to overcome rifampicin-induced corticosteroid non-responsiveness in childhood nephrotic syndrome.

    Science.gov (United States)

    Barman, H; Dass, R; Duwarah, S G

    2016-01-01

    Inducing remission in nephrotic children on anti-tubercular therapy is difficult due to the increased metabolism of prednisolone induced by rifampicin. We report a child with nephrotic syndrome treated successfully with an increased dose of steroids without discontinuing anti-tubercular therapy.

  2. Correlation between Oxidative Stress and Thyroid Function in Patients with Nephrotic Syndrome

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    Sangita U. Sawant

    2011-01-01

    Full Text Available Background. The present study is to look for a correlation between oxidative stress and thyroid function in patients with the nephrotic syndrome in the remission phase as well as in a persistent proteinuric state. Introduction. Nephrotic syndrome is a form of chronic kidney disease due to which blood loses protein through the urine. We wanted to know if there was an increased loss of thyroid hormones in urine affecting thyroid function. Methods. 60 patients with nephrotic syndrome and 20 healthy non-proteinuric individuals as control subjects were enrolled in the study. We measured their serum tri-iodothyronine, thyroxine and thyroid-stimulating hormone. Estimation of lipid peroxidation (LPx catalase, superoxide dismutase (SOD, and Glutathione peroxidase (GPx were carried out by standard methods. Results. TSH was elevated in the nephrotic patients compared to controls, while TT4 and TT3 were significantly lower in the patients than in controls. Lipid Peroxidation and GPx were significantly higher in the nephrotic syndrome patients than in the controls, while SOD and catalase were significantly lower than in patients than in the control subjects. Conclusion. Nephrotic patients can lose significant amounts of thyroid hormones along with protein in urine, which can affect thyroid status, but this is reversible on remission.

  3. Response to childhood immunizations in congenital nephrotic syndrome.

    Science.gov (United States)

    Nguyen, Stephanie; Winnicki, Erica; Butani, Lavjay

    2015-05-01

    Infections are a leading cause of morbidity in children following transplantation. It is therefore imperative to ensure that children are immunized before a transplant. Contrary to this recommendation, it has long been suggested that children with congenital nephrotic syndrome (CNS) not receive immunizations due to their perceived lack of response. We report a child with CNS who was immunized before transplantation per the routine pediatric immunization protocol and responded appropriately. The intent of this report is to encourage health care providers to immunize children with CNS, as the practice of withholding immunizations in these patients may have adverse health implications.

  4. Perspectives on edema in childhood nephrotic syndrome.

    Science.gov (United States)

    Teoh, Chia Wei; Robinson, Lisa A; Noone, Damien

    2015-10-01

    There have been two major theories surrounding the development of edema in nephrotic syndrome (NS), namely, the under- and overfill hypotheses. Edema is one of the cardinal features of NS and remains one of the principal reasons for admission of children to the hospital. Recently, the discovery that proteases in the glomerular filtrate of patients with NS are activating the epithelial sodium channel (ENaC), resulting in intrarenal salt retention and thereby contributing to edema, might suggest that targeting ENaC with amiloride might be a suitable strategy to manage the edema of NS. Other potential agents, particularly urearetics and aquaretics, might also prove useful in NS. Recent evidence also suggests that there may be other areas involved in salt storage, especially the skin, and it will be intriguing to study the implications of this in NS. Copyright © 2015 the American Physiological Society.

  5. Nephrotic syndrome associated with metastatic thymoma treated with chemotherapy.

    Science.gov (United States)

    Yoo, Shin Hye; Kim, Hyean-Ji; Kim, Jeong-Han; Lee, Gyeong-Won; Lee, Jeong Hee; Kim, Se Hyun; Kim, Ji-Won; Kim, Jin Won; Lee, Jeong-Ok; Kim, Yu Jung; Lee, Keun-Wook; Kim, Jee Hyun; Bang, Soo-Mee; Lee, Jong Seok

    2017-01-01

    Nephropathy with concurrent invasive thymoma is a type of paraneoplastic syndrome. We report a 32-year-old female with nephrotic syndrome that was first diagnosed along with invasive thymoma and treated by means of cisplatin-based chemotherapy for the thymoma. The patient initially presented with dyspnea and generalized edema. Chest radiography and computed tomography scans revealed right pleural effusion and a mass in the right middle lung field, which were confirmed by a percutaneous lung biopsy as metastatic invasive thymoma. Severe hypoalbuminemia, heavy proteinuria, hyponatremia, and hypercholesterolemia were features of the nephrotic syndrome. A kidney needle biopsy suggested focal segmental glomerulosclerosis. All of the symptoms of nephrotic syndrome were resolved simultaneously during the first 2 cycles of chemotherapy. The patient was on regular follow-up with no specific treatment for nephrotic syndrome and underwent successful resection of the left pleura and anterior thymoma. The patient has shown no evidence of recurrence for 2 years. We conclude that chemotherapy for invasive thymoma is an effective treatment for nephrotic syndrome accompanying the thymoma.

  6. Management of idiopathic nephrotic syndrome in childhood

    Directory of Open Access Journals (Sweden)

    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

  7. Plasmapheresis-induced Clinical Improvement in a Patient with Steroid-Resistant Nephrotic Syndrome Due to Podocin (NPHS2 Gene Station

    Directory of Open Access Journals (Sweden)

    Sylva Skálová

    2010-01-01

    Full Text Available Podocin mutations (NPHS2 gene are mostly responsible for steroid-resistant nephrotic syndrome (SRNS of childhood onset. Patients with NPHS2 gene mutations do not respond to corticoids and other immunosuppressive agents; partial remission can be rarely induced by cyclosporin A. We present a boy, where SRNS was diagnosed within first year of life. By the age of 15 years, proteinuria reached 9000 mg/24 h, cholesterolemia 15 mmol/L, albuminemia 19.6 g/L, in spite of combined therapy with cyclosporine A, methylprednisolone, enalapril and losartan. At that time a combined heterozygous form of two NPHS2 gene mutations (p.R138Q and p.V290M was diagnosed, methylprednisolone was discontinued and patient underwent ten plasmapheresis procedures. This resulted in clinical improvement (proteinuria 3000 mg/24 h, S-cholesterol 6 mmol/L, albumin 30g/L lasting for three years. In conclusion, plasmapheresis can result in clinical improvement and stabilization of SRNS caused by podocine mutation, before renal replacement therapy is initiated.

  8. Thromboembolic complications in childhood nephrotic syndrome: a clinical profile.

    Science.gov (United States)

    Suri, Deepti; Ahluwalia, Jasmina; Saxena, Akshay K; Sodhi, Kushaljit S; Singh, Paramjeet; Mittal, Bhagwant R; Das, Reena; Rawat, Amit; Singh, Surjit

    2014-10-01

    Thromboembolism is a rare life-threatening complication of childhood nephrotic syndrome. We present the clinical profile and outcome of 34 children with 35 events of thromboembolic complications with nephrotic syndrome. Cerebral venous thrombosis (CVT) was the commonest complication seen in 11 (31.4 %) children followed by pulmonary thromboembolism and deep venous thrombosis in 9 (25.7 %) and 6 (16.6 %) children, respectively. Arterial thrombosis resulting in central nervous system infarcts was observed in 7 (20 %) children and 2 children had thrombosis of the peripheral arteries. Episodes were equal in steroid-resistant nephrotic syndrome and steroid-dependent nephrotic syndrome groups. Most of the thromboembolic complications occurred with relapse but 11.4 % of children developed intracranial thrombosis during remission. The most sensitive symptom of CVT was persistent headache while unexplained respiratory distress and hypoxemia pointed towards pulmonary thromboembolism. Hypoalbuminemia was seen in 82.8 % of children, while concurrent infection was seen in 31.4 %. Coexistence of genetic prothrombotic condition was identified and merits evaluation. Early heparin therapy followed by oral anticoagulants resulted in complete recovery in 91.1 % of children. Death occurred in 3 (8.5 %) children and autopsy revealed pulmonary thromboembolism in 2 children. Venous and arterial thrombotic complications can occur in children with nephrotic syndrome. A high index of suspicion is required as the clinical features may be subtle. Neuroimaging and angiographic techniques help in confirming diagnosis. Early aggressive heparin therapy followed by oral anticoagulants is necessary for a favorable outcome.

  9. 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.

  10. Stem cell mobilization in idiopathic steroid-sensitive nephrotic syndrome.

    Science.gov (United States)

    Lapillonne, Hélène; Leclerc, Annelaure; Ulinski, Tim; Balu, Laurent; Garnier, Arnaud; Dereuddre-Bosquet, Nathalie; Watier, Hervé; Schlageter, Marie-Hélène; Deschênes, Georges

    2008-08-01

    Steroid-sensitive nephrotic syndrome (SSNS) is classically thought to be a T-cell disorder. The aim of this study was to examine whether or not thymus homeostasis was affected in SSNS. Mature and naive T cell recent thymic emigrants were quantified in the peripheral blood of nephrotic patients and controls. Because the generation of new T cells by the thymus ultimately depends on hematopoietic stem cells, CD34+ cells were also included in the study. Nineteen patients with SSNS during relapse, 13 with SSNS during proteinuria remission, and 18 controls were studied. Cell-surface markers (CD3, CD4, CD8, CD19, CD16, CD56, CD45RA, CD62L, CD34, and CD38) were analyzed by flow cytometric analysis. T-cell rearrangement excision circles (TRECs) were quantified in CD2+ cells by real-time polymerase chain reaction. Stroma cell-derived factor-1 (SDF-1) genotype and metalloproteinase-9 (MMP-9) plasma levels were also determined. Mature T cells (CD4+ and CD8+), circulating naive T cells (CD62L+ and CD3+ CD62L+), and recent thymic emigrants (CD45RA+) as well as TRECs, that measure thymus production, had a similar level in the three groups of patients. Conversely, CD34+ hematopoietic stem cells displayed a two-fold increase in SSNS patients during relapse either compared with controls or SSNS patients at remission. In addition, compared with controls, SSNS patients at remission displayed (1) a decrease in CD19+ cells (B cells) and (2) an increase in CD16CD56+ cells [natural killer (NK) cells]. In conclusion, thymus homeostasis is not significantly affected in nephrotic patients. Hematopoietic stem-cell mobilization at proteinuria relapse, as well as changes in B and NK cells during remission, suggest that SSNS might be due to a general disturbance of hematopoietic and immune cell trafficking.

  11. Primary peritonitis in nephrotic syndrome | Elidrissy | African Journal ...

    African Journals Online (AJOL)

    ... syndrome and were not related to steroid therapy. Treatment with appropriate antibiotics usually leads to good recovery with no need for surgical interference. Conclusion: Nephrotic syndrome associated peritonitis is usually spontaneous and caused mainly by pneumococci organism sensitive to penicillin or vancomycin.

  12. Childhood Nephrotic Syndrome in Aminu Kano Teaching Hospital ...

    African Journals Online (AJOL)

    Methods: A prospective study spanning two years (July 2002 – August 2004). Twenty two children with nephrotic syndrome were seen ate the Aminu Kano Teaching Hospital, Kano. The demographic, clinical and laboratory features and response to treatment were documented. Results: Nephritic syndrome made up of 1.2% ...

  13. 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.

  14. Steroid-resistant nephrotic syndrome secondary to primary focal segmental glomerulosclerosis and smoldering multiple myeloma.

    Science.gov (United States)

    Shah, Rupin; Shah, Nishi; Shah, Arun; Mehta, Ankit N

    2014-01-01

    We present a patient with steroid-resistant nephrotic syndrome due to focal segmental glomerulosclerosis along with smoldering multiple myeloma. While investigating the cause of proteinuria, a monoclonal gammopathy with a negative kidney biopsy for myeloma-related pathology was discovered.

  15. Steroid-resistant nephrotic syndrome secondary to primary focal segmental glomerulosclerosis and smoldering multiple myeloma

    OpenAIRE

    Shah, Rupin; Shah, Nishi; Shah, Arun; Mehta, Ankit N.

    2014-01-01

    We present a patient with steroid-resistant nephrotic syndrome due to focal segmental glomerulosclerosis along with smoldering multiple myeloma. While investigating the cause of proteinuria, a monoclonal gammopathy with a negative kidney biopsy for myeloma-related pathology was discovered.

  16. Role of the immune system in the pathogenesis of idiopathic nephrotic syndrome

    NARCIS (Netherlands)

    van den Berg, José G.; Weening, Jan J.

    2004-01-01

    Idiopathic NS (nephrotic syndrome) is characterized by massive proteinuria, due to a leak in the glomerular barrier to proteins. Genetic defects that affect the function and the composition of the glomerular capillary wall, in particular of the visceral epithelial cells, have recently been

  17. Nephrotic Syndrome in a Child Suffering from Tetralogy of Fallot: A Rare Association

    Directory of Open Access Journals (Sweden)

    Pépé Mfutu Ekulu

    2015-01-01

    Full Text Available Nephrotic syndrome is an uncommon complication of tetralogy of Fallot and has been rarely reported in pediatric population. We describe a 4-year-old female Congolese child who was referred for investigation for persistent dyspnea, edema, and cyanosis and nephrotic range proteinuria. Our patient presented with a tetralogy of Fallot and nephrotic syndrome. Conclusion. This case reminds us that children with tetralogy of Fallot may develop nephrotic proteinuria.

  18. 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.

  19. 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.

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

    Science.gov (United States)

    Derakhshan, Nima; Derakhshan, Dorna; Torabinejad, Simin; Derakhshan, Ali

    2011-01-01

    BK virus (BKV) is increasingly found as an important cause of allograft nephropathy. 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 transplanted from a 16-year-old cadaver, and after 18 months of uneventful transplantation on triple immunosuppressive therapy (mycophenolate mofetil (MMF), cyclosporin and prednisolone), presented with nephrotic feature (edema, heavy proteinuria, hypoalbuminemia and hyperlipidemia). Kidney biopsy was in favor of BKV infection and eventually ended in graft failure.

  1. Lymphoma and the nephrotic syndrome | Sagel | South African ...

    African Journals Online (AJOL)

    South African Medical Journal. Journal Home · ABOUT THIS JOURNAL · Advanced Search · Current Issue · Archives · Journal Home > Vol 45, No 3 (1971) >. Log in or Register to get access to full text downloads. Username, Password, Remember me, or Register. Lymphoma and the nephrotic syndrome. Sagel Sagel, H ...

  2. The management of nephrotic syndrome in children | Hodson ...

    African Journals Online (AJOL)

    Data from RCTs supports the use of alkylating agents (cyclophosphamide, chlorambucil), cyclosporin and levamisole in these children to achieve prolonged periods of remission after induction of remission with prednisone. Steroid resistant nephrotic syndrome is more common in Africa. Few therapies are effective. In such ...

  3. [Differential diagnosis of nephritic and nephrotic syndroms in children].

    Science.gov (United States)

    Masalskiene, Jūrate

    2003-01-01

    The aim of this study was to compare clinical and laboratory features of children with acute poststreptococcal glomerulonephritis and steroid sensitive nephrotic syndrome. We have examined 30 children with acute poststreptococcal glomerulonephritis and 17 children with steroid sensitive nephrotic syndrome, who arrived for hospitalization at Kaunas University of Medicine Hospital in 1997-2000. All patients not later than in ten days from the onset of acute glomerulonephritis and 14 days later were given a standartized clinical and laboratorical examination following a record. We determined that hematuria is universal finding of acute poststreptococcal glomerulonephritis and proteinuria was found in all patients with steroid sensitive nephrotic syndrome. Microscopic hematuria and proteinuria were persisted in children who had acute poststreptococcal glomerulonephritis. Anemia was found only in patients who were ill with acute poststreptococcal glomerulonephritis. After 14 days of treatment it was determined in 38.5% of children. Hypoproteinemia was established in 69.2% of children and hypercholesterolemia in 92.3% of children with steroid sensitive nephrotic syndrome after two weeks of treatment. Decreased glomerular filtration rate was characteristic feature of acute poststreptococcal glomerulonephritis.

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

    African Journals Online (AJOL)

    2013-11-14

    Nov 14, 2013 ... generally has a favorable response to glucocorticoid therapy in over 80% of patients.[6‑9] Children having steroid‑resistant nephrotic syndrome (SRNS) with focal and segmental glomerular sclerosis (FSGS) or MCNS run a high risk of resistance to immunosuppressive therapy.[10] The incidence of FSGS ...

  5. CASE REPORT Thirty years old lady with nephrotic syndrome: a ...

    African Journals Online (AJOL)

    CASE REPORT. Thirty years old lady with nephrotic syndrome: a case of biopsy proven lupus nephritis in Tanzania. FRANCIS FREDRICK1,2*, PASCHAL J. RUGGAJO2,3,GYAVIIRA MAKANGA3, CHARLES K. SHIJA3, MIKAEL. AMDEMARIAM3, BELSON RUGWIZAGONGA4 and JAMES N. KITINYA4. 1Department of ...

  6. Renal Doppler Indices in Children with Nephrotic Syndrome ...

    African Journals Online (AJOL)

    Summary: The resistive and pulsatility indices are known tools for assessing renal function in kidney diseases, especially in proteinuric conditions like Paediatric Nephrotic syndrome (NS) which is a glomerular disease. However, there is a limited knowledge in the use of Doppler Resistive and pulsatility indices in the ...

  7. Glucocorticoid Treatment in Childhood Nephrotic Syndrome : weighting the cornerstone

    NARCIS (Netherlands)

    N. Teeninga (Nynke)

    2013-01-01

    textabstractUnderstanding which factors influence relapse patterns in childhood nephrotic syndrome is clinically very relevant and could aid in developing new treatment strategies. Clinicians are continuously challenged to reduce relapse rates and at the same time to avoid glucocorticoid toxicity.

  8. Recent Treatment Advances and New Trials in Adult Nephrotic Syndrome

    Directory of Open Access Journals (Sweden)

    Eva Königshausen

    2017-01-01

    Full Text Available The etiology of nephrotic syndrome is complex and ranges from primary glomerulonephritis to secondary forms. Patients with nephrotic syndrome often need immunosuppressive treatment with its side effects and may progress to end stage renal disease. This review focuses on recent advances in the treatment of primary causes of nephrotic syndrome (idiopathic membranous nephropathy (iMN, minimal change disease (MCD, and focal segmental glomerulosclerosis (FSGS since the publication of the KDIGO guidelines in 2012. Current treatment recommendations are mostly based on randomized controlled trials (RCTs in children, small RCTs, or case series in adults. Recently, only a few new RCTs have been published, such as the Gemritux trial evaluating rituximab treatment versus supportive antiproteinuric and antihypertensive therapy in iMN. Many RCTs are ongoing for iMN, MCD, and FSGS that will provide further information on the effectiveness of different treatment options for the causative disease. In addition to reviewing recent clinical studies, we provide insight into potential new targets for the treatment of nephrotic syndrome from recent basic science publications.

  9. Rituximab for troublesome cases of childhood nephrotic syndrome.

    Science.gov (United States)

    Safdar, Osama Y; Aboualhameael, Adila; Kari, Jameela A

    2014-11-08

    Nephrotic syndrome (NS) is the most common glomerular disease of childhood. Steroid-dependent and steroid-resistant nephrotic syndrome present challenges in their pharmaceutical management; patients may need several immunosuppressive medication for optimum control, each of which medication has its own safety profile. Rituximab (RTX) is a monoclonal antibody that targets B cells and has been used successfully for management of lymphoma and rheumatoid arthritis. Recent clinical studies showed that rituximab may be an efficacious and safe alternative for the treatment of complicated nephrotic syndrome. In this review article, we aim to review the efficacy and safety of RTX therapy in nephrotic syndrome. We reviewed the literature pertaining to this topic by searching for relevant studies on PubMed and Medline using specific keywords. The initial search yielded 452 articles. These articles were then examined to ensure their relevance to the topic of research. We focused on multicenter randomized controlled trials with relatively large numbers of patients. A total of 29 articles were finally identified and will be summarized in this review. The majority of clinical studies of RTX in complicated pediatric NS showed that rituximab is effective in approximately 80% of patients with steroid-dependent NS, as it decreases the number of relapses and steroid dosage. However, RTX is less effective at achieving remission in steroid-resistant NS. RTX use was generally safe, and most side effects were transient and infusion-related. More randomized, double-blinded clinical studies are needed to assess the role of RTX in children with nephrotic syndrome.

  10. 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.

  11. A case of nephrotic syndrome associated with hydatiform mole.

    Science.gov (United States)

    Mohammadjafari, Razieh; Abedi, Parvin; Belady, Syfolah; Hamidehkho, Tarlan; Razi, Taghi

    2010-12-31

    The present case study is on a 16-year-old woman who was suffering from nephrotic syndrome after recovery from complete type of hydatiform mole. She was admitted in hospital because of proteinurea and hematuria. Then she was showing a generalized edema compatible with neprhotic syndrome. In her past medical history she had a suction curettage for hydatiform mole. After she received 4 courses chemotherapy, she completely recovered and βhCG has fallen from 12127 IU/L to under 10 IU/mL. Then she showed generalized edema, proteinurea and hematuria compatible with nephritic syndrome. After six courses chemotherapy the symptoms of nephrotic syndrome and invasive mole diminished, she released from hospital and scheduled for follow-up.

  12. Cyclosporine utilization in Idiopathic Nephrotic Syndrome in children

    International Nuclear Information System (INIS)

    Saeed, B.; Sheriff, S.; Ossman, Mohd Imad

    2006-01-01

    The treatment of steroid-resistant focal segmental glomerulosclerosis (FSGS) imposes one the most perplexing and frustrating problems on nephrologists. Cyclosporine A (CsA) is widely considered as the treatment of choice for steroid-resistant or dependent nephrotic children. We reviewed the clinical outcome in children with idiopathic nephrotic syndrome (INS) under CsA treatment. A total of 22 children presented with either steroid resistant nephrotic syndrome (SRNS) (14 children), or steroid-dependent nephrotic syndrome (SDNS) (8 children) during the period from August 2002 to February 2005; the mean age for both groups was 7.6 years (range: 23 months-15 years). Renal histology showed FSGS in 14 (63%) patients, minimal change disease (MCD) in 4(18%), diffuse mesangial glomerulonphritis (MesGN) in three (13.6%), and membranous glomerulonephritis (MGN) in two (6.8%). Treatment with CsA in combination with alternate-day prednisolone induced remission in 15(68%) patients; 9(60%) patients had complete remission and six (40%) partial remission. Seven (50%) patients in SRNS group responded to CsA treatment; two (14.2%) patients had complete remission and 5 (35.7%) had partial remission. Seven ( 87.5%) children in SDNS group had complete remission and one (13.5%) had partial remission. We conclude that this study demonstrates the efficacy of CsA in inducing remission in the steroid dependent is higher than in the steroid resistant nephrotic children. We believe that CsA is probably a good alternative therapy in this population. (author)

  13. Corticosteroid-responsive nephrotic syndrome in children with myelodysplastic syndromes

    Directory of Open Access Journals (Sweden)

    Bogdanović Radovan

    2002-01-01

    Full Text Available Several reports have documented various forms of glomerular diseases in adults with myelodysplastic syndromes (MDS, but similar reports in children are lacking. We describe two children with MDS-associated with steroid-responsive nephrotic syndrome (NS. Patient 1, who had MDS with myelofibrosis, presented also hepatosplenomegaly, pancytopenia, chronic hepatitis, moderate proteinuria, hypocomplementemia and elevated ANA titer. During initial prednisone treatment proteinuria markedly diminished and partial but transient haematological improvement occurred. Relapse subsequently occurred that was manifested by overt NS and pancytopenia. High doses of prednisolone led to remission of the renal disease but haematological remission did not occur. Persisting pancytopenia and repeated infections terminated in sepsis, two years after the onset of MDS. Patient 2, who had refractory anemia with clonal monosomy 19, manifested bowel disease, hepatospleno- megaly, anaemia and non-organic specific autoantibodies. Prednisone led to both clinical and haematological remission. Haematologic disease relapsed 12 months later, when nephrotic-range proteinuria, haematuria and mild azotaemia were also found. Corticosteroid treatment led to long-lasting renal and haematologic remission, maintained by a small dosage of prednisone. In both patients renal biopsy findings were consistent with those seen in idiopathic NS. A Medline search disclosed 16 cases of glomerulopathy in the course of MDS in adult patients. Clinical features included NS, usually accompanied by renal insufficiency with either acute, chronic, or rapidly progressive glomerulonephritis. On biopsy, membranous nephropathy, crescentic or mesangial proliferative glomerulonephritis and AL amyloidosis, were found. We conclude: (1 that glomerular disease may be present and should be searched for in patients with MDS; (2 that MDS can be added to the list of rare conditions associated with corticosteroid

  14. [Clinical characteristics of children with an initial onset of IgA nephropathy with nephrotic syndrome].

    Science.gov (United States)

    Zhang, Liang; Li, Zhi-Hui; Yin, Yan; Duan, Cui-Rong; Xun, Mai; Wu, Tian-Hui; Zhang, Yi; Ding, Yun-Feng

    2015-08-01

    To study the clinical characteristics of children with an initial onset of IgA nephropathy with nephrotic syndrome and compare them with children with primary nephrotic syndrome, in order to provide a theoretical basis for the differential diagnosis of the two diseases. Fifty children diagnosed with an initial onset of IgA nephropathy with nephrotic syndrome were included in this study. Seventy-two children diagnosed with an initial onset of primary nephrotic syndrome served as the control group. The clinical and laboratory examination characteristics were compared between the two groups. The IgA nephropathy group had significantly higher incidence rates of gross haematuria, microscopic haematuria, hypertension, acute kidney injury, low serum high-density lipoprotein cholesterol, anemia, low serum complement C4, steroid resistance, and nephritis-type nephrotic syndrome and a significantly lower incidence of elevated serum IgE compared with the control group (Psyndrome and primary nephrotic syndrome. Children with IgA nephropathy presenting nephrotic syndrome manifest mainly as nephritis type and steroid-resistant type in the clinical classification. Cinical manifestations accompanied by serum levels of high-density lipoprotein cholesterol and IgE are helpful for differential diagnosis of IgA nephropathy presenting nephrotic syndrome and primary nephrotic syndrome.

  15. Clinical presentation & management of glomerular diseases: hematuria, nephritic & nephrotic syndrome.

    Science.gov (United States)

    Khanna, Ramesh

    2011-01-01

    Because the differential diagnosis for glomerulonephritis (GN) is broad, using a classification schema is helpful to narrow the causes of GN in a systematic manner. The etiology of glomerulonephritis can be classified by their clinical presentation (nephrotic, nephritic, rapidly progressive GN, chronic GN) or by histopathology. GN may be restricted to the kidney (primary glomerulonephritis) or be a secondary to a systemic disease (secondary glomerulonephritis). The nephrotic syndrome is defined by the presence of heavy proteinuria (protein excretion greater than 3.0 g/24 hours), hypoalbuminemia (less than 3.0 g/dL), and peripheral edema. Hyperlipidemia and thrombotic disease may be present. The nephritic syndrome is associated with hematuria and proteinuria and abnormal kidney function and carries poorer prognosis and is typically associated with hypertension. The predominant cause of the nephrotic syndrome in children is minimal change disease. The most common causes of nephritic syndrome are post infectious GN, IgA nephropathy and lupus nephritis. Chronic GN is slowly progressive and is associated with hypertension and gradual loss of kidney function. Treatment includes non-specific measure aimed at controlling hypertension, edema, proteinuria and disease modifying immunosuppression.

  16. 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.

  17. Chryseobacterium gleum pneumonia in an infant with nephrotic syndrome

    OpenAIRE

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

    2016-01-01

    Introduction: Chryseobacterium gleum is commonly distributed in the environment. It can cause a wide variety of infections in immunocompromised patients in hospital setting. Case presentation: 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 ...

  18. A Study of Hypoalbuminemia and Pleural Effusionin Pediatric Nephrotic Syndrome

    Directory of Open Access Journals (Sweden)

    Tovan Perinandika

    2017-06-01

    Full Text Available Background: Nephrotic syndrome (NS is a kidney disease that is most often found in children. Hypoalbuminemia in NS can cause a decrease in oncotic pressure causing extravasation of fluid into the interstitial space. In conditions of severe hypoalbuminemia, fluid extravasation may cause occurrence of pleural effusion. The objectives of this study was to analyze the correlation between hypoalbuminemia and pleural effusion in children with NS. Methods: An analytical study was conducted on 69 medical records of pediatric nephrotic syndrome from 1 January 2008–31 December 2013 in dr. Hasan Sadikin General Hospital. Inclusion criteria were pediatric patients between 1-14 years old with NS. Exclusion criteria were patients who already had albumin transfusion, malnutrition, patients with chronic disease, and incomplete medical record information. Contingency coefficient test was carried out to discover the correlation between variables. Results: Out of 89 samples, 69 samples were included. Characteristics of the included patients are male (n=48, female (n=21, age 1–5 (n=24, 6–10 (n=22, 11–14 (n=23, mild hypoalbuminemia (n=3, moderate hypoalbuminemia (n=27, severe hypoalbuminemia (n=39, patients with pleural effusion (n=23, and non-pleural effusion (n=46. There was a significant correlation between  hypoalbuminemia and pleural effusion with p=0.000 (p<0.05 and moderate correlation (r=0.437. Conclusions: Hypoalbuminemia has correlation with pleural effusion in pediatric nephrotic syndrome. Keywords: Hypoalbuminemia, pediatric nephrotic syndrome, pleural effusion DOI: 10.15850/amj.v4n2.1075

  19. Renal Doppler Indices in Children with Nephrotic Syndrome ...

    African Journals Online (AJOL)

    olayemitoyin

    *p<0.05. Table 4. Comparison of the Mean Resistive Index of the Different Degree of Proteinuria among the Nephrotic Syndrome children. Right Kidney. Left Kidney. Degree of Proteinuria. RI f. P-value. RI f. P-value. Upper Pole. 1+. 0.564. 0.301. 0.742. 0.631. 4.819. 0.014. 2+. 0.578. 0.550. 3+. 0.586. 0.599. Middle Pole. 1+.

  20. CLINICO PATHOLOGICAL STUDY OF NEPHROTIC SYNDROME IN ADULTS

    Directory of Open Access Journals (Sweden)

    Deepa

    2015-11-01

    Full Text Available CONTEXT: Etiology of nephrotic syndrome (NS in adults varies depending on the geographical location and is poorly studied in the Indian subcontinent. AIMS: To study the clinical features, biochemical profile and histopathological pattern of various types of glomerulonephritis in adult patients with nephrotic syndrome. METHODS AND MATERIAL: Patients (≥15 years old with nephrotic syndrome presenting to our center and undergoing a kidney biopsy from May 2009 to August 2011 were included for this study. All biopsies were subjected to light microscopy.The histopathological spectrum was analyzed according to the various clinical parameters. STATISTICAL ANALYSIS USED: Analysis was performed using SPSS software version 19.Measures obtained included percentages,medians,correlation coefficients and chi square tests. RESULTS: A total of 50 kidney biopsies were included in analysis. Twenty six(52% patients were male and twenty four(48% patients were female. The average age at presentation was 15-24 years. Among the patients, 22(44% were diagnosed with primary glomerular diseases (PGD and 4(8% with secondary glomerular diseases (SGD. The most common histological lesions was membranous nephropathy(24.4% followed by minimal change disease (MCD (17% and membranous nephropathy (MN (17%. The most common form of SGD was lupus nephritis (LN (10%. Membranous nephropathy and focal segmental glomerulosclerosis were the commonest lesions in males.Among females,membranous nephropathy was the commonest. 31(62% patients were in the age group of 15-34 yrs, 17(34% were in the age group 34- 54yrs and only 2(4% were aged above 55yrs. Among the patients, 6(12% had serum creatinine ≥1.5 mg/dL and 12(24% had either macroscopic or microscopic hematuria. CONCLUSIONS: Membranous nephropathy is still the commonest type of nephrotic syndrome in adults followed closely by focal segmental glomerulosclerosis and minimal change disease as per this study.

  1. Histopathological diagnosis and outcome of paediatric nephrotic syndrome

    International Nuclear Information System (INIS)

    Ejaz, I; Khan, H.I.; Javaid, B.K.; Bhatti, M.T.; Rasool, G.

    2004-01-01

    Objective: To determine the histological picture and outcome of treatment in cases of childhood nephrotic syndrome who needed renal biopsy. Subjects and Methods: Children suffering from nephrotic syndrome who had atypical features at presentation were initially or late non-responders; frequent relapsers on > 1 mg kg/day and were steroid dependent or frequently relapsed on < 1 mg kg/day but developed steroid toxicity were included. Renal biopsy was performed in these patients. Treatment was administered according to the histopathology reports. Prednisolone 60 mg /m/sup 2//day followed after response by 40 mg /m/sup /2 on alternate days (AD) which was later tapered off. In minimal change nephrotic syndrome (MCNS) with frequent relapses cyclophosphamide, cyclosporine and levimisole were used. For steroid resistant focal segmental glomerulosclerosis (FSGS) intravenous pulses of methylprednisolone and cyclosporine were also given. These patients were followed to see the response of the therapy. Results: The commonest diagnosis was focal segmental glomerulosclerosis (FSGS) (42%) followed by minimal change disease (MCNS) (22%), membranoproliferative or mesangiocapillary glomerulonephritis (MPGN) (14%) and Mesangioproliferative glomerulonephritis (Mes PGN) (12%). There were 6% cases of membranous nephropathy and 4% of diffuse proliferative glomerulonephritis. On presentation, 40% had hematuria, 20% were found to be hypertensive, 12% patients had renal insufficiency and in 40% C3 level was low. Majority of the patients with MPGN and FSGS had atypical features whereas none of the patients with membranous nephropathy had any of these features. Thirty percent cases each of FSGS and MCNS were responders. Among non-responders there were 4 cases of FSGS and one of MPGN. Conclusion: FSGS was the commonest histology in cases of childhood nephrotic syndrome that needed renal biopsy. Highest frequency of atypical features was seen in MPGN and FSGS. (author)

  2. Is rituximab effective in childhood nephrotic syndrome? Yes and no.

    Science.gov (United States)

    Kemper, Markus J; Lehnhardt, Anja; Zawischa, Anna; Oh, Jun

    2014-08-01

    The idiopathic nephrotic syndrome (i.e. MCNS and FSGS) in children has been regarded as a disorder of T-cell function. Recent studies, however, also describe abnormalities of B-cell function. This supports the use of B-cell modulating treatment for idiopathic nephrotic syndrome (INS), especially rituximab, which has been used in other glomerular disorders as well. Many studies indicate that rituximab is effective in steroid-sensitive and -dependent nephrotic syndrome, by either inducing long-term remission or reducing relapses. In most series, children with primary (and recurrent) focal segmental glomerulosclerosis (FSGS) do not respond as well. The exact mechanisms of action of rituximab (as well as those of the other treatment options) in INS are as yet unclear. In addition to hosting mechanisms a direct stabilizing effect on the podocyte may also be of relevance, especially in FSGS. Although results are encouraging especially in steroid-sensitive patients, further studies on the clinical use of rituximab and the short- and long-term immunological effects and side-effects are necessary.

  3. 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.

  4. GENETICS OF CHILDHOOD STEROID SENSITIVE NEPHROTIC SYNDROME (SSNS)

    Science.gov (United States)

    Karp, Alana M.; Gbadegesin, Rasheed A.

    2016-01-01

    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 twenty 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 (FSGS 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. This article reviews what is currently known about the genetics of SSNS and also discusses how recent careful phenotypic and genomic studies reinforce the role of adaptive immunity in the molecular mechanisms of SSNS. PMID:27470160

  5. Parental Health Literacy and Outcomes of Childhood Nephrotic Syndrome.

    Science.gov (United States)

    Borges, Karlota; Sibbald, Cathryn; Hussain-Shamsy, Neesha; Vasilevska-Ristovska, Jovanka; Banh, Tonny; Patel, Viral; Brooke, Josefina; Piekut, Monica; Reddon, Michele; Aitken-Menezes, Kimberly; McNaughton, Ashley; Pearl, Rachel J; Langlois, Valerie; Radhakrishnan, Seetha; Licht, Christoph P B; Piscione, Tino D; Levin, Leo; Noone, Damien; Hebert, Diane; Parekh, Rulan S

    2017-03-01

    Determine the association of parental health literacy with treatment response among children with nephrotic syndrome. This was a cohort study of children aged 1-18 with nephrotic syndrome and their parent. Health literacy was measured using the validated Short Test of Functional Health Literacy in Adults assessing reading comprehension and numeracy. Outcomes included initial relapse-free period, frequently relapsing disease, relapse rate, second-line medication use, and complete remission after therapy. Of 190 parents, 80% had adequate health literacy (score >67 of 100), and higher scores were not correlated with higher education. Almost all achieved perfect numeracy scores (>86%); numeracy was not associated with outcomes. After adjusting for immigration, education, and income, higher reading comprehension scores (tertile 3) compared with lower scores (tertile 1) were significantly associated with lower risk of first relapse (hazard ratio 0.67, 95% confidence interval [CI] 0.48-0.94, P trend = .02), lower odds of frequently relapsing disease (odds ratio [OR] 0.38, 95% CI 0.21-0.70, P trend = .002), lower relapse rate (rate ratio 0.77, 95% CI 0.73-0.80, P trend nephrotic syndrome and fewer achieving complete remission. This underscores the importance of assessing and targeting health literacy for chronic management of childhood-onset diseases. Copyright © 2017 by the American Academy of Pediatrics.

  6. Relapse of nephrotic syndrome triggered by Kawasaki disease.

    Science.gov (United States)

    Maeda, Ryo; Kawasaki, Yukihiko; Suzuki, Shigeo; Ohara, Shinichiro; Kazuhide, Suyama; Hosoya, Mitsuaki

    2018-05-01

    Minor infections, allergies, insect bites, and bee stings are commonly reported causes of nephrotic syndrome (NS). Herein, we report, to the best of our knowledge, the first case of NS relapse due to Kawasaki disease (KD). An 8-year-old boy presented with high fever of 4-day duration. He had developed steroid-dependent NS at the age of 4 years and remained in remission after steroid and mizonbin therapy. Renal biopsy, performed at the age of four, showed minimal change (MC) disease. Upon examination, the patient fulfilled 5 of 6 criteria for KD under the Japanese diagnostic guidelines, with positive proteinuria. He was diagnosed with NS relapse caused by KD. Proteinuria resolved after treatment with intravenous immunoglobulin and cyclosporine A. We present the case of an 8-year-old boy, whose NS relapsed due to KD. To the best of our knowledge, this is the first case report. It is necessary to recognize that KD can trigger relapse of MCNS.

  7. 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.

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

    Science.gov (United States)

    Cebeci, Egemen; Demir, Secil; Gursu, Meltem; Sumnu, Abdullah; Yamak, Mehmet; Doner, Barıs; Karadag, Serhat; Uzun, Sami; Behlul, Ahmet; Ozkan, Oktay; Ozturk, Savas

    2015-01-01

    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. PMID:26000182

  9. Peritubular capillaries and renal function in pediatric idiopathic nephrotic syndrome

    Directory of Open Access Journals (Sweden)

    Kamaljeet Singh

    2013-01-01

    Full Text Available Nephrotic syndrome (NS is a common renal disorder with significant tubulo-interstitial damage due to the combined effects of proteinuria and obstruction of efferent blood flow. Peritubular capillary (PTC loss has also been correlated with interstitial fibrosis. This study included 30 pediatric cases of idiopathic NS. Clinical details, including biochemical parameters, were recorded and renal biopsy slides were reviewed for histological features. PTCs were highlighted using anti-CD34 antibody and quantified with the help of image analysis software. Postmortem kidney biopsies from seven children were taken as controls for quantification of PTCs and interstitial fibrosis. Wherever possible, as ultrastructural examination of the renal biopsy was performed. Appropriate statistical methods were applied. Patients with minimal change disease (MCD had lower serum creatinine as compared with those with focal and segmental glomerulosclerosis (FSGS. Similarly, tubular atrophy and interstitial fibrosis were significantly lower in MCD than in FSGS. PTC density was lower in all groups of NS as compared with the controls. Biopsies with FSGS had a lower PTC density compared with both MCD and mesangioproliferative glomerulonephritis. PTC density showed a negative correlation with serum creatinine and degree of proteinuria. PTC loss appears to play an important role in the development of renal biopsy changes in pediatric NS. This aspect of the renal vasculature requires further study in idiopathic NS.

  10. Corticosteroids therapy and peptic ulcer disease in nephrotic syndrome patients

    Science.gov (United States)

    Hsiang, Kuo-Wei; Ng, Yee-Yung; Lu, Ching-Liang; Chen, Tseng-Shing; Lin, Hsiao-Yi; Luo, Jiing-Chyuan; Wu, Jia-Min; Lin, Han-Chieh; Chang, Full-Young; Lee, Shou-Dong

    2010-01-01

    AIMS Whether corticosteroids induce peptic ulcer disease (PUD) remains uncertain. The study evaluated and compared the occurrence of PUD between nephrotic patients receiving oral prednisolone therapy and nephritic patients without steroid therapy. METHODS The prospective case control study compared 60 nephrotic syndrome patients who received 60 mg daily prednisolone therapy for 3 months with 30 age-and sex-matched nephritic patients without steroid therapy. Each patient underwent endoscopic examination and tissue and blood sampling before and after the study. Examined parameters included Helicobacter pylori (H. pylori) infection, and gastric and serum prostaglandin (PG) E2 and thromboxane (TX) B2 concentrations. The primary endpoint was the occurrence of endoscopic peptic ulcers between the two groups, while the secondary end point was the occurrence of ulcer complications. RESULTS The two groups were comparable in sex, age, smoking habits, alcohol drinking, past history of PUD, H. pylori infection rate and serum creatinine. There were no differences in the occurrence of endoscopic peptic ulcers (1.6% vs. 3.3%) and ulcer complications (0% vs. 0%), pre-therapy gastric PGE2, and pre- and post-therapy gastric TXB2, serum PGE2 and serum TXB2 between the two groups. However, there was significantly lower post-therapy gastric PGE2 concentrations in the prednisolone group. CONCLUSIONS Three-month therapy with 60 mg daily prednisolone caused few endoscopic ulcers (1.6%) and no ulcer complications in nephrotic patients. Corticosteroids therapy did not increase PUD in nephrotic syndrome patients [odds ratio 0.492 with 95% confidence interval (CI) 0.03, 8.142, P= 0.620]. Further larger studies are needed to clarify the role of corticosteroids in PUD.

  11. 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.

  12. Transjugular renal biopsy in a case of nephrotic syndrome with extrahepatic portal venous obstruction

    Directory of Open Access Journals (Sweden)

    N Siddiqi

    2013-01-01

    Full Text Available Renal biopsy in patients with nephrotic syndrome helps to establish the pathological diagnosis and subsequent treatment. In certain circumstances, biopsies are difficult to obtain because of the risk of bleeding. We report a case where renal biopsy was obtained through the transjugular route in a patient who had nephrotic syndrome with extrahepatic portal venous obstruction.

  13. Risk of Nephrotic Syndrome following Enteroviral Infection in Children: A Nationwide Retrospective Cohort Study.

    Science.gov (United States)

    Lin, Jiun-Nong; Lin, Cheng-Li; Yang, Chi-Hui; Lin, Ming-Chia; Lai, Chung-Hsu; Lin, Hsi-Hsun; Kao, Chia-Hung

    2016-01-01

    Nephrotic syndrome is a common chronic illness encountered during childhood. Infections have been identified as a cause of nephrotic syndrome. The aim of this study was to evaluate the association between enteroviral infection and nephrotic syndrome. A nationwide retrospective cohort study was conducted by analyzing data from the National Health Insurance Research Database in Taiwan. Children aged children were randomly selected as the comparison cohort. The primary endpoint was the occurrence of nephrotic syndrome. This study included 280,087 enterovirus-infected children and 280,085 non-enterovirus-infected children. The mean age of the enterovirus-infected children was 2.38 years, and 53.7% of these children were boys. The overall incidence densities of nephrotic syndrome for enterovirus- and non-enterovirus-infected children were 2.65 and 2.21 per 10,000 person-years, respectively. The enterovirus-infected cohort had a higher cumulative incidence of nephrotic syndrome than did the non-enterovirus-infected cohort (log-rank test, p = 0.01). Multivariable analyses revealed that children with enteroviral infection were significantly associated with an increased risk of nephrotic syndrome compared with those without enteroviral infection (adjusted hazard ratio, 1.20; 95% confidence interval, 1.04-1.39; p = 0.01), particularly in children infected with coxsackievirus. Subgroup analyses revealed that enterovirus-infected girls, children of blue-collar workers, and children without allergies had a higher risk of nephrotic syndrome than did children in the non-enterovirus-infected cohort. This study revealed a significant association between enteroviral infection and nephrotic syndrome. Additional studies elucidating the role and pathogenesis of enterovirus in nephrotic syndrome are warranted.

  14. Risk of Nephrotic Syndrome following Enteroviral Infection in Children: A Nationwide Retrospective Cohort Study.

    Directory of Open Access Journals (Sweden)

    Jiun-Nong Lin

    Full Text Available Nephrotic syndrome is a common chronic illness encountered during childhood. Infections have been identified as a cause of nephrotic syndrome. The aim of this study was to evaluate the association between enteroviral infection and nephrotic syndrome.A nationwide retrospective cohort study was conducted by analyzing data from the National Health Insurance Research Database in Taiwan. Children aged <18 years with enteroviral infection were enrolled. Non-enterovirus-infected children were randomly selected as the comparison cohort. The primary endpoint was the occurrence of nephrotic syndrome.This study included 280,087 enterovirus-infected children and 280,085 non-enterovirus-infected children. The mean age of the enterovirus-infected children was 2.38 years, and 53.7% of these children were boys. The overall incidence densities of nephrotic syndrome for enterovirus- and non-enterovirus-infected children were 2.65 and 2.21 per 10,000 person-years, respectively. The enterovirus-infected cohort had a higher cumulative incidence of nephrotic syndrome than did the non-enterovirus-infected cohort (log-rank test, p = 0.01. Multivariable analyses revealed that children with enteroviral infection were significantly associated with an increased risk of nephrotic syndrome compared with those without enteroviral infection (adjusted hazard ratio, 1.20; 95% confidence interval, 1.04-1.39; p = 0.01, particularly in children infected with coxsackievirus. Subgroup analyses revealed that enterovirus-infected girls, children of blue-collar workers, and children without allergies had a higher risk of nephrotic syndrome than did children in the non-enterovirus-infected cohort.This study revealed a significant association between enteroviral infection and nephrotic syndrome. Additional studies elucidating the role and pathogenesis of enterovirus in nephrotic syndrome are warranted.

  15. Evaluation of bone and mineral metabolism in primary corticosteroid-sensitive pediatric nephrotic syndrome

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    Miguel Liern

    2017-04-01

    Full Text Available Introduction: Steroid treatment for corticosteroid-sensitive nephrotic syndrome (CSNS could cause bone and mineral metabolism alterations, preventable with calcium and Vitamin D. Objectives: We carried out a preliminary retrospective study along 36 months with the following objectives. 1 To evaluate the relationship between Cumulative Corticosteroid Doses (CCD and 25-0 Vitamin D serum concentration and with Bone Mineral Content (BMC; 2 To evaluate the relationship between CCD and Bone Mineral Densitomety (BMD. Methods: We included patients between 2 and 12 years of age with corticosteroid sensitive primary nephrotic syndrome (CSNS (first episode, relapsing nephrotic syndrome, corticosteroid dependent nephrotic syndrome normotensive, eutrophic and FG>:90ml/min/1.73 m2, who were divided into three groups: GROUP A: =3 or 4 relapses/year, GROUP C: CSNS, we measured: a Quarterly: calcemia, phosphatemia, alkaline phosphatase; b half-yearly: 25-OH cholecalcipherol levels, CCD; c annually BMD in children >6 years (score Z and BMC, bone age, PTHi. Results: We evaluated 29 patients, average age: 4.4 years. The BMD was performed on 11 patients and there were no significant differences among the groups (p=0.08. No significant differences were seen between chronologic age and average bone age (p=0.3. Change in 25-OH cholecalcipherol levels due to the increase of ergocalcipherol dose was significant (T:32.4 Q:<0.001. There were significant correlation in the three groups, between Vitamin D dose and Vitamin D serum levels (Pearson correlation R=0.9, between CCD and 25 OH cholecalcipherol dose: (Pearson correlation R=0.62 and between CCD and BMC (Pearson correlation R=0.44. Finally, in these three groups the average increase of vitamin D was: 1833IU. Conclusions: We found a relationship between CCD and hypovitaminosis D, which could be corrected increasing Vitamin D dose.

  16. A case report of minimal change nephrotic syndrome complicated with portal, splenic and superior mesenteric vein thrombosis.

    Science.gov (United States)

    Wang, Jun; Fan, QiuLing; Chen, Ying; Dong, Xuezhu; Zhang, YuXia; Feng, JiangMin; Ma, JianFei; Wang, LiNing

    2012-06-01

    Venous thrombosis is common in nephrotic syndrome, but portal vein thrombosis has a relatively low incidence in patients with nephrotic syndrome. We describe here a case of an 18-year old male student with newly diagnosed nephrotic syndrome that was complicated with portal, splenic and superior mesenteric vein thrombosis. In the presence of newly diagnosed nephrotic syndrome of minimal change disease, thrombus formation can occur and should be noted, particularly when it occurs, in rare sites. The recognition in nephrotic syndrome complicated with portal, splenic and superior mesenteric vein thrombosis should be emphasized.

  17. 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.

  18. Association between childhood nephrotic syndrome and hemophagocytic lymphohistiocytosis.

    Science.gov (United States)

    Landau, Daniel; Gurevich, Evgenia; Kapelushnik, Joseph; Tamary, Hannah; Shelef, Ilan; Lazar, Isaac

    2013-12-01

    Hemophagocytic lymphohistiocytosis (HLH) is caused by an excessive activation of nonmalignant macrophages. Renal lesions have been described in association with, but always after, HLH diagnosis. We describe a previously healthy 26-month-old girl who presented originally with steroid-responsive nephrotic syndrome (NS), but after 4 months, on the first NS relapse, experienced numerous complications (many of them reported to accompany NS as single events). Clinical and laboratory signs of HLH evolved with time and led to deterioration of her condition and death, within 5 months of her original presentation. To our knowledge, this is the first report of NS antedating the presentation of HLH.

  19. 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.

  20. 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.

  1. History of Nephrotic Syndrome and Evolution of its Treatment

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    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.

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

    Science.gov (United States)

    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.

  3. A case of nephrotic syndrome associated with hydatiform mole

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    Tarlan Hamidehkhou

    2010-12-01

    Full Text Available The present case study is on a 16-year-old woman who was suffering from nephritic syndrome after recovery from complete type of hydatiform mole. She admitted in hospital because of proteinurea and hematuria. In her past medical history she had a suction curettage for hydatiform mole. After she received 4 courses chemotherapy, she completely recovered and βhCG has fallen from 1980 IU/L to under 10 IU/mL. Then she showed generalized edema, proteinurea and hematuria compatible with nephritic syndrome. After six courses chemotherapy the symptoms of nephrotic syndrome and invasive mole diminished, she released from hospital and scheduled for follow-up.

  4. Pathogenesis of minimal change nephrotic syndrome: an immunological concept

    Science.gov (United States)

    Kim, Seong Heon; Park, Se Jin; Han, Kyoung Hee; Kronbichler, Andreas; Saleem, Moin A.; Oh, Jun; Lim, Beom Jin

    2016-01-01

    Idiopathic nephrotic syndrome (INS) in children is characterized by massive proteinuria and hypoalbuminemia. Minimal change nephrotic syndrome (MCNS) is the most common form of INS in children. The pathogenesis of MCNS still remains unclear, however, several hypotheses have been recently proposed. For several decades, MCNS has been considered a T-cell disorder, which causes the impairment of the glomerular filtration barrier with the release of different circulating factors. Increased levels of several cytokines are also suggested. Recently, a "two-hit" theory was proposed that included the induction of CD80 (B7-1) and regulatory T-cell (Treg) dysfunction, with or without impaired autoregulatory functions of the podocyte. In contrast to the well-established involvement of T cells, the role of B cells has not been clearly identified. However, B-cell biology has recently gained more attention, because rituximab (a monoclonal antibody directed against CD20-bearing cells) demonstrated a very good therapeutic response in the treatment of childhood and adult MCNS. Here, we discuss recent insights into the pathogenesis of MCNS in children. PMID:27279884

  5. Nephrotic syndrome with a nephritic component associated with toxoplasmosis in an immunocompetent young man.

    Science.gov (United States)

    Barrios, Julio E; Duran Botello, Claudia; González Velásquez, Tania

    2012-07-01

    Although the association of infection by toxoplasmosis with the development of nephrotic syndrome is uncommon, cases of this association have nevertheless been reported in the literature for more than two decades, not only for congenital toxoplasmosis, but also in acquired cases, and occasionally in immunocompetent patients. A case is presented of an immunocompetent patient aged 15 with clinical and laboratory indications of nephrotic/nephritic syndrome, in whom serological tests showed Toxoplasma infection. The presentation of nephrotic syndrome in ages where it is not commonly seen, leads to clinical suspicion of secondary causes. Active search for possible causes should include common tropical infections.

  6. Cerebral Venous Thrombosis in the Nephrotic Syndrome

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    Al Fakeeh Khalid

    2000-01-01

    Full Text Available A 4-year-old boy with idiopathic steroid responsive nephritic syndrome developed bilateral sixth-nerve palsy and lethargy secondary to cerebral sinus thrombosis. Treatment with heparin, fresh frozen plasma as source of antithrombin III and vitamin K inhibitors may have prevented further sequels. However, anti-coagulation, as assessed by partial thromboplastin and prothrombin time, was difficult to achieve. Despite these problems the child made a complete neurological recovery.

  7. Steroidogenic impairment due to reduced ovarian transcription of cytochrome P450 side-chain-cleavage (P450scc) and steroidogenic acute regulatory protein (StAR) during experimental nephrotic syndrome.

    Science.gov (United States)

    Peña-Rico, Miguel; Guadalupe Ortiz-López, María; Camacho-Castillo, Luz; Cárdenas, Mario; Pedraza-Chaverri, José; Menjívar, Marta

    2006-07-10

    The nephrotic syndrome is a renal disease characterized by proteinuria, hypoproteinemia, edema and hyperlipidemia. It has been reported that female nephrotic rats are characterized by loss of the oestrus cycle, follicle atresia, low gonadotropin and steroid concentrations; particularly, undetectable estradiol levels. Therefore, to determine the mechanisms involved in the ovarian steroidogenesis impairment, in this present study we evaluated the ovarian expression of the essential steroidogenesis components: cytochrome P450 side cholesterol chain cleavage enzyme (P450scc) and steroidogenic acute regulatory protein (StAR). The experiments were conducted in the rat experimental model of nephrosis induced by puromycin aminonucleoside (PAN) and in control groups. The evaluation of the expression of P450scc and StAR mRNA were performed during the acute phase of nephrosis as well as after the exogenous administration of 1 or 4 doses of human chorionic gonadotrophin (hCG), or a daily dose of FSH or FSH+hCG for 10 days. In addition, serum hormone concentrations, intra-ovarian steroid content, and the reproductive capacity were determined. The results revealed a decreased expression of mRNA of P450scc enzyme and StAR during nephrosis, and eventhough they increased after gonadotropins treatment, they did not conduce to a normal cycling rat period or fertility recovery. This study demonstrates that the mechanism by which ovarian steroid biosynthesis is altered during acute nephrosis involves damage at the P450scc and StAR mRNA synthesis and processing.

  8. Patterns of childhood nephrotic syndrome in Aljouf region, Saudi Arabia

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    Abdulaziz Alhassan

    2013-01-01

    Full Text Available To determine the patterns in children with nephrotic syndrome (NS in our region, we retrospectively studied 25 nephrotic patients evaluated and followed-up in the hospitals of the Aljouf region in Saudi Arabia. The male to female ratio was 2:1. The incidence of idiopathic NS was two to six cases per 100,000 children/year, while the prevalence was 12 cases per 100,000 children. Five patients presented with hypertension, seven (28% with respiratory tract infection, three (12% with tender abdomen, two (8% with gross hematuria, one (4% with thrombosis of renal veins with seizure and shock and the remaining seven presented to the hospital without complications. Twenty-three (92% patients were sensitive to the first steroid course and two (8% patients were steroid resistant, and both of them proved to have focal segmental glomerulosclerosis (FSGS on biopsy. Of those who responded, six (24% patients remained in remission, while 17 (68% patients became steroid dependant. Of those who were diagnosed as steroid dependent, three patients were biopsied and one of them was diagnosed as FSGS, while the remaining two had minimal change glomerulonephritis. Regarding steroid-dependent patient relapses, seven (41% patients showed infrequent relapses and ten (59% patients had frequent relapses. We conclude that the patterns of NS and the response to treatment observed in this study did not differ significantly from studies from other places in the world.

  9. Late-Onset Nephrotic Syndrome in Galloway-Mowat Syndrome: A Case Report

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    Hazza Issa

    1999-01-01

    Full Text Available Galloway-Mowat Syndrome (GMS has a wide variety of clinical manifestations and histologic findings. All reported cases had developed nephrotic syndrome in the first two years of life. We report a case of 12 years old boy with microcephaly, mental retardation, and typical dysmorphic features of GMS with a late onset of minimal change nephritic syndrome which first manifested at seven years of age.

  10. 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.

  11. The efficacy and safety of rituximab in treating childhood nephrotic syndrome: an Italian perspective.

    Science.gov (United States)

    Maratea, Dario; Bettio, Monica; Corti, Maria Grazia; Montini, Giovanni; Venturini, Francesca

    2016-07-12

    Nephrotic syndrome is a disorder characterized by proteinuria, hypoalbuminemia and dyslipidemia. Low-dose alternate-day steroid regimen is the standard of care. In case of relapse or significant adverse events, steroid-sparing agents may be used. This analysis was aimed at assessing the efficacy and safety of rituximab for the treatment of children with nephrotic syndrome. Four studies were included in the final meta-analysis. The end-point of our analysis was the percentage of patients in remission at 6 months. Pooled data from the four studies favours the use of rituximab (RR 5.25, 95 % CI: 3.05-9.06; p nephrotic syndrome: 1. favourable clinical efficacy and safety data; 2. no available alternatives; 3. outcome data collecting by AIFA through prescribers. In conclusion, our results report a significant incremental benefit of adding rituximab to corticosteroid and/or calcineurin inhibitors for the treatment of nephrotic syndrome.

  12. Dimethylarginines as risk markers of atherosclerosis and chronic kidney disease in children with nephrotic syndrome.

    Science.gov (United States)

    Hyla-Klekot, Lidia; Bryniarski, Piotr; Pulcer, Barbara; Ziora, Katarzyna; Paradysz, Andrzej

    2015-01-01

    Nephrotic syndrome in children is commonly associated with dyslipidemia, which is considered a risk factor for endothelial dysfunction and atherosclerosis. Recently new markers of endothelial dysfunction, such as asymmetric dimethylarginine (ADMA), have gained importance. Another L-arginine derivative--symmetric dimethylarginine (SDMA)--may reflect the glomerular filtration rate (GFR). The main aim of this study was to assess ADMA as a marker of atherosclerosis. Secondly, SDMA was examined for GFR assessment. The study involved 32 children with nephrotic syndrome. Several parameters were examined in the remission and relapse phases of nephrotic syndrome, including ADMA, SDMA, cholesterol, triglycerides and GFR. In the relapse phase there was a negative correlation between ADMA and lipids (cholesterol and triglycerides). In both phases SDMA was negatively correlated with GFR. The role of ADMA as a marker for endothelial dysfunction is not significant. SDMA may be utilized to monitor GFR in children with nephrotic syndrome.

  13. 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.

  14. Superior sagittal sinus thrombosis: a rare complication in a child with nephrotic syndrome

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    Pirogovsky, A.; Adi, M.; Barzilai, N. [Dept. of Radiology, Kaplan Medical Center, Rehovot (Israel); Dagan, A.; Sinai, L.; Sthoeger, D. [Div. of Paediatrics, Kaplan Medical Center, Rehovot (Israel); Tabachnik, E. [Div. of Paediatrics, Kaplan Medical Center, Rehovot (Israel); Paediatric ICU, Kaplan Hospital, Rehovot (Israel)

    2001-10-01

    A 2-year-old boy with new-onset nephrotic syndrome developed recurrent vomiting, apathy and papilloedema. Superior sagittal sinus thrombosis was diagnosed on cranial CT and MRI. He gradually recovered after treatment with heparin, fresh frozen plasma and warfarin with complete resolution of the thrombosis after 1 month. Superior sagittal sinus thrombosis is an extremely rare complication of nephrotic syndrome in children. Early diagnosis is essential for institution of anticoagulation therapy and a successful outcome. (orig.)

  15. Superior sagittal sinus thrombosis: a rare complication in a child with nephrotic syndrome

    International Nuclear Information System (INIS)

    Pirogovsky, A.; Adi, M.; Barzilai, N.; Dagan, A.; Sinai, L.; Sthoeger, D.; Tabachnik, E.

    2001-01-01

    A 2-year-old boy with new-onset nephrotic syndrome developed recurrent vomiting, apathy and papilloedema. Superior sagittal sinus thrombosis was diagnosed on cranial CT and MRI. He gradually recovered after treatment with heparin, fresh frozen plasma and warfarin with complete resolution of the thrombosis after 1 month. Superior sagittal sinus thrombosis is an extremely rare complication of nephrotic syndrome in children. Early diagnosis is essential for institution of anticoagulation therapy and a successful outcome. (orig.)

  16. Candida spp. and gingivitis in children with nephrotic syndrome or type 1 diabetes

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    Olczak-Kowalczyk, Dorota; Pyr?ak, Beata; D?bkowska, Maria; Pa?czyk-Tomaszewska, Ma?gorzata; Miszkurka, Gra?yna; Rogozi?ska, Izabela; Swoboda-Kope?, Ewa; Gozdowski, Dariusz; Kali?ska, Angelika; Pir?g, Anna; Mizerska-Wasiak, Ma?gorzata; Roszkowska-Blaim, Maria

    2015-01-01

    Background Diabetes and Nephrotic syndrome (NS) promote plaque-related gingivitis and yeast-like fungal infections. The study assesses the impact of Candida spp. and general disease- or treatment-related factors on plaque-related gingivitis severity in children and adolescents with Nephrotic syndrome /diabetes. Methods Body mass index (BMI), BMI standard deviation score, and oral cavity (Plaque Index ? PLI,?Gingival Index ? GI, mucosa status, presence and Candida enzymatic activity) were asse...

  17. 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.

  18. Idiopathic Nephrotic Syndrome in Childhood: A Retrospective Analysis of Two Hundred and Eighty Nine Patients

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    Kenan Yılmaz

    2017-12-01

    Full Text Available Aim: In this study, we aimed to evaluate the demographic and histopathological characteristics and response to medications in children with idiopathic nephrotic syndrome in Turkey. Methods: We reviewed medical records of patients older than one year, who were newly diagnosed with nephrotic syndrome and had been followed for at least one year in our department between November 1994 and March, 2013. Results: A total of 289 children (169 boys were included in the study. Fifty theree patients (18.4% were with steroid-resistant nephrotic syndrome, 33 (11.4% with frequently relapsing nephrotic syndrome and 53 (18.4% were with steroid-dependent nephrotic syndrome. Cyclosporine A (CsA, cyclophosphamide, mycophenolate mofetil, levamisole, azathioprine, and rituximab were used as steroid-sparing agents in some patients. The number of patients who were responder to steroid and to CsA was similar. Majority of patients with steroid-resistant nephrotic syndrome were also resistant to mycophenolate mofetil and CsA. Conclusion: There was a high prevalence of minimal change disease based on kidney biopsy especially in boys younger than six years of age and response to steroid and CsA was almost similar.

  19. Sonography and dynamic scintigraphy with 99mTc-DTPA in children with nephrotic syndrome

    International Nuclear Information System (INIS)

    Bliznakova, D.; Klisarove, A.

    2000-01-01

    The aim of the study is to assay the clinical application of kidney sonography and dynamic scintigraphy with 99m Tc-DTPA in children presenting nephrotic syndrome. A total of 32 children (mean age 9.5±1.2) are covered by the study, with the most important laboratory investigations being performed. All patients undergo abdominal sonography and dynamic kidney scintigraphy following iv administration of 100 μC/kg 99m Tc-DTPA, and glomerular filtration clarence (GFR) measured by the method of full and empty syringe activity. The functional curves are also shown. In children with primary nephrotic syndrome the sonographic imaging reveals enlarged kidney size and enhanced sonogeneity of parenchyma in the first stage. In patients with secondary nephrotic syndrome increased kidney size is likewise observed with enhanced sonogeneity of parenchyma in second stage and unclear visualization of pyramids. In children with idiopathic nephrotic syndrome the scintigraphic data confirm the enlarged kidneys with moderately increased values of GFR. In the mixed forms of nephrotic syndrome the kidneys preserve their moderate enlargement against the background of heterogeneous GFR values. In 5 patients the functional curves show kidney excretion impairment. The study confirms that sonographic imaging correlates well with the dynamic scintigraphic 99m Tc-DTPA imaging in children with nephrotic syndrome. The functional curves and GFR values promote accurate diagnosing and monitoring of the dynamic pathological processes. (author)

  20. 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.

  1. 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.

  2. The rationale and design of Insight into Nephrotic Syndrome: Investigating Genes, Health and Therapeutics (INSIGHT): a prospective cohort study of childhood nephrotic syndrome.

    Science.gov (United States)

    Hussain, Neesha; Zello, J Anastasia; Vasilevska-Ristovska, Jovanka; Banh, Tonny M; Patel, Viral P; Patel, Pranali; Battiston, Christopher D; Hebert, Diane; Licht, Christoph P B; Piscione, Tino D; Parekh, Rulan S

    2013-01-26

    Nephrotic syndrome is one of the most commonly diagnosed kidney diseases in childhood and its progressive forms can lead to chronic kidney disease (CKD) and/or end-stage renal disease (ESRD). There have been few longitudinal studies among a multi-ethnic cohort to determine potential risk factors influencing disease susceptibility, treatment response, and progression of nephrotic syndrome. Temporal relationships cannot be studied through cross-sectional study design. Understanding the interaction between various factors is critical to developing new strategies for treating children with kidney disease. We present the rationale and the study design of a longitudinal cohort study of children with nephrotic syndrome, the Insight into Nephrotic Syndrome: Investigating Genes, Health and Therapeutics (INSIGHT) study. The specific aims are to determine: 1) socio-demographic, environmental, and genetic factors that influence disease susceptibility; 2) rates of steroid treatment resistance and steroid treatment dependence, and identify factors that may modify treatment response; 3) clinical and genetic factors that influence disease susceptibility and progression to CKD and ESRD; and 4) the interaction between the course of illness and socio-demographic, environmental, and clinical risk factors. INSIGHT is a disease-based observational longitudinal cohort study of children with nephrotic syndrome. At baseline, participants complete questionnaires and provide biological specimen samples (blood, urine, and toenail clippings). Follow-up questionnaires and repeat biological specimen collections are performed annually for up to five years. The proposed cohort will provide the structure to test various risk factors predicting or influencing disease susceptibility, treatment response, and progression to CKD among children with nephrotic syndrome. ClinicalTrials.gov Identifier NCT01605266.

  3. Childhood Idiopathic Steroid Resistant Nephrotic Syndrome, Different Drugs and Outcome

    International Nuclear Information System (INIS)

    Shah, S. S. H.; Hafeez, F.

    2016-01-01

    Background: The management of steroid resistant nephrotic syndrome (SRNS) is quite difficult in paediatric patients. Not only the remission is difficult but also these patients are at risk of progression to end stage renal disease (ESRD). The goal of treatment is either to achieve complete remission or even partial remission as it is the most important predictor of disease outcome. Methods: This study was conducted at The Children Hospital, Lahore from February 2014 to May 2015. The SRNS patients of either sex between ages of 1-12 years were included with histology showing mesangioproliferative glomerulonephritis (MesangioPGN), focal segmental glomerulosclerosis (FSGS) or minimal change disease (MCD). Patients were given different immunosuppressant drugs and steroid 30 mg/m/sup 2/ alternate day therapy on case to case basis and kept on regular follow up to check for response and adverse effects. Results: Total of 105 patients included, 63 (60 percent) male and 42 (40 percent) female patients. The age ranges from 1.08 to 12 years, mean age of 6.53 years and SD of ±3.17. Tacrolimus was the most common drug used 43 (41 percent) patients followed by cyclosporine in 38 (36.2 percent) patients, while Mycophenolate mofetil (MMF) was prescribed in 21 (20 percent) patients. Complete response was in 96 (91.4 percent) initially while partial response was seen in 8 (7.6 percent) patients. On follow up, 92 (87.6 percent) patients showed complete response and partial response was in 5 (4.7 percent) patients. Cushingoid features and hypertrichosis were the most common adverse effect seen. Conclusion: Steroid resistant nephrotic syndrome can be managed well with various immunosuppressant drugs and steroids but treatment should be individualized according to clinical presentation, disease histology and cost/social factors. (author)

  4. Paraoxnase1 Gene Polymorphism in Childhood Idiopathic Nephrotic Syndrome.

    Science.gov (United States)

    Al-Eisa, Amal A; Sukumaran, Vazhappilly J; Haider, Mohammad Z

    2016-01-01

    Paraoxonase1 (PON1) is a serum enzyme bound to high-density lipoproteins with antioxidant properties. Molecular studies of PON1 revealed 2 polymorphic sites at amino acids 55 and 192 resulting in 2 different allozymes, the L and M-genotype at residue 55 and A and B at site 192, respectively. We have studied the association between PON1 gene polymorphisms and the minimal change nephrotic syndrome/focal segmental glomerulosclerosis (MCNS/FSGS) types of idiopathic nephrotic syndrome (INS) in Kuwaiti Arab children. The PON1 gene, 55 and 192 polymorphisms were analyzed in 50 children with INS (32 MSCN, 18 FSGS) and compared to 50 controls. Serum creatinine, albumin and lipids were measured in all subjects. The LL genotype was detected in 50% of the INS patients compared to 48% of controls (p = 0.84). The heterozygous LM genotype was detected in 42% of INS patients compared 36% of controls (p = 0.68). The MM-genotype was detected in 8% of INS patients and 16% of controls (p = 0.35). The L-allele frequency in its homozygous and heterozygous forms was found in 71% of INS patients compared to 66% controls (p = 0.54). The L-allele frequency (LM and LL) was significantly higher in FSGS compared to MCNS patients (p = 0.0001) and when compared to controls (p = 0.0007). All patients and controls had the AA form of the 192 PON1 gene polymorphism. Our data demonstrate a strong association between the L-allele of PON1 gene 55 polymorphism with FSGS in Kuwaiti Arab children with INS. PON1 genotyping can help in the early prediction of FSGS, which might guide clinicians to a better therapeutic approach. © 2016 S. Karger AG, Basel.

  5. Childhood Idiopathic Steroid Resistant Nephrotic Syndrome, Different Drugs And Outcome.

    Science.gov (United States)

    Hussain Shah, Syed Sajid; Hafeez, Farkhanda

    2016-01-01

    The management of steroid resistant nephrotic syndrome (SRNS) is quite difficult in paediatric patients. Not only the remission is difficult but also these patients are at risk of progression to end stage renal disease (ESRD). The goal of treatment is either to achieve complete remission or even partial remission as it is the most important predictor of disease outcome. This study was conducted at The Children's Hospital, Lahore from February 2014 to May 2015. The SRNS patients of either sex between ages of 1-12 years were included with histology showing mesangioproliferative glomerulonephritis (MesangioPGN), focal segmental glomerulosclerosis (FSGS) or minimal change disease (MCD). Patients were given different immunosuppressant drugs and steroid 30 mg/m2 alternate day therapy on case to case basis and kept on regular follow up to check for response and adverse effects. Total of 105 patients included, 63 (60%) male and 42 (40%) female patients. The age ranges from 1.08 to 12 years, mean age of 6.53 years and SD of ±3.17. Tacrolimus was the most common drug used 43 (41%) patients followed by cyclosporine in 38 (36.2%) patients, while Mycophenolate mofetil (MMF) was prescribed in 21 (20%) patients. Complete response was in 96 (91.4%) initially while partial response was seen in 8 (7.6%) patients. On follow up, 92 (87.6%) patients showed complete response and partial response was in 5 (4.7%) patients. Cushingoid features and hypertrichosis were the most common adverse effect seen. Steroid resistant nephrotic syndrome can be managed well with various immunosuppressant drugs and steroids but treatment should be individualized according to clinical presentation, disease histology and cost/social factors.

  6. Nephrotic syndrome in primary myelofibrosis with renal extramedullary hematopoiesis and glomerulopathy in the JAK inhibitor era.

    Science.gov (United States)

    Del Sordo, Rachele; Brugnano, Rachele; Covarelli, Carla; Fiorucci, Gioia; Falzetti, Franca; Barbatelli, Giorgio; Nunzi, Emidio; Sidoni, Angelo

    2017-01-01

    Primary myelofibrosis (PMF) is an uncommon form of myeloproliferative neoplasm (MPN) characterized by a proliferation of predominantly megakaryocytes and granulocytes in the bone marrow that, in fully-developed disease, is associated with reactive deposition of fibrous connective tissue, extramedullary hematopoiesis (EMH), and splenomegaly. Kidney involvement is rare and clinically presents with proteinuria, nephrotic syndrome, and renal insufficiency. Renal damage can be due to EMH and glomerulopathy. Renal EMH presents three patterns: infiltration of the interstitium with possible renal failure caused by functional damage of parenchyma and vessels, infiltration of capsule and pericapsular adipose tissue, and sclerosing mass-like lesions that can cause hydronephrosis and hydroureter with obstructive uropathy and renal failure. Glomerulopathy associated with PMF is rarely described, ranging from 1 month to 18 years from diagnosis of the neoplasm to renal biopsy. It is characterized by expansion and hypercellularity mesangial, segmental sclerosis, features of chronic thrombotic microangiopathy (TMA), and intracapillary hematopoietic cells infiltrating in absence of immune-mediated glomerulonephritis. We present a nephrotic syndrome in PMF-related glomerulopathy, associated with EMH, without renal failure, in a patient under treatment for 2 years with JAK2 inhibitor ruxolitinib. Despite treatment, the patient died 7 months after renal biopsy. Nephrologists still know very little about this topic and there is no homogeneous data about incidence, pathogenesis, and optimal treatment of this poor prognostic PMF-associated nephrotic syndrome. We focus on data in the literature in the hope of stimulating hematologists, nephrologists, pathologists to future studies about the natural history of renal involvement, useful for optimal management of this rare pathology.

  7. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

  9. Severe ocular hypertension secondary to systemic corticosteroid treatment in a child with nephrotic syndrome

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    Brito PN

    2012-10-01

    Full Text Available Pedro Nuno Brito,1 Sérgio Estrela Silva,1 José Silva Cotta,1 Fernando Falcão-Reis1,21Ophthalmology Department, Hospital S João, Porto, Portugal; 2Faculty of Medicine of Porto, University of Porto, Porto, PortugalPurpose: To report a case of severe, acute ocular hypertension in a 6-year-old child, 7 days after initiating treatment with oral prednisolone, due to nephrotic syndrome.Methods: A 6-year-old female Caucasian child was diagnosed with nephrotic syndrome and treated with oral prednisolone (60 mg/day. Seven days later the child initiated complaints of headache, vomiting, ocular pain, and photophobia. Ophthalmologic examination revealed a severely increased intraocular pressure (IOP of 52 mmHg in the right eye and 56 mmHg in the left eye. Anterior segment morphology was evaluated with ultrasound biomicroscopy. Optic disc status was evaluated by disc photography, kinetic perimetry, and optical coherence tomography.Results: Treatment was initiated with latanoprost, brimonidine, and the fixed association of timolol and dorzolamide. At each follow-up examination, progressively better control of IOP was obtained. Simultaneous with corticosteroid dosage decrease we were able to reduce antiglaucomatous medication while maintaining IOP under control. Ultrasound biomicroscopy revealed an open angle with normal anterior segment echographic findings. Perimetric evaluation revealed normal visual fields in both eyes. Four months after presentation, steroid treatment had been completed and IOP was 10 mmHg in both eyes without any antiglaucomatous medication. Optical coherence tomography revealed normal retinal nerve fiber layer thickness in all peripapillary sectors.Conclusions: Systemic steroid treatment can cause a severe, acute increase in IOP in children. Children undergoing steroid treatment should have routine ophthalmologic examinations during treatment duration. Prompt antiglaucomatous treatment prevents retinal nerve fiber layer damage and

  10. The role of novel biomarkers in childhood idiopathic nephrotic syndrome: a narrative review of published evidence

    Directory of Open Access Journals (Sweden)

    Uwaezuoke SN

    2017-06-01

    Full Text Available Samuel N Uwaezuoke Department of Pediatrics, Pediatric Nephrology Firm, University of Nigeria Teaching Hospital, Ituku-Ozalla, Enugu, Nigeria Abstract: Two histological subtypes of idiopathic nephrotic syndrome are commonly recognized in children, namely minimal change nephropathy and focal segmental glomerulosclerosis. Children with minimal change nephropathy (the majority of whom are steroid-sensitive and focal segmental glomerulosclerosis (the majority of whom are steroid-resistant require early identification in order to ensure appropriate therapeutic intervention and better outcome. Although renal biopsy and histology remain the ideal diagnostic steps to identify these histological subtypes, reports indicate that serum and urinary biomarkers are now being utilized in the investigation of childhood idiopathic nephrotic syndrome. This paper aims to review the diagnostic and prognostic utility of novel biomarkers in childhood idiopathic nephrotic syndrome and to highlight their role in differentiating steroid-sensitive nephrotic syndrome (SRNS from steroid-resistant nephrotic syndrome (SSNS. Using the terms “idiopathic nephrotic syndrome,” “children,” and “biomarkers” the PubMed database was searched for relevant studies related to the topic. Biomarkers such as adiponectin, neopterin, β2-microglobulin, and N-acetyl-β-D glucosaminidase were reported as diagnostic markers. In addition to neopterin and N-acetyl-β-D glucosaminidase, urine vitamin D-binding protein and α1β-glycoprotein were shown to differentiate SRNS from SSNS while N-acetyl-β-D glucosaminidase and β2-microglobulin could predict steroid responsiveness and renal outcome in SRNS. Although progress has been made in demonstrating the diagnostic and prognostic utility of these biomarkers, their limited availability in most laboratories has precluded a complete paradigm shift from the conventional renal biopsy. Nevertheless, further longitudinal studies are required

  11. Congenital nephrotic syndrome, an uncommon presentation of cytomegalovirus infection.

    Science.gov (United States)

    Rahman, H; Begum, A; Jahan, S; Muinuddin, G; Hossain, M M

    2008-07-01

    Cytomegalovirus (CMV) infection is a very rare infection in children as well as in infancy. In CMV infection extra renal manifestations in central nervous system (CNS), eyes and hematological are prominent and common than renal manifestation. We are describing two case reports one at the age of 5 months and another at one month with features of both extra renal and renal manifestations of nephritic syndrome. Our first case presented with predominant features of CNS manifestations like convulsion and spastic monoplegia, sensory type of deafness and absence of light reflexes along with nephritic features. Deafness and absence of light reflex were confirmed by audiometric and ophthalmological examination and brain atrophy was confirmed by CT scan. Our second case had features of hypothyroidism along with nephritic features. Hypothyroid status was confirmed by elevated serum TSH level and reduced T4 level. In both cases nephritic features were confirmed at bed site urine examination by heat coagulation test and other relevant investigations. CMV infection was confirmed in both cases by detecting anti CMV IgM by ELISA method. Both patients were clinically improved with intravenous gangcyclovir therapy. In these two cases reports, clinical, serological and therapeutic observation established the causal relationship between the CMV infection and nephrotic syndrome.

  12. Efficacy of rituximab therapy in children with refractory nephrotic syndrome: a prospective observational study in Shanghai.

    Science.gov (United States)

    Sun, Li; Xu, Hong; Shen, Qian; Cao, Qi; Rao, Jia; Liu, Hai-Mei; Fang, Xiao-Yan; Zhou, Li-Jun

    2014-02-01

    Idiopathic nephrotic syndrome is the most common glomerular disease in children. This study was undertaken to observe the efficacy and side-effects of rituximab (RTX) in treating children with different types of refractory primary nephrotic syndrome. Twelve patients with steroid dependent nephrotic syndrome (SDNS), frequently relapsing nephritic syndrome (FRNS), and steroid resistant nephrotic syndrome (SRNS) were enrolled in our study. There were obvious drug side-effects, and proteinuria remained difficult to control. RTX was administered at a dose of 375 mg/m(2) body surface area, once or twice weekly. The male to female ratio was 3:1, and the onset age was 1.6-8.9 years. There were 9 patients with steroid sensitive nephrotic syndrome (SDNS or FRNS), and 3 patients with SRNS. There were 7 patients with minimal change disease (MCD), 3 patients with focal segmental glomerular sclerosis (FSGS), 1 with focal proliferative glomerulonephritis, and 1 without renal biopsy. The total effective treatment rate of RTX was 91.67%, and for 77.78% of the patients, steroid dosage could be reduced. Six months before and after RTX infusion, the mean steroid dosage was significantly decreased (P=0.014) and the recurrence number was significantly reduced (Psyndrome, and it could significantly reduce the use of steroid and immunosuppressants.

  13. Nivolumab-associated Nephrotic Syndrome in a Patient With Renal Cell Carcinoma: A Case Report.

    Science.gov (United States)

    Daanen, Robin A; Maas, Rutger J H; Koornstra, Rutger H T; Steenbergen, Eric J; van Herpen, Carla M L; Willemsen, Annelieke E C A B

    Immune checkpoint inhibitors have taken an important place in the treatment of different types of malignancies. These drugs are known to have specific immune-mediated adverse events. We describe a case of severe nephrotic syndrome secondary to treatment with nivolumab in a patient with renal cell carcinoma. A 62-year-old man was treated with nivolumab for papillary renal cell carcinoma type 2 for 8 weeks when he was admitted to the hospital with a severe nephrotic syndrome and acute kidney injury. Renal biopsy showed focal segmental glomerulosclerosis. Treatment with high-dose corticosteroids had insufficient effect, but the addition of mycophenolate mofetil resulted in remission of the nephrotic syndrome and recovery of renal function. Proteinuria subsequently relapsed during corticosteroid tapering. The time course in this patient strongly suggests that the nephrotic syndrome occurred as an adverse drug reaction to nivolumab treatment. If during nivolumab treatment renal insufficiency, hypoalbuminemia, or proteinuria develops, further analysis for a possible nephrotic syndrome is warranted for early detection and treatment of this life-threatening complication.

  14. [Simultaneous onset of steroid-sensitive nephrotic syndrome and type 1 diabetes].

    Science.gov (United States)

    Rego Filho, Eduardo A; Mello, Solange F R; Omuro, André M; Loli, José O C

    2003-01-01

    We describe the case of a boy with steroid-sensitive nephrotic syndrome coexisting with type-1 diabetes mellitus. 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. The simultaneous occurrence of steroid-sensitive nephrotic syndrome and type-1 diabetes mellitus is rare.

  15. Rituximab in steroid-sensitive nephrotic syndrome: lessons from clinical trials.

    Science.gov (United States)

    Iijima, Kazumoto; Sako, Mayumi; Kamei, Koichi; Nozu, Kandai

    2017-07-17

    Idiopathic nephrotic syndrome is the most common chronic glomerular disease in children. A total of 80-90% of patients with childhood idiopathic nephrotic syndrome achieve remission with steroid therapy [steroid-sensitive nephrotic syndrome (SSNS)]. However, approximately 50% of children with SSNS develop frequently relapsing nephrotic syndrome (FRNS) or steroid-dependent nephrotic syndrome (SDNS). Children with FRNS or SDNS are usually treated with immunosuppressive agents, but 10-20% of children receiving immunosuppressive agents still show frequent relapses or steroid dependence during or after treatment, defined as complicated FRNS or SDNS. Rituximab, a chimeric anti-CD20 monoclonal antibody that was originally developed to treat patients with B-cell non-Hodgkin's lymphoma, is currently used for treating SSNS. In this review we highlight recent studies, mainly randomized controlled trials of rituximab for SSNS, including complicated and uncomplicated forms of FRNS or SDNS in children. We also discuss the effects of these studies on the management of patients suffering from these conditions.

  16. The Importance of Mean Platelet Volume in Children with Nephrotic Syndrome

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    Nilüfer Göknar

    2016-06-01

    Full Text Available Objective: The importance of mean platelet volume (MPV in chronic inflammation was demonstrated in many dis­eases. We aimed to evaluate the role of MPV in nephrotic syndrome of childhood and relation with steroid response. Methods: We enrolled 59 children with nephrotic syndrome (24 steroid-resistance and 35 steroid-sensitive and 34 healthy controls into the study. We evaluated total cholesterol, proteinuria, CRP, and albumin from study group and MPV and platelet count from both control and study group. Results: MPV level was higher in children with nephrotic syndrome when compared with control (8.6 ± 1.39 fl versus 7.09 ± 0.96 fl, p<0.001. Also platelet count was elevated in study group (406.092 ± 125.891 / μl versus 321.382 ± 78.666 / μl, p<0.001. MPV level and platelet count was similar in control and study subjects (p=0.798 and p=0.56. Conclusion: We demonstrated elevated MPV and platelet count in children with nephrotic syndrome. MPV is cheap, simple and important method to demonstrate platelet activation in nephrotic syndrome.

  17. [Idiopathic Nephrotic Syndrome: recommendations of the Nephrology Branch of the Chilean Society of Pediatrics. Part two].

    Science.gov (United States)

    Hevia, Pilar; Nazal, Vilma; Rosati, María Pía; Quiroz, Lily; Alarcón, Claudia; Márquez, Sonia; Cuevas, Karen

    2015-01-01

    Idiopathic nephrotic syndrome is the most common glomerular disease in childhood, affecting 1 to 3 per 100,000 children under the age of 16. It most commonly occurs in ages between 2 and 10. Its cause is unknown, and its histology corresponds to minimal change disease in 90% of cases, or focal segmental glomerulosclerosis. Steroid-resistant nephrotic syndrome represents 10-20% of idiopathic nephrotic syndrome in pediatrics. It has a poor prognosis, and its management is a significant therapeutic challenge. Half of patients evolve to end-stage renal disease within 5 years, and are additionally exposed to complications secondary to persistent NS and to the adverse effects of immunosuppressive therapy. The primary goal of treatment is to achieve complete remission, but even a partial remission is associated with a better renal survival than the lack of response. This paper is the result of the collaborative effort of the Nephrology Branch of the Chilean Society of Pediatrics with aims at helping pediatricians and pediatric nephrologists to treat pediatric idiopathic nephrotic syndrome. In this second part, handling of steroid-resistant nephrotic syndrome as well as nonspecific therapies are discussed. Copyright © 2015 Sociedad Chilena de Pediatría. Publicado por Elsevier España, S.L.U. All rights reserved.

  18. CHILDHOOD IDIOPATHIC NEPHROTIC SYNDROME AND ATOPIC DISEASES: IS THERE A RELATIONSHIP?

    Science.gov (United States)

    Chimenz, R; Romeo, A C; Fede, C; Piraino, B; Miraglia Del Giudice, M; Leonardi, S; Cuppari, C

    2015-01-01

    Nephrotic syndrome is a condition of massive proteinuria that leads to hypoalbuminaemia and oedema. In the pediatric age, the most common form of nephrotic syndrome is childhood idiopathic nephrotic syndrome (CINS). Although the etiological mechanisms underlying CINS are still unclear, the disease is considered to be immune-mediated. Several studies have previously reported a possible association between CINS and atopy, with the latter defined as abnormal immunoglobulin-E response on the background of a T-helper 2 (Th2)-driven immune system. In fact, both experimental and clinical studies have suggested that idiopathic nephrotic syndrome can be associated and/or triggered by a wide array of atopic diseases, though this remains a highly controversial topic. Exposure to inhalant-allergens (and/or introduction of food-allergens) has been previously correlated with the onset and/or the relapse of CINS in some children and a significant worse response to steroid therapy has been also described in reports of CINS associated to concomitant atopic diseases. In this review, we analyzed previous studies with the aim to clarify, basing on the existent literature, the association between atopy and idiopathic nephrotic syndrome. Additionally, we also speculated on the underlying immunological pathways that could potentially make some children prone to both CINS and atopic diseases.

  19. Determinants of plasma homocyst(e)ine in patients with nephrotic syndrome.

    Science.gov (United States)

    Joven, J; Arcelús, R; Camps, J; Ordóñez-Llanos, J; Vilella, E; González-Sastre, F; Blanco-Vaca, F

    2000-01-01

    Hyperhomocyst(e)inemia is an independent risk factor for atherothrombosis in several clinical settings in which renal function is impaired, but its prevalence in the nephrotic syndrome has not been investigated in detail, even though this syndrome provides an excellent model in which to study a possible link between albuminuria, proteinuria, and hyperhomocyst(e)inemia. We obtained plasma and urine from 27 patients with biopsy-confirmed membranous glomerulonephritis presenting nephrotic syndrome and 27 matched controls and determined the concentrations of homocyst(e)ine and proteins considered putative markers of glomerular and tubular function. Hyperhomocyst(e)inemia, defined as the mean +SD of the plasma homocyst(e)ine concentration of the controls [plasma homocyst(e)ine concentration >10.8 micromol/l] was present in 26% of the patients with nephrotic syndrome but in only 7.4% of the controls. Furthermore, the degree of hyperhomocyst(e)inemia was more severe in the nephrotic patients than in the controls. The existence of renal failure, tubular damage, and, interestingly, relatively well conserved glomerular function barrier were the main predictors of increased levels of plasma homocyst(e)ine. In conclusion, hyperhomocyst(e)inemia is a frequent cardiovascular risk factor present in patients with nephrotic syndrome and renal failure, but it is not directly associated with proteinuria.

  20. Indications for kidney biopsy in idiopathic childhood nephrotic syndrome.

    Science.gov (United States)

    Alshami, Alanoud; Roshan, Abishek; Catapang, Marisa; Jöbsis, Jasper J; Kwok, Trevor; Polderman, Nonnie; Sibley, Jennifer; Sibley, Matt; Mammen, Cherry; Matsell, Douglas G

    2017-10-01

    Most cases of childhood nephrotic syndrome (NS) are due to minimal change disease (MCD), while a minority of children have focal segmental glomerulosclerosis (FSGS) and an unfavorable clinical course, requiring a kidney biopsy to confirm diagnosis. We hypothesized that clinical characteristics at diagnosis and initial response to corticosteroid treatment accurately predict FSGS and can be used to guide consistent practice in the indications for kidney biopsy. This was a case control study (1990-2012). Inclusion criteria included age 1-17 years, meeting the diagnostic criteria for NS, and having biopsy-proven FSGS or MCD. Clinical characteristics at diagnosis included age, kidney function [estimated glomerular filtration rate (eGFR)], hypertension, hematuria, nephritis (reduced eGFR, hematuria, hypertension), and response to steroids. From a total of 169 children who underwent kidney biopsy for NS we included 65 children with MCD and 22 with FSGS for analysis. There were no significant between-group differences in age, sex, or eGFR at the time of diagnosis. The FSGS group had a higher proportion of hypertension (40 vs. 15%; p = 0.02), hematuria (80 vs. 47%; p = 0.01), and nephritis (22 vs. 2%; p = 0.004) and was more likely to be steroid resistant after 6 weeks of treatment than the MCD group (67 vs. 19%; p < 0.001). As predictors of FSGS, hematuria had a high sensitivity of 0.80 [95% confidence interval (CI) 0.56-0.93] and low specificity of 0.53 (95% CI 0.39-0.66), nephritis had a low sensitivity of 0.22 (95% CI 0.07-0.48) and high specificity of 0.98 (95% CI 0.88-0.99), and steroid resistance had a low sensitivity of 0.67 (95% CI 0.43-0.85) and high specificity of 0.81 (95% CI 0.68-0.90). The combination of steroid resistance after 6 weeks of therapy and/or nephritis at diagnosis yielded the optimal sensitivity and specificity at 0.80 (95% CI 0.56-0.93) and 0.75 (95% CI 0.60-0.86), respectively, confirmed by the highest receiver operator characteristic

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

    Science.gov (United States)

    Ellis, Demetrius

    2015-01-01

    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.

  2. Hepatitis B viral infection with nephrotic syndrome treated with lamivudine.

    Science.gov (United States)

    Banu, N A; Khatoon, S; Quadir, E; Rahman, M M; Khan, M A

    2007-07-01

    A 04 years old boy with 02 months history of generalized oedema and scanty micturition was diagnosed as nephrotic syndrome with hepatitis B viral infection. He had evidence of active viral replication. After 01 month treatment with oral lamivudine, his urine became protein free and after 04 months, he had seroconversion from HBeAg+ve to HBeAg-ve. Lamivudine was continued for 01 year. He had no relapse after discontinuation of therapy and remained well after 36 months of completion of therapy. He had no evidence of active viral replication during this period, however HBsAg remained positive indication carrier state. As most children with HBV associated nephropathy have no evidence of chronic hepatitis, all such children must undergo HBV screening and for chronic liver disease if HBV screening is positive. As such children do not respond to prednisolone or other immunosuppresive therapy which might harm them, antiviral therapy should be considered. Lamivudine is a suitable alternative to IFN alpha owing to its low cost, ease of administration and fewer side effects.

  3. Cyclosporine/ketoconazole reduces treatment costs for nephrotic syndrome

    Directory of Open Access Journals (Sweden)

    A Iyengar

    2013-01-01

    Full Text Available Cyclosporine A (CyA is an effective agent for the treatment of glucocorticoid-dependent idiopathic nephrotic syndrome (GCDNS, but costs are prohibitive in resource-poor societies. The objectives of this study were to evaluate the efficacy and safety of reducing the dose of CyA by co-administering ketoconazole. A prospective study targeting children 2-18 years of age with GCDNS in remission with CyA monotherapy was conducted. CyA dose was reduced by 50% and ketoconazole was added at 25% of the recommended therapeutic dose, and the drug levels and therapeutic and adverse effects (AE were monitored. Continued combined therapy after completion of the 4-week trial period was offered. Ten patients (median age 9.5 years, range 3.0-16.0 years were enrolled in the study. At week 4, the CyA dose was 2.2 ± 0.7 mg/kg/day compared with 5.6 ± 0.9 mg/kg/day at enrolment ( P 50% without increased adverse events or drug monitoring needs. This intervention demonstrates how access of patients with limited resources to needed drugs can be improved by interference with physiological drug elimination.

  4. 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

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

    Directory of Open Access Journals (Sweden)

    Demetrius eEllis

    2016-01-01

    Full Text Available 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 physiologic 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 pathophysiologic, 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

  6. Treatment approaches in children with steroid sensitive nephrotic syndrome

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    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.

  7. The incidence of the nephrotic syndrome in childhood in Germany.

    Science.gov (United States)

    Franke, Ingo; Aydin, Malik; Llamas Lopez, Corinna Elke; Kurylowicz, Lisa; Ganschow, Rainer; Lentze, Michael; Born, Mark

    2018-02-01

    The incidence of childhood nephrotic syndrome (NS) in Germany is not well known. An ESPED-based nationwide collection of epidemiological data of children in 2005 and 2006. The mean age of NS at onset was 5.5 ± 3.7 years. The gender ratio of boys to girls was 1.8:1. The average length of stay was 15.5 ± 11.2 days, with younger children remaining significantly longer in hospital. Steroid-resistance was more common in children ≥8 years (p = 0.023). Focal-segmental glomerulosclerosis (FSGS) was more common in children >10 years (p = 0.029). The ratio of males to females with FSGS was 1:1.9, thus the FSGS risk for girls at onset was 3.3-times greater. Considering the available data, the incidence of NS in Germany is 1.2/100,000 in the population childhood NS.

  8. The non-immunosuppressive management of childhood nephrotic syndrome.

    Science.gov (United States)

    McCaffrey, James; Lennon, Rachel; Webb, Nicholas J A

    2016-09-01

    Idiopathic nephrotic syndrome (INS) is one of the most common renal diseases found in the paediatric population and is associated with significant complications, including infection and thrombosis. A high proportion of children enter sustained remission before adulthood, and therapy must therefore mitigate the childhood complications, while minimising the long-term risk to health. Here we address the main complications of INS and summarise the available evidence and guidance to aid the clinician in determining the appropriate treatment for children with INS under their care. Additionally, we highlight areas where no consensus regarding appropriate management has been reached. In this review, we detail the reasons why routine prophylactic antimicrobial and antithrombotic therapy are not warranted in INS and emphasise the conservative management of oedema. When pharmacological intervention is required for the treatment of oedema, we provide guidance to aid the clinician in determining the appropriate therapy. Additionally, we discuss obesity and growth, fracture risk, dyslipidaemia and thyroid dysfunction associated with INS. Where appropriate, we describe how recent developments in research have identified potential novel therapeutic targets.

  9. Evaluation of children with steroid-sensitive nephrotic syndrome in terms of allergies.

    Science.gov (United States)

    Yılmaz, Dilek; Yenigün, Ayşe; Sönmez, Ferah; Kurt Ömürlü, İmran

    2015-04-01

    The etiology of minimal-change disease is not fully known, it is believed to be mediated by the immune system. Minimal-change disease also reported as having association with atopy. In this study, atopy history, the levels of serum IgE, and skin prick test in children with steroid-sensitive nephrotic syndrome were investigated. A group of 30 children (mean age 7.7 ± 2.2 years, 56.6% male) diagnosed with steroid-sensitive nephrotic syndrome were included in the study. Serum immunoglobulin E levels and eosinophil counts were evaluated in children with steroid-sensitive nephrotic syndrome both in relapse and remission. Skin prick test was performed in remission. Of the 30 children investigated, 11 (36.7%) had a history of atopy. The median serum total IgE levels in nephrotic children in relapse, with (445 IU/mL) and without atopy (310 IU/mL) were significantly higher than those in remission (respectively, 200 IU/mL, p = 0.021, and 42 IU/mL, p = 0.001). The skin prick tests for all the allergens were evaluated as negative in all the patients. It was thought that increased IgE may reflect the activation of immune mechanism following various stimuli rather than a direct association with atopy in children with steroid-sensitive nephrotic syndrome.

  10. The clinical value of pulmonary perfusion imaging complicated with pulmonary embolism in children of nephrotic syndrome

    International Nuclear Information System (INIS)

    Lin Jun; Chen Ning; Miao Weibing; Peng Jiequan; Jiang Zhihong; Wu Jing

    2001-01-01

    To investigate the clinical features of complicated with pulmonary embolism nephrotic syndrome in children. 99m Tc-MAA pulmonary perfusion imaging was performed on 30 nephrotic syndrome in children with elevated plasma D-dimer. Results shown that 14 of 30 patients were found to have pulmonary embolism (46.7%). Pulmonary perfusion imaging showed an involvement of 1 pulmonary segment in 3 cases, 2 segments in 2 cases and over 3 segments in other 9 cases. Among them, there were 7 segments involved in one case. After two weeks of heparin anti-coagulative therapy, most cases showed a recovery. The result of this study suggested that pulmonary embolism is a common complication of nephrotic syndrome. Pulmonary perfusion imaging is simple, effective and accurate method for the diagnosis of pulmonary embolism, and it also can help to assess the value of clinical therapy

  11. Biochemical alteration in children with idiopathic nephrotic syndrome associated with an increased risk of sensorineural hearing loss; additional insights in cochlear renal relationship.

    Science.gov (United States)

    El Mashad, Ghada Mohamed; Abo El Fotoh, Wafaa Moustafa M; Zein El Abedein, Ahmed Mahmoud; Abd El Sadek, Fatma Abd El Raoof

    2017-06-01

    Children with Idiopathic Nephrotic Syndrome (INS) are at risk of hearing loss due to the adverse impact of medications and related immunological and genetic factors on both cochlea and kidney. So this work was planned to evaluate hearing status in children with INS and to clarify the possible associated risk factors by interpreting the clinical and laboratory profiles of those children. Ninety children with INS aged 5-14 years [30 patients with steroid-sensitive nephrotic syndrome (SSNS), 30 patients with steroid dependent/frequently relapsing nephrotic syndrome (SDNS/FRNS), and 30 patients with steroid-resistant nephrotic syndrome (SRNS)], and 90 age and sex matched normal controls were enrolled into this study. Laboratory measurements of serum calcium, creatinine, cholesterol, blood urea and other relevant investigations were done. Pure tone audiometry was done with the sensory-neural hearing loss (SNHL) diagnosed when the level bone conduction was >20 dB and the difference in air to the bone gap was children with INS had SNHL, mostly of mild degree HL and primarily occurred at the lower frequencies. A highly significant statistical difference between controls and various types of nephrotic syndrome regarding pure tone audiometry measurements at frequencies 250, 500, 1000 Hz, whereas insignificant difference interpreting pure tone audiometry measurements in 2000, 4000 and 8000 Hz. Children with different phenotypes of nephrotic syndrome are at risk of sensorineural hearing impairment. The hazards associated with this impairment were higher blood pressure, hypercholesterolemia, hypoalbuminemia, and hypocalcemia. Copyright © 2017 Elsevier B.V. All rights reserved.

  12. The role of novel biomarkers in childhood idiopathic nephrotic syndrome: a narrative review of published evidence.

    Science.gov (United States)

    Uwaezuoke, Samuel N

    2017-01-01

    Two histological subtypes of idiopathic nephrotic syndrome are commonly recognized in children, namely minimal change nephropathy and focal segmental glomerulosclerosis. Children with minimal change nephropathy (the majority of whom are steroid-sensitive) and focal segmental glomerulosclerosis (the majority of whom are steroid-resistant) require early identification in order to ensure appropriate therapeutic intervention and better outcome. Although renal biopsy and histology remain the ideal diagnostic steps to identify these histological subtypes, reports indicate that serum and urinary biomarkers are now being utilized in the investigation of childhood idiopathic nephrotic syndrome. This paper aims to review the diagnostic and prognostic utility of novel biomarkers in childhood idiopathic nephrotic syndrome and to highlight their role in differentiating steroid-sensitive nephrotic syndrome (SRNS) from steroid-resistant nephrotic syndrome (SSNS). Using the terms "idiopathic nephrotic syndrome," "children," and "biomarkers" the PubMed database was searched for relevant studies related to the topic. Biomarkers such as adiponectin, neopterin, β2-microglobulin, and N-acetyl-β-D glucosaminidase were reported as diagnostic markers. In addition to neopterin and N-acetyl-β-D glucosaminidase, urine vitamin D-binding protein and α1β-glycoprotein were shown to differentiate SRNS from SSNS while N-acetyl-β-D glucosaminidase and β2-microglobulin could predict steroid responsiveness and renal outcome in SRNS. Although progress has been made in demonstrating the diagnostic and prognostic utility of these biomarkers, their limited availability in most laboratories has precluded a complete paradigm shift from the conventional renal biopsy. Nevertheless, further longitudinal studies are required to establish their usefulness as noninvasive predictors of disease response to immunosuppressive therapy.

  13. FCGR2A single nucleotide polymorphism confers susceptibility to childhood-onset idiopathic nephrotic syndrome.

    Science.gov (United States)

    Rossi, Giovanni M; Bonatti, Francesco; Adorni, Alessia; Alberici, Federico; Bodria, Monica; Bonanni, Alice; Ghiggeri, Gian M; Martorana, Davide; Vaglio, Augusto

    2018-01-01

    Childhood-onset idiopathic nephrotic syndrome affects 1.15-3.4 children/100,000 children/year in Western Countries. Immune-mediated mechanisms, particularly T cell-mediated, are thought to play a key pathogenic role. The genetic basis of the disease is still poorly understood. We tested the association between single nucleotide polymorphisms (SNPs) of four genes encoding Fc gamma receptors (FCGR2A, FCGR2B, FCGR3A, FCGR3B) and idiopathic nephrotic syndrome in a case-control study of paediatric patients. Children with idiopathic nephrotic syndrome (aged 1-16 years) were included. FCGR2A rs1801274 and FCGR3A rs396991 SNPs were genotyped using real-time PCR with the TaqMan method, while FCGR2B rs1050501 and FCGR3B NA1/NA2 were genotyped using Sanger sequencing. Fisher's exact test was used to explore genetic association. We enrolled 103 idiopathic nephrotic syndrome patients and 181 healthy controls. A significant association was found between idiopathic nephrotic syndrome and FCGR2A rs1801274 SNP (both with the T allele and the TT genotype, p value=0.0009, OR 1.81, 95% CI 1.27-2.59 and p value=0.0007, OR 2.39, 95% CI 1.44-3.99, respectively). No associations were found for the remaining SNPs. Fc gamma receptors might modulate response to rituximab; since 60 of the enrolled patients were treated with rituximab, we also tested the association between the studied SNPs and rituximab efficacy in this patient subgroup, but found only a weak association with FCGR2A CC genotype (p value=0.03). The FCGR2A rs1801274 SNP in the gene encoding the activating receptor CD32A confers susceptibility to idiopathic nephrotic syndrome. Copyright © 2017 European Federation of Immunological Societies. Published by Elsevier B.V. All rights reserved.

  14. The increasing incidence of initial steroid resistance in childhood nephrotic syndrome.

    Science.gov (United States)

    Banaszak, Beata; Banaszak, Paweł

    2012-06-01

    Recently, a number of reports have highlighted changes in the histopathology and response to corticosteroid treatment in childhood nephrotic syndrome; however, these involved ethnically mixed populations. For comparison, the purpose of our research was to search for changes in the characteristics of nephrotic syndrome in a homogeneous population of Caucasian children over two consecutive decades. Chart analysis was performed to identify children with new-onset nephrotic syndrome. The children were admitted to the Division of Pediatric Nephrology, Zabrze, during two periods: 1986-1995 (76 patients) and 1996-2005 (102 patients). Specifically, a comparison of clinical characteristics and morphology of nephrotic syndrome between the two groups was performed. Steroid resistance was defined as no remission within 8 weeks of corticosteroid treatment. Histopathology was available in 36.8% and 43.1% of patients respectively. There was a significant increase in primary steroid resistance in the latter decade: 15.8% vs 31.4% (P = 0.017). Changes in the histopathology did not reach the level of statistical significance: minimal change nephrotic syndrome 25% vs 9% (P = 0.095), mesangial proliferative glomerulonephritis 46.4% vs 61.3% (P = 0.21), focal segmental glomerulosclerosis 17.9% vs 20.4% (P = 0.78), membranoproliferative glomerulonephritis 7.1% vs 6.8% (P = 1.0), membranous glomerulonephritis 3.6% vs 0% (P = 0.39). Our results show the increasing incidence of primary steroid resistance in childhood nephrotic syndrome.

  15. Posterior reversible encephalopathy syndrome associated with nephrotic syndrome.

    Science.gov (United States)

    Zhang, Hong-Liang; Yang, Yi; Wu, Jiang

    2010-01-01

    Kabicek and colleagues described a case of nephritic-syndrome-associated posterior reversible encephalopathy syndrome (PRES) (Kabicek, et al. 2010). This adds to the accumulating evidence that PRES can be associated with disorders other than hypertension. However, we wonder how the authors would explain the neuroimaging findings unsuggestive of vasogenic oedema. PRES (also named reversible posterior leukoencephalopathy syndrome, RPLS) represents a clinicoradiological syndrome characterized by vasogenic oedema as revealed by apparent diffusion coefficient (ADC) map of diffusion-weighted imaging (DWI) (Bartynski, 2008). The pathogenesis of PRES has been suggested to be autoregulation failure and endothelial dysfunction (Sharma, et al.). ...

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

    Science.gov (United States)

    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. Copyright © 2016 by the American Academy of Pediatrics.

  17. 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.

  18. 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.

  19. Childhood idiopathic steroid resistant nephrotic syndrome in Southwestern Nigeria

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    Olowu Wasiu

    2010-01-01

    Full Text Available Clinical charts of 23 Nigerian children diagnosed with Idiopathic Steroid Resistant Nephrotic Syndrome (iSRNS between January 2001 and December 2007 were retrospectively re-viewed to determine their clinicopathologic characteristics and outcome. iSRNS (54.8% was pri-mary in 19 patients (83% and secondary in four (17%. The mean age at diagnosis was 8.3 ± 3.5 years (2.1-13 years. Histopathology revealed membranoproliferative glomerulonephritis (MPGN in 43.5%, focal and segmental glomerulosclerosis (FSGS in 39.1% and mesangial proliferative glo-merulonephritis in 8.7% of the patients while minimal change disease (MCD and membranous nephropathy accounted for 4.35% each. Routine treatment protocol comprised pulse intravenous (i.v. cylophosphamide infusion and i.v. dexamethasone ΁ lisinopril or spironolactone. Cumulative Com-plete Remission (CR rate was 57.12%. The overall median time to CR from start of steroid sparing agents in 12/21 treated patients was 4.5 weeks. CR was better achieved in MPGN than FSGS (P = 0.0186. Five patients had eight relapses with the overall median relapse-free duration being four months. Cumulative renal survival at 36 months was 41.8%. The median follow-up duration was eight months. Our study revealed that there was a high prevalence of iSRNS and preponderance of non-MCD lesions, with MPGN and FSGS being the major morphologic lesions. The outcome with steroid and cyclophosphamide-based treatment for iSRNS was further enhanced with addition of either lisinopril or spironolactone.

  20. 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 .

  1. Nivolumab-associated Nephrotic Syndrome in a Patient With Renal Cell Carcinoma: A Case Report

    NARCIS (Netherlands)

    Daanen, R.A.; Maas, R.J.H.; Koornstra, R.H.; Steenbergen, E.J.; Herpen, C.M.L. van; Willemsen, A.E.C.A.B.

    2017-01-01

    INTRODUCTION: Immune checkpoint inhibitors have taken an important place in the treatment of different types of malignancies. These drugs are known to have specific immune-mediated adverse events. We describe a case of severe nephrotic syndrome secondary to treatment with nivolumab in a patient with

  2. Thirty years old lady with nephrotic syndrome: a case of biopsy ...

    African Journals Online (AJOL)

    We describe a case of a 30 years old female patient who presented with nephrotic syndrome and impaired renal function which was diagnosed to have systemic lupus erythematosus (SLE) with lupus nephritis. This is the first biopsy proven lupus nephritis in Tanzania. SLE is common among females and is reported be more ...

  3. 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

  4. Levamisole in steroid-sensitive nephrotic syndrome of childhood: the lost paradise?

    NARCIS (Netherlands)

    Davin, J. C.; Merkus, M. P.

    2005-01-01

    Among the different drugs used for sparing steroids in steroid-sensitive nephrotic syndrome (SSNS) with frequent relapses and steroid dependency, levamisole is the least toxic and the least expensive. However, it is neither approved for this indication nor widely used in Europe. This may be

  5. 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. [=Anthonius; de Vries, P. J.

    2015-01-01

    The aim was to investigate the population pharmacokinetics of levamisole in children with steroid-sensitive nephrotic syndrome. Non-linear mixed effects modelling was performed on samples collected during a randomized controlled trial. Samples were collected from children who were receiving 2.5 mg

  6. Simplified quantification of urinary protein excretion in children with nephrotic syndrome

    International Nuclear Information System (INIS)

    Mustafa, G.; Khan, P.A.; Hussain, Z.; Iqbal, M.

    2007-01-01

    To assess the value of single voided random (spot) urinary protein to creatinine ratio in accurately predicting the 24-hour urinary protein excretion in Pakistani pediatric population with nephrotic syndrome. Fifty seven children between 1-18 years with nephrotic syndrome were included. Seventy pairs of spot urine (5 milliliter) and 24-hour urine were collected in different phases of their disease e.g. initial, induction and remission. The protein to creatinine ratio was determined in spot urine samples and total protein content in 24-hour urine samples. The correlation between the ratio and 24-hour urinary protein excreted was determined using Pearson's coefficient (r) linear regression analysis. The protein to creatinine ratio in a spot urine sample was significantly correlated with the 24-hour urinary protein. The correlation coefficient (least square method) was found to be significant (r=0.9444). A random (spot) urinary protein to creatinine ratio of greater than 2 correlated well with the massive proteinuria (i.e. nephrotic syndrome), between 2 to 0.2 indicated glomerulopathy while a ratio of less than 0.2 was suggestive of physiological values. The random spot urinary protein to creatinine ratio can reliably be used to assess the degree of proteinuria in children with nephrotic syndrome and can replace the 24-hour urinary protein excretion/collection. (author)

  7. Randomized Clinical Trial Design to Assess Abatacept in Resistant Nephrotic Syndrome

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    Howard Trachtman

    2018-01-01

    Conclusion: This study advances efforts to validate CD80 as a therapeutic target for treatment-resistant nephrotic syndrome, and implements a precision medicine-based approach to this serious kidney condition in which the selection of a therapeutic agent is guided by the underlying disease mechanism operating in individual patients.

  8. Pattern of steroid-resistant nephrotic syndrome in children and the ...

    African Journals Online (AJOL)

    Steroid-resistant nephrotic syndrome (SRNS) remains a challenge for paediatric nephrologists. e underlying histopathology usually affects the course of the disease and the response to treatment.[1] ere is still controversy over the role of renal biopsy in the management of children with. SRNS.[2] Studies by the International ...

  9. Pattern of steroid-resistant nephrotic syndrome in children and the ...

    African Journals Online (AJOL)

    Background. Steroid-resistant nephrotic syndrome (SRNS) is a common problem in paediatric nephrology practice. There is currently little information on the spectrum of histopathological lesions in children presenting with SRNS in India and other south-east Asian countries. Objective. To determine the histopathological ...

  10. 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.

  11. [Idiopathic Nephrotic Syndrome: recommendations of the Nephrology Branch of the Chilean Society of Pediatrics. Part One].

    Science.gov (United States)

    Hevia, Pilar; Nazal, Vilma; Rosati, María Pía; Quiroz, Lily; Alarcón, Claudia; Márquez, Sonia; Cuevas, Karen

    2015-01-01

    Idiopathic nephrotic syndrome is the most common glomerular disease in childhood, affecting 1 to 3 per 100,000 children under the age of 16. It most commonly occurs in ages between 2 and 10. Its cause is unknown and its histology corresponds to minimal change disease in 90% of cases, or focal segmental glomerulosclerosis. 80 to 90% of cases respond to steroids (steroid-sensitive nephrotic syndrome) with good prognosis and long-term preservation of renal function over time. 70% of patients with SSNS have one or more relapses in their evolution, and of these, 50% behave as frequent relapsing or steroid-dependent, a group that concentrate the risk of steroid toxicity. Patients with steroid-resistant nephrotic syndrome have a poor prognosis and 50% of them evolve to end-stage renal disease. The goal of therapy is to induce and maintain remission of the disease, reducing the risk secondary to proteinuria while minimizing the adverse effects of treatments, especially with prolonged use of corticosteroids. This paper is the result of the collaborative effort of the Nephrology Branch of the Chilean Society of Pediatrics with aims at helping pediatricians and pediatric nephrologists to treat pediatric SNI. In this first part, recommendations of steroid-sensitive nephrotic syndrome are discussed. Copyright © 2015 Sociedad Chilena de Pediatría. Publicado por Elsevier España, S.L.U. All rights reserved.

  12. Extending Prednisolone Treatment Does Not Reduce Relapses in Childhood Nephrotic Syndrome

    NARCIS (Netherlands)

    Teeninga, N.; Kist-van Holthe, J.E.; van Rijswijk, N.; de Mos, N.I.; Hop, W.C.J.; Wetzels, J.F.M.; van der Heijden, A.J.; Nauta, J.

    2013-01-01

    Prolonged prednisolone treatment for the initial episode of childhood nephrotic syndrome may reduce relapse rate, butwhether this results fromthe increased duration of treatment or a higher cumulative dose remains unclear.We conducted a randomized, double-blind, placebo-controlled trial in 69

  13. Fc-receptor function in minimal change nephrotic syndrome of childhood

    NARCIS (Netherlands)

    Davin, J. C.; Foidart, J. B.; Mahieu, P. R.

    1983-01-01

    This study was undertaken to establish whether the Fc-receptor function of circulating monocytes (CM) and/or of splenic macrophages (SM) is modified during the course of minimal change nephrotic syndrome (MCNS) of childhood. The Fc-receptor function of SM was ascertained by measuring the spleen to

  14. The efficacy and safety of rituximab in treating childhood refractory nephrotic syndrome: a meta-analysis.

    Science.gov (United States)

    Zhao, Zhihong; Liao, Guixiang; Li, Yongqiang; Zhou, Shulu; Zou, Hequn

    2015-02-03

    Rituximab is considered to be a promising drug for treating childhood refractory nephrotic syndrome. However, the efficacy and safety of rituximab in treating childhood refractory nephrotic syndrome remain inconclusive. This meta-analysis aimed to investigate the efficacy and safety of rituximab treatment compared with other immunosuppressive agents in children with refractory nephrotic syndrome. Three randomized controlled trials and two comparative control studies were included in our analysis. The included studies were of moderately high quality. Compared with other immunotherapies, rituximab therapy significantly improved relapse-free survival (hazard ratio = 0.49, 95% confidence interval [CI], 0.26-0.92, P = 0.03). Rituximab also achieved a higher rate of complete remission (risk ratio,1.62; 95% CI, 0.92 to 2.84, P = 0.09) and reduced the occurrence of proteinuria (mean difference = -0.25, 95% CI = -0.29 to -0.21, P childhood refractory nephrotic syndrome; however, the long-term effects and cost-effectiveness of rituximab treatment were not fully assessed, and there were limited studies that evaluated the clinical benefits of a concurrent infusion of rituximab plus a steroid compared with an infusion of rituximab only. Additional studies are required to address these issues.

  15. Pathogenesis of childhood idiopathic nephrotic syndrome: a paradigm shift from T-cells to podocytes.

    Science.gov (United States)

    Kaneko, Kazunari; Tsuji, Shoji; Kimata, Takahisa; Kitao, Tetsuya; Yamanouchi, Sohsaku; Kato, Shogo

    2015-02-01

    Nephrotic syndrome is the most common cause of kidney disease in children, but its pathogenesis remains unclear. This article reviews the novel aspects of the mechanisms underlying massive proteinuria in minimal-change disease, which is the most common form of childhood nephrotic syndrome. This article integrates the findings of a PubMed database search for English language articles published in the past 40 years (from September 1974 to February 2014) using the key words "pathogenesis", "minimal change nephrotic syndrome" or "idiopathic nephrotic syndrome". Unknown humoral factors associated with T-cell dysfunction have been thought to play an important role in the pathogenesis of minimal-change disease. However, recent findings are changing this paradigm, i.e., visceral glomerular epithelial cells (podocytes) may be involved via expression of molecules such as CD80 and angiopoietin-like 4. Recent evidence suggests that minimal-change disease results from interactions between humoral factors and dysfunctional podocytes. In addition to immunosuppressant drugs that target lymphocytes, a biological agent such as an antibody against the abnormal molecule(s) expressed by podocytes may provide novel drug treatment for minimal-change disease.

  16. Cyclosporine A and steroid therapy in childhood steroid-resistant nephrotic syndrome

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    Gargah Tahar

    2010-08-01

    Full Text Available Gargah Tahar, Lakhoua M RachidDepartment of Pediatric Nephrology, Charles Nicolle Hospital, Tunis, TunisiaBackground: In children, idiopathic nephrotic syndrome (INS is primarily treated using corticosteroids. When remission is not achieved, the coadministration of potent immunosuppressant therapy becomes imperative. Cyclosporine A (CsA is reportedly associated with a higher incidence of remission in comparison with other immunosuppressive agents.Methods: The present study investigated the response of combination therapy using CsA and prednisolone in 30 Tunisian children with idiopathic steroid-resistant nephrotic syndrome (ISRNS. Renal histopathology was compatible with focal segmental glomerular sclerosis (FSGS in 15 children, minimal change disease (MCD in nine children, and diffuses mesangiale proliferation (DMP in six children.Results: The therapy protocol produced a complete remission of proteinuria in 15 patients (50% and a partial remission in nine patients (30%. Six patients (20% showed no response to therapy. Progression to end stage renal disease occurred in five CsA-resistant children and in four CsA-responsive patients. CsA-related nephrotoxicity was detected by renal biopsy in one patient.Conclusions: CsA remains the primary cytotoxic treatment for childhood steroid-resistant nephrotic syndrome. Its use in combination with corticosteroids provides optimum efficiency without high risk of nephrotoxicity.Keywords: children, corticosteroids, cyclosporine A, nephrotic syndrome

  17. Extending prednisolone treatment does not reduce relapses in childhood nephrotic syndrome

    NARCIS (Netherlands)

    N. Teeninga (Nynke); J.E. Kist-Van Holthe (Joana); N. Rijswijk (Nienske); N.I. de Mos (Nienke); J.F.M. Wetzels (Jack); A.J. van der Heijden (Bert); J. Nauta (Jeroen); W.C.J. Hop (Wim)

    2013-01-01

    textabstractProlonged prednisolone treatment for the initial episode of childhood nephrotic syndrome may reduce relapse rate, butwhether this results fromthe increased duration of treatment or a higher cumulative dose remains unclear.We conducted a randomized, double-blind, placebo-controlled trial

  18. Examine of Thyroid Function in Pediatric Nephrotic Syndrome; Tehran-Iran

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    Niloofar Hajizadeh

    2015-03-01

    Full Text Available Introduction In children with nephrotic syndrome, it is probable to determine a hypothyroid state because of thyroxine (T4, tri-iodothyronine (T3 and thyroid-binding globulin loss in presence of proteinuria. Objectives: To examine thyroid function in pediatric cases of nephrotic syndrome. Methods: In a cross-sectional study, from march 2010 to march 2012, thyroid function tests were performed in 104 patients referred to the nephrology department of children’s medical center, because of nephrotic  syndrome. Collected data analyzed with SPSS Statistics 17 and pResults: Sixty one cases identified as hypothyroid patients and were treated with supplementary levothyroxine. There were 41 (67.2% males and 20 (32.8% females with the mean age of 3.72±3.35 years. Our patients showed lowered T3 (68.3% and T4 (64.4% in comparison with normal values. Median TSH (Thyroid-stimulating hormone was 11.65±6.71 Micu/ml and 2.82±0.82 in the hypothyroid and euthyroid patients respectively. In all, TSH was negatively correlated with the total urinary protein content . Conclusions: According to this study, the occurrence of hypothyroidism in any child with nephrotic syndrome needs to be mentioned. It is proposed to systematically search hypothyroidism by measuring TSH and free T4 in these patients particularly when proteinuria is prolonged.

  19. Extending prednisolone treatment does not reduce relapses in childhood nephrotic syndrome

    NARCIS (Netherlands)

    Teeninga, N.; Kist-van Holthe, J.E.; Rijswijk, N. van; Mos, N.I. de; Hop, W.C.J.; Wetzels, J.F.M.; Heijden, A.J. van der; Nauta, J.

    2013-01-01

    Prolonged prednisolone treatment for the initial episode of childhood nephrotic syndrome may reduce relapse rate, but whether this results from the increased duration of treatment or a higher cumulative dose remains unclear. We conducted a randomized, double-blind, placebo-controlled trial in 69

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

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    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.

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

    NARCIS (Netherlands)

    Clement, L.C.; Mace, C.; Avila-Casado, C.; Joles, J.A.; Kersten, A.H.; Chugh, S.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

  2. The Canadian Childhood Nephrotic Syndrome (CHILDNEPH Project: Overview of Design and Methods

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    Susan Samuel

    2014-07-01

    Full Text Available Background: Nephrotic syndrome is a commonly acquired kidney disease in children that causes significant morbidity due to recurrent episodes of heavy proteinuria. The management of childhood nephrotic syndrome is known to be highly variable among physicians and care centres. Objectives: The primary objective of the study is to determine centre-, physician-, and patient-level characteristics associated with steroid exposure and length of steroid treatment. We will also determine the association of dose and duration of steroid treatment and time to first relapse as a secondary aim. An embedded qualitative study utilizing focus groups with health care providers will enrich the quantitative results by providing an understanding of the attitudes, beliefs and local contextual factors driving variation in care. Design: Mixed-methods study; prospective observational cohort (quantitative component, with additional semi-structured focus groups of healthcare professionals (qualitative component. Setting: National study, comprised of all 13 Canadian pediatric nephrology clinics. Patients: 400 patients under 18 years of age to be recruited over 2.5 years. Measurements: Steroid doses for all episodes (first presentation, first and subsequent relapses tracked over course of the study. Physician and centre-level characteristics catalogued, with reasons for treatment preferences documented during focus groups. Methods: All patients tracked prospectively over the course of the study, with data comprising a prospective registry. One focus group at each site to enrich understanding of variation in care. Limitations: Contamination of treatment protocols between physicians may occur as a result of concurrent focus groups. Conclusions: Quantitative and qualitative results will be integrated at end of study and will collectively inform strategies for the development and implementation of standardized evidence-based protocols across centres.

  3. The Canadian Childhood Nephrotic Syndrome (CHILDNEPH) Project: overview of design and methods.

    Science.gov (United States)

    Samuel, Susan; Scott, Shannon; Morgan, Catherine; Dart, Allison; Mammen, Cherry; Parekh, Rulan; Nettel-Aguirre, Alberto; Eddy, Allison; Flynn, Rachel; Pinsk, Maury; Wade, Andrew; Arora, Steven; Benoit, Geneviève; Bitzan, Martin; Erickson, Robin; Feber, Janusz; Filler, Guido; Geier, Pavel; Girardin, Colette; Grisaru, Silviu; Tee, James; Kemp, Kyle; Zappitelli, Michael

    2014-01-01

    Nephrotic syndrome is a commonly acquired kidney disease in children that causes significant morbidity due to recurrent episodes of heavy proteinuria. The management of childhood nephrotic syndrome is known to be highly variable among physicians and care centres. The primary objective of the study is to determine centre-, physician-, and patient-level characteristics associated with steroid exposure and length of steroid treatment. We will also determine the association of dose and duration of steroid treatment and time to first relapse as a secondary aim. An embedded qualitative study utilizing focus groups with health care providers will enrich the quantitative results by providing an understanding of the attitudes, beliefs and local contextual factors driving variation in care. Mixed-methods study; prospective observational cohort (quantitative component), with additional semi-structured focus groups of healthcare professionals (qualitative component). National study, comprised of all 13 Canadian pediatric nephrology clinics. 400 patients under 18 years of age to be recruited over 2.5 years. Steroid doses for all episodes (first presentation, first and subsequent relapses) tracked over course of the study. Physician and centre-level characteristics catalogued, with reasons for treatment preferences documented during focus groups. All patients tracked prospectively over the course of the study, with data comprising a prospective registry. One focus group at each site to enrich understanding of variation in care. Contamination of treatment protocols between physicians may occur as a result of concurrent focus groups. Quantitative and qualitative results will be integrated at end of study and will collectively inform strategies for the development and implementation of standardized evidence-based protocols across centres.

  4. Long-term results of children diagnosed with idiopathic nephrotic syndrome; single center experience.

    Science.gov (United States)

    Hacıhamdioğlu, Duygu Övünç; Kalman, Süleyman; Gök, Faysal

    2015-03-01

    The aim of this study was to determine the long-term results of children followed up with a diagnosis of nephrotic syndrome in a single center. The medical data of 33 patients aged between 6 months and 10 years who were diagnosed with idiopathic nephrotic syndrome in our center between January 2000 and December 2012 and followed up for a period of 2-12 years were reviewed (Gulhane Military Medical Academy Ethics committee, 07.11.2012/10). The mean age of disease onset was 3.2±2.04 years (range: 0.5-10 years) and the mean follow-up period was 6±3.4 years (range: 2-12 years). Thirteen (39.4%) of the study group (or the patients) were female and 20 (60.6%) were male. Twenty seven (1.8%) of the patients were sensitive to steroid and 6 (18.1%) were resistant to steroid. Four (12.1%) of the steroid-resistant patients had steroid-dependent nephrotic syndrome, 5 (15.2%) had frequently relapsing nephrotic syndrome and 18 (54.5%) had rarely relapsing nephrotic syndrome. Histopathological diagnoses of six patients who underwent biopsy because of resistance to steroid were as follows: focal segmental glomerulosclerosis (n=3), C1q nephropathy (n=1), diffuse mesangial proliferation (n=1) and membraneous nephropathy (n=1). Fifteen (45.5%) patients entered into full remission and 2 (6%) patients developed chronic renal failure. Treatment complications including decreased bone mineral density in three patients (9%), short stature in 2 patients (6%) and cataract in 2 patients (6%) developed. Children with nephrotic syndrome carry a risk in terms of short stature, osteoporosis, cataract and renal failure in the long-term follow-up. It was observed that our rates of response to steroid were similar to the literature and the most common histopathological diagnosis was focal segmental glomerulosclerosis in our patients who underwent biopsy because of resistance to steroid. It was thought that multi-center studies should be conducted to demonstrate regional or national differences

  5. Modeling Monogenic Human Nephrotic Syndrome in the Drosophila Garland Cell Nephrocyte.

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    Hermle, Tobias; Braun, Daniela A; Helmstädter, Martin; Huber, Tobias B; Hildebrandt, Friedhelm

    2017-05-01

    Steroid-resistant nephrotic syndrome is characterized by podocyte dysfunction. Drosophila garland cell nephrocytes are podocyte-like cells and thus provide a potential in vivo model in which to study the pathogenesis of nephrotic syndrome. However, relevant pathomechanisms of nephrotic syndrome have not been studied in nephrocytes. Here, we discovered that two Drosophila slit diaphragm proteins, orthologs of the human genes encoding nephrin and nephrin-like protein 1, colocalize within a fingerprint-like staining pattern that correlates with ultrastructural morphology. Using RNAi and conditional CRISPR/Cas9 in nephrocytes, we found this pattern depends on the expression of both orthologs. Tracer endocytosis by nephrocytes required Cubilin and reflected size selectivity analogous to that of glomerular function. Using RNAi and tracer endocytosis as a functional read-out, we screened Drosophila orthologs of human monogenic causes of nephrotic syndrome and observed conservation of the central pathogenetic alterations. We focused on the coenzyme Q 10 (CoQ 10 ) biosynthesis gene Coq2 , the silencing of which disrupted slit diaphragm morphology. Restoration of CoQ 10 synthesis by vanillic acid partially rescued the phenotypic and functional alterations induced by Coq2 -RNAi. Notably, Coq2 colocalized with mitochondria, and Coq2 silencing increased the formation of reactive oxygen species (ROS). Silencing of ND75 , a subunit of the mitochondrial respiratory chain that controls ROS formation independently of CoQ 10 , phenocopied the effect of Coq2 -RNAi. Moreover, the ROS scavenger glutathione partially rescued the effects of Coq2 -RNAi. In conclusion, Drosophila garland cell nephrocytes provide a model with which to study the pathogenesis of nephrotic syndrome, and ROS formation may be a pathomechanism of COQ2 -nephropathy. Copyright © 2017 by the American Society of Nephrology.

  6. Second-line immunosuppressive treatment of childhood nephrotic syndrome: a single-center experience.

    Science.gov (United States)

    Kim, J; Patnaik, N; Chorny, N; Frank, R; Infante, L; Sethna, C

    2014-01-01

    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. 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. 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. 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.

  7. 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.

  8. Ethnic Differences in Incidence and Outcomes of Childhood Nephrotic Syndrome.

    Science.gov (United States)

    Banh, Tonny H M; Hussain-Shamsy, Neesha; Patel, Viral; Vasilevska-Ristovska, Jovanka; Borges, Karlota; Sibbald, Cathryn; Lipszyc, Deborah; Brooke, Josefina; Geary, Denis; Langlois, Valerie; Reddon, Michele; Pearl, Rachel; Levin, Leo; Piekut, Monica; Licht, Christoph P B; Radhakrishnan, Seetha; Aitken-Menezes, Kimberly; Harvey, Elizabeth; Hebert, Diane; Piscione, Tino D; Parekh, Rulan S

    2016-10-07

    Ethnic differences in outcomes among children with nephrotic syndrome are unknown. We conducted a longitudinal study at a single regional pediatric center comparing ethnic differences in incidence from 2001 to 2011 census data and longitudinal outcomes, including relapse rates, time to first relapse, frequently relapsing disease, and use of cyclophosphamide. Among 711 children, 24% were European, 33% were South Asian, 10% were East/Southeast Asian, and 33% were of other origins. Over 10 years, the overall incidence increased from 1.99/100,000 to 4.71/100,000 among children ages 1-18 years old. In 2011, South Asians had a higher incidence rate ratio of 6.61 (95% confidence interval, 3.16 to 15.1) compared with Europeans. East/Southeast Asians had a similar incidence rate ratio (0.76; 95% confidence interval, 0.13 to 2.94) to Europeans. We determined outcomes in 455 children from the three largest ethnic groups with steroid-sensitive disease over a median of 4 years. South Asian and East/Southeast Asian children had significantly lower odds of frequently relapsing disease at 12 months (South Asian: adjusted odds ratio; 0.55; 95% confidence interval, 0.39 to 0.77; East/Southeast Asian: adjusted odds ratio; 0.42; 95% confidence interval, 0.34 to 0.51), fewer subsequent relapses (South Asian: adjusted odds ratio; 0.64; 95% confidence interval, 0.50 to 0.81; East/Southeast Asian: adjusted odds ratio; 0.47; 95% confidence interval, 0.24 to 0.91), lower risk of a first relapse (South Asian: adjusted hazard ratio, 0.74; 95% confidence interval, 0.67 to 0.83; East/Southeast Asian: adjusted hazard ratio, 0.65; 95% CI, 0.63 to 0.68), and lower use of cyclophosphamide (South Asian: adjusted hazard ratio, 0.82; 95% confidence interval, 0.53 to 1.28; East/Southeast Asian: adjusted hazard ratio, 0.54; 95% confidence interval, 0.41 to 0.71) compared with European children. Despite the higher incidence among South Asians, South and East/Southeast Asian children have significantly

  9. Childhood nephrotic syndrome in tropical Africa: then and now.

    Science.gov (United States)

    Olowu, Wasiu A; Ademola, Adebowale; Ajite, Adebukola B; Saad, Yauba M

    2017-11-01

    This descriptive and comparative review examines the changing epidemiology, treatment, renal and patient outcome of childhood nephrotic syndrome (NS) in tropical Africa (TpAfr). In the 1960s to 1980s, corticosteroid-resistant non-minimal change disease (nMCD) including quartan malaria nephropathy (QMN) was the dominant renal histopathology type. The overall incidence of NS was 0.35-1.34% of hospital admissions. Median age at onset of NS ranged between 4.0 and 12.0 years while the mean (SD) age range was 5.8 (3.8) to 10.3 (4.8) years across studies. The male: female ratio was 1.6:1.0. The overall mean (SD) incidence of idiopathic minimal change disease (MCD) [21.6 (18.6%)] compared with idiopathic nMCD [59.1 (25.7%)] demonstrates significant dominance of the latter (p = 0.0001). Post-1989, the following mean (SD) incidences of histopathological types were: MCD 20.4 (17.7%), focal segmental glomerulosclerosis 39.0 (26.3%), membranoproliferative glomerulonephritis 25.4 (16.8%), proliferative glomerulonephritis 16.7 (27.0%) and membranous nephropathy 7.4 (4.5%). While the mean (SD) proportion of steroid resistance (SR) [73.5 (19.2%)] was significantly greater than the mean complete remission (CR) [26.5 (19.2%)] during 1960-1989 (p=0.005), mean (SD) SR [27.4 (25.3%)] was significantly lower than mean (SD) CR [66.1 (28.0%)] post-1989 (p < 0.001). Unlike QMN, hepatitis B virus, HIV infection, sickle cell disease and systemic lupus erythematosus are now increasingly being associated with NS in TpAfr. Mean (SD) renal survival post-1989 was 58.3 (37.0%) while all-cause mortality was 9.8%. Children with NS now survive better than before, reflecting improved access to healthcare and transition to a clinical pattern favouring idiopathic NS and increased sensitivity to corticosteroids.

  10. A Pilot Study of IL2 in Drug-Resistant Idiopathic Nephrotic Syndrome.

    Directory of Open Access Journals (Sweden)

    Alice Bonanni

    Full Text Available Tregs infusion reverts proteinuria and reduces renal lesions in most animal models of nephrotic syndrome (i.e. Buffalo/Mna, Adriamycin, Promycin, LPS. IL2 up-regulates Tregs and may be an alternative to cell-therapy in this setting. To evaluate a potential role of IL2 as Tregs inducer and proteinuria lowering agent in human nephrotic syndrome we treated 5 nephrotic patients with 6 monthly cycles of low-dose IL2 (1x106 U/m2 first month, 1.5x106 U/m2 following months. The study cohort consisted of 5 children (all boys, 11–17 years resistant to all the available treatments (i.e. steroids, calcineurin inhibitors, mycophenolate, Rituximab. Participants had Focal Segmental Glomerulosclerosis (3 cases or Minimal Change Nephropathy (2 cases. IL2 was safe in all but one patient who had an acute asthma attack after the first IL2 dose and did not receive further doses. Circulating Tregs were stably increased (>10% during the whole study period in 2 cases while were only partially modified in the other two children who started with very low levels and partially responded to single IL2 Proteinuria and renal function were not modified by IL2 at any phase of the study. We concluded that low-dose IL2 given in monthly pulses is safe and modifies the levels of circulating Tregs. This drug may not be able to lower proteinuria or affect renal function in children with idiopathic nephrotic syndrome. We were unable to reproduce in humans the effects of IL2 described in rats and mice reducing de facto the interest on this drug in nephrotic syndrome.ClinicalTrials.gov NCT02455908.

  11. Experience with second line drugs in frequently relapsing and steroid dependent childhood nephrotic syndrome in a large Saudi center

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    Khalid Alsaran, M.D.

    2017-06-01

    Conclusion: All the second line drugs in our study were equally effective. However, we recommend that the initial treatment of FR/SD nephrotic syndrome should be chosen with the least toxic yet equally efficacious drug Levamisole.

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

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    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.

  13. Rituximab for childhood-onset, complicated, frequently relapsing nephrotic syndrome or steroid-dependent nephrotic syndrome: a multicentre, double-blind, randomised, placebo-controlled trial.

    Science.gov (United States)

    Iijima, Kazumoto; Sako, Mayumi; Nozu, Kandai; Mori, Rintaro; Tuchida, Nao; Kamei, Koichi; Miura, Kenichiro; Aya, Kunihiko; Nakanishi, Koichi; Ohtomo, Yoshiyuki; Takahashi, Shori; Tanaka, Ryojiro; Kaito, Hiroshi; Nakamura, Hidefumi; Ishikura, Kenji; Ito, Shuichi; Ohashi, Yasuo

    2014-10-04

    Rituximab could be an effective treatment for childhood-onset, complicated, frequently relapsing nephrotic syndrome (FRNS) and steroid-dependent nephrotic syndrome (SDNS). We investigated the efficacy and safety of rituximab in patients with high disease activity. We did a multicentre, double-blind, randomised, placebo-controlled trial at nine centres in Japan. We screened patients aged 2 years or older experiencing a relapse of FRNS or SDNS, which had originally been diagnosed as nephrotic syndrome when aged 1-18 years. Patients with complicated FRNS or SDNS who met all other criteria were eligible for inclusion after remission of the relapse at screening. We used a computer-generated sequence to randomly assign patients (1:1) to receive rituximab (375 mg/m(2)) or placebo once weekly for 4 weeks, with age, institution, treatment history, and the intervals between the previous three relapses as adjustment factors. Patients, guardians, caregivers, physicians, and individuals assessing outcomes were masked to assignments. All patients received standard steroid treatment for the relapse at screening and stopped taking immunosuppressive agents by 169 days after randomisation. Patients were followed up for 1 year. The primary endpoint was the relapse-free period. Safety endpoints were frequency and severity of adverse events. Patients who received their assigned intervention were included in analyses. This trial is registered with the University Hospital Medical Information Network clinical trials registry, number UMIN000001405. Patients were centrally registered between Nov 13, 2008, and May 19, 2010. Of 52 patients who underwent randomisation, 48 received the assigned intervention (24 were given rituximab and 24 placebo). The median relapse-free period was significantly longer in the rituximab group (267 days, 95% CI 223-374) than in the placebo group (101 days, 70-155; hazard ratio: 0·27, 0·14-0·53; pchildhood-onset, complicated FRNS and SDNS. Japanese Ministry of

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

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    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.

  15. Coexistence of cellulitis and primary peritonitis in a pediatric patient with nephrotic syndrome: A case report

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    Luisa Lagos

    2017-05-01

    Full Text Available Nephrotic syndrome is a chronic disease that increases the risk of skin, respiratory and urinary tract infection, while also increasing the chance for other diseases, like peritonitis and meningitis. A four year old patient with a history of nephrotic syndrome was admitted to emergency room (ER with the following symptoms: abdominal pain, fever, diarrhea and vomiting, associated to abdominal wall erythema, abdominal distension and peritoneal signs. In order to make a differential diagnosis of the infection, peritoneal fluid was extracted and, according to the characteristics found, treatment with broad-spectrum antibiotics was started. Cases in which different infections like pneumonia, abdominal wall peritonitis and cellulitis occur simultaneously have been reported rarely. Early diagnosis and dismissal of other causes of acute abdominal pain, as well as early introduction of antibiotics are fundamental in the treatment of these kind of infections.

  16. Platelet function in nephrotic syndrome patients at the Kenyatta National Hospital.

    Science.gov (United States)

    Kayima, J K; Otieno, L S; McLigeyo, S O; Kyobe, J

    1990-02-01

    Platelet function was assessed in 40 patients with nephrotic syndrome but without renal failure in order to establish whether or not there is any haemostatic disorder lending to hypercoagulable state. The findings were compared with those from 40 normal controls. There was no clinical evidence of thromboembolic phenomena in the patients. Values for the mean platelet counts and clot retraction were similar (P less than 0.05), whereas significant decrease of platelet adhesiveness (P less than 0.001) as well as prolonged platelet aggregation time (P less than 0.001) were noted. This is different from some reports in literature which have frequently reported enhanced platelet function. There may be a difference in the way platelets metabolise arachidonic acid to potent aggregating agents, in the African patients as compared to patients studied elsewhere. The hypercoagulable state in our nephrotic syndrome may be explained by alterations in other haemostatic parameters rather than in platelet function.

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

    Science.gov (United States)

    Sevillano, Ángel M; Hernandez, Eduardo; Gonzalez, Esther; Mateo, Isabel; Gutierrez, Eduardo; Morales, Enrique; Praga, Manuel

    2016-01-01

    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. Copyright © 2015 Sociedad Española de Nefrología. Published by Elsevier España, S.L.U. All rights reserved.

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

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    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.

  19. DEVELOPMENT OF PURULENT MENINGITIS IN A CHILD WITH NEPHROTIC SYNDROME WHILE RECEIVING CEPHTRIAXONE PLUS AMIKACIN

    OpenAIRE

    Bilici, Meki; Gürkan, Fuat; Ece, Aydın

    2015-01-01

    Malatya State Hospital1, Malatya, Dicle University Medical School, Department of Pediatrics2, Diyarbakır, Turkey A child with nephrotic syndrome (NS) presented with peritonitis caused by E. coli susceptible to both cephtriaxone and amikacin. Purulent meningitis developed in accompanying with clinical and laboratory findings at the 5th day of treatment. Pneumococcal antigen was detected in cerebro-spinal fluid and meningitis was treated with meropenem. Development of pneumococal meningit...

  20. Randomized Clinical Trial Design to Assess Abatacept in Resistant Nephrotic Syndrome

    OpenAIRE

    Howard Trachtman; Debbie S. Gipson; Michael Somers; Cathie Spino; Sharon Adler; Lawrence Holzman; Jeffrey B. Kopp; John Sedor; Sandra Overfield; Ayanbola Elegbe; Michael Maldonado; Anna Greka

    2018-01-01

    Introduction Treatment-resistant nephrotic syndrome is a rare form of glomerular disease that occurs in children and adults. No Food and Drug Administration−approved treatments consistently achieve remission of proteinuria and preservation of kidney function. CD80 (B7-1) can be expressed on injured podocytes, and administration of abatacept (modified CTLA4-Ig based on a natural ligand to CD80) has been associated with sustained normalization of urinary protein excretion and maintenance of glo...

  1. Long-term outcomes in idiopathic nephrotic syndrome: from childhood to adulthood.

    Science.gov (United States)

    Skrzypczyk, Piotr; Panczyk-Tomaszewska, Malgorzata; Roszkowska-Blaim, Maria; Wawer, Zofia; Bienias, Beata; Zajgzkowska, Malgorzata; Kilis-Pstrusinska, Katarzyna; Jakubowska, Anna; Szczepaniak, Marta; Pawlak-Bratkowska, Monika; Tkaczyk, Marcin

    2014-03-01

    The aim of the study was to assess idiopathic nephrotic syndrome (INS) relapse rate, co-morbidities, and social status of adults diagnosed with INS in childhood. A written questionnaire was sent to 118 adults treated for INS in childhood. In 61 (51.7%) responders (aged 26.0 ± 6.2 years, range 18 - 51.5 years), we used available medical records to evaluate age at the onset of INS, number of INS relapses below 18 years of age, response to corticosteroids (CS), renal biopsy findings, and immunosuppressive treatment as well as questionnaire to evaluate the number and treatment of INS relapses above 18 years of age, co-morbidities, age at menarche, marital status, offspring, educational status, and occupation. In the group of 61 responders, median age at the onset of INS was 3 (range 1.3 - 14.0) years, median number of INS relapses at nephrotic syndrome (SSNS) was diagnosed in 37 (60.7%) patients, steroid-dependent nephrotic syndrome SDNS in 18 (29.5%) patients, and steroid-resistant nephrotic syndrome (SRNS) in 6 (9.8%) patients. Mesangial proliferation was the most common pattern in renal biopsy (35.7%). All patients received CS, 15 were treated with methylprednisolone pulses, 13 with cyclophosphamide, 11 with chlorambucil, 2 with cyclosporine, and 21 with levamisole. All patients achieved remission and had normal renal function at the age of 18. In adulthood, INS relapsed in 10 (16.4%) patients, including 5 (13.5%) patients with SSNS, 4 (22.2%) with SDNS, and 1 (16.7%) with SRNS (p = 0.72). Median number of relapses was 2 (range 1 - 11). Patients with relapses at > 18 years of age had more (p childhood is a risk factor for recurrences in adulthood. 2. INS relapses in childhood do not preclude active professional life in adulthood.

  2. Infusion reactions associated with rituximab treatment for childhood-onset complicated nephrotic syndrome.

    Science.gov (United States)

    Kamei, Koichi; Ogura, Masao; Sato, Mai; Ito, Shuichi; Ishikura, Kenji

    2018-02-09

    Infusion reaction (IR) is defined as an adverse event within 24 h after monoclonal antibody infusion. In non-Hodgkin lymphoma, IR incidence following rituximab treatment is high (77-80%), but there are no data in complicated nephrotic syndrome. Records of rituximab infusions in patients with complicated nephrotic syndrome between February 2006 and December 2014 at the National Center for Child Health and Development were reviewed. Rituximab was administered at doses of 375 mg/m 2 . The severity of IR was evaluated using the Common Terminology Criteria for Adverse Events ver. 4.0. For 309 rituximab infusions in 159 patients (male, 110; median age, 12 years), IR was observed in 165 infusions (53.4%). Respiratory symptoms were most common (66% of all events). Ninety-five percent of the IR was observed within 3 h after rituximab infusion initiation. Sixty-eight percent of the events were classified as grade 1 and others classified as grade 2. Only 18% required medical intervention. CD20 cell count in patients with IR was significantly higher than in patients without IR. Incidence of IR was similar in subsequent rituximab treatment after B-cell recovery. Patients who experienced IR at first rituximab treatment were more likely to experience recurrent IR with subsequent treatments compared to those not having IR at initial treatment (odds ratio 3.64; p nephrotic syndrome, respiratory symptoms were the major type of IR, mostly observed within 3 h of infusion. Incidence of IR was lower and its severity milder in patients with complicated nephrotic syndrome than those with lymphoma.

  3. Mesangioproliferative glomerulonephritis in a patient with Kimura′s disease presenting as Nephrotic syndrome

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    Surendra Singh Rathore

    2015-01-01

    Full Text Available Kimura′s disease is a rare chronic eosinophilic inflammatory disorder of unknown etiology. Majority of cases have been reported from South East Asia, while sporadic occurrences have been reported worldwide, including the Indian subcontinent. Nephrotic syndrome may be the presenting manifestation of Kimura′s disease, and a variety of renal lesions are observed histologically in such patients. We herein describe a case of steroid-responsive mesangioproliferative glomerulonephritis related to kimura′s disease.

  4. Setting New Directions for Research in Childhood Nephrotic Syndrome: Results From a National Workshop.

    Science.gov (United States)

    Samuel, Susan M; Takano, Tomoko; Scott, Shannon; Benoit, Genevieve; Bitzan, Martin; Mammen, Cherry; Ryan, Laurel; Morgan, Catherine

    2017-01-01

    We report on the proceedings of a national workshop held in Canada with the aims to identify priorities for research in childhood nephrotic syndrome and to develop a national strategy to address these priorities. A diverse group of participants attended the meeting, including patients, family members, researchers, and health care providers. We used small group discussions to explore priorities as perceived by patients and families and by health care providers and researchers. Research evaluating glucocorticoid minimization or glucocorticoid-sparing regimens was a consistent theme in the patient and family discussion group. Families also indicated the need for precise prognostic information at diagnosis, more information to help them choose the best available therapy, and more resources for disease management. Health care providers emphasized the importance of better disease characterization including genotyping and phenotyping patients, better understanding the pathogenesis, and the need of providing targeted therapy and precise prognostic information. These priorities will inform the development and future directions of the Canadian Childhood Nephrotic Syndrome (CHILDNEPH) project, a national research initiative to improve care and outcomes of patients with childhood onset nephrotic syndrome.

  5. Management of steroid resistant nephrotic syndrome in children with cyclosporine - a tertiary care centre experience

    International Nuclear Information System (INIS)

    Shah, S.S.H.; Akhtar, N.; Sunbleen, F.

    2015-01-01

    Objective: To observe the response and adverse effects of cyclosporine in combination with oral steroids for management of idiopathic steroid resistant nephrotic syndrome in pediatric patients. Methodology: It was an observational study conducted at Children Hospital, Lahore, Pakistan from March 2014 to June 2015. Forty normotensive patients of idiopathic steroid resistant nephrotic syndrome between one and twelve years of age with normal renal function were included in the study. Patients were prescribed cyclosporine with prednisolone and were followed to see the response and adverse effects of drugs. Results: Out of 40 patients, 20(50%) were males and 20(50%) females. Mesangioproliferative glomerulonephritis was found in 27(67.5%) patients followed by Focal segmental glomerulosclerosis in 9(22.5%) patients. Complete response was observed in 32(80%) children while partial response in 8(20%) patients at the end of six months. The most common adverse effects were cushingoid features seen in 26(65%) and cyclosporine related hypertrichosis in 34(85%). Conclusion: Management of idiopathic steroid resistant nephrotic syndrome in children with a combination of cyclosporine and prednisolone provided good results as response to treatment was seen in 80% patients. (author)

  6. Setting New Directions for Research in Childhood Nephrotic Syndrome: Results From a National Workshop

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    Susan M. Samuel

    2017-04-01

    Full Text Available Background: We report on the proceedings of a national workshop held in Canada with the aims to identify priorities for research in childhood nephrotic syndrome and to develop a national strategy to address these priorities. Methods: A diverse group of participants attended the meeting, including patients, family members, researchers, and health care providers. We used small group discussions to explore priorities as perceived by patients and families and by health care providers and researchers. Results: Research evaluating glucocorticoid minimization or glucocorticoid-sparing regimens was a consistent theme in the patient and family discussion group. Families also indicated the need for precise prognostic information at diagnosis, more information to help them choose the best available therapy, and more resources for disease management. Health care providers emphasized the importance of better disease characterization including genotyping and phenotyping patients, better understanding the pathogenesis, and the need of providing targeted therapy and precise prognostic information. Conclusions: These priorities will inform the development and future directions of the Canadian Childhood Nephrotic Syndrome (CHILDNEPH project, a national research initiative to improve care and outcomes of patients with childhood onset nephrotic syndrome.

  7. 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.

  8. Cyclophosphamide and rituximab in frequently relapsing/steroid-dependent nephrotic syndrome.

    Science.gov (United States)

    Webb, Hazel; Jaureguiberry, Graciana; Dufek, Stephanie; Tullus, Kjell; Bockenhauer, Detlef

    2016-04-01

    Steroid-sensitive nephrotic syndrome is the most common form of nephrotic syndrome in childhood, defined by the response to treatment with glucocorticoids with consequent remission. While most children eventually experience spontaneous resolution of the disease, some have a difficult course with frequent relapses or steroid dependence nephrotic syndrome (FRSDNS). The consequent steroid toxicity often prompts administration of other immunosuppressive drugs, traditionally cyclophosphamide. Recently, rituximab has been reported as effective in this disorder, but long-term experience is lacking. Retrospective note review of all children with FRSDNS treated with a first course of cyclophosphamide and/or rituximab in our center between December 2006 and April 2015. We reviewed time to first relapse after treatment, co-medications, and side effects. A total of 102 children were treated with cyclophosphamide (79) and/or rituximab (42). Of these, 34 received cyclophosphamide prior to rituximab. Median time to first relapse was 7 months after cyclophosphamide and 14 months after rituximab. Documented side effects of cyclophosphamide included neutropenia, hair loss, and hemorrhagic cystitis (1). Rituximab was associated with an allergic reaction at infusion in two patients. Rituximab was used in children with the most difficult to treat FRSDNS, yet was associated with longer remission time and less side effects than cyclophosphamide. A randomized controlled trial is needed to directly compare these drugs.

  9. 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.

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

    Directory of Open Access Journals (Sweden)

    So Min Lee

    2014-01-01

    Full Text Available 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.

  11. Association of eNOS gene intron 4 a/b VNTR polymorphisms in children with nephrotic syndrome.

    Science.gov (United States)

    Dursun, Hasan; Noyan, Aytul; Matyar, Selcuk; Buyukcelik, Mithat; Soran, Mustafa; Cengiz, Nurcan; Bayazit, Aysun K; Seydaoglu, Gulsah; Attila, Gulen; Anarat, Ali

    2013-06-15

    To investigate the association of endothelial nitric oxide synthase gene intron 4 (eNOS4) polymorphisms with nephrotic syndrome, the eNOS4 genotypes were assessed in 161 children with nephrotic syndrome in comparison with 78 healthy subjects. We classified the children with nephritic syndrome into 2 groups: as steroid-sensitive nephrotic syndrome (SSNS) (n=125) and steroid-resistant nephrotic syndrome (SRNS) (n=36). The eNOS4 polymorphisms were analyzed by polymerase chain reaction. The frequencies of eNOS4 aa, ab and bb genotypes were 3%, 31%, and 66% in all the nephrotic syndrome groups, and 1%, 23%, and 76% in the control group (x(2)=2.87, p>0.05). In addition, the frequencies of eNOS4 aa, ab and bb genotypes were 2%, 33%, and 65% in SSNS group, and 5%, 28%, and 67% in the SRNS group (x(2)=1.13, p=0.567). The present study is the first to investigate eNOS4 gene polymorphisms in children with SSNS and SRNS. Our data show that the eNOS4 gene polymorphisms were not associated with the development, frequent relapse and response to steroid in nephritic syndrome. Copyright © 2013 Elsevier B.V. All rights reserved.

  12. Long-term follow-up of children with steroid-responsive nephrotic syndrome

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    Esfehani S.T.

    2008-06-01

    Full Text Available Background: Nephrotic syndrome is one of the most remarkable diseases in childhood. The majority of patients have prompt response to corticosteroids. Methods: In this study, we retrospectively evaluate the outcome of patients with steroid-responsive nephritic syndrome. Medical records from January 1996 to September 2006 were reviewed to identify all children with steroid sensitive nephrotic syndrome at the Pediatric Medical Center, Tehran, Iran. Initial steroid therapy was 60 mg/m2 per day for four weeks. Levamisole, a steroid-sparing agent, was prescribed at a dose of 2.5 mg/kg on alternate days in conjunction with alternate-day prednisolone. If no benefit was observed by three months, levamisole was discontinued and immunosuppressive therapy with cyclophosphamide at a dose of 3 mg/kg daily for 8 weeks, or cyclosporin A at a dose of 3-5 mg/kg was prescribed.   Result: Of 745 children with steroid sensitive nephrotic syndrome, 63.1% of patients were male. The most common causes were minimal change disease (98/324, 30.2% and focal segmental glomerulosclerosis (81/324, 25%. At presentation, microscopic hematuria was found in 22.6% of the patients. During follow-up, 9.2% had no relapse at any time, while 15.8% were frequent relapsers. The remission period ranged from 3.5 to 168 months. At the last follow-up, 57.6% of the patients were in remission, 37.7% relapsed and 29 children developed chronic renal failure. The outcome of nephrotic syndrome was not associated with age or gender. The end clinical status of patients correlated with duration of remission, number of subsequent relapses and response to cytotoxic agents.Conclusions: Steroid-responsive nephrotic syndrome in children should be followed over a long period, especially patients with early relapse. Relapse was seen in more than 90% of patients. Documentation of histopathology by renal biopsy may be helpful to identify those at increased risk for a poor outcome.

  13. Randomized Clinical Trial Design to Assess Abatacept in Resistant Nephrotic Syndrome.

    Science.gov (United States)

    Trachtman, Howard; Gipson, Debbie S; Somers, Michael; Spino, Cathie; Adler, Sharon; Holzman, Lawrence; Kopp, Jeffrey B; Sedor, John; Overfield, Sandra; Elegbe, Ayanbola; Maldonado, Michael; Greka, Anna

    2018-01-01

    Treatment-resistant nephrotic syndrome is a rare form of glomerular disease that occurs in children and adults. No Food and Drug Administration-approved treatments consistently achieve remission of proteinuria and preservation of kidney function. CD80 (B7-1) can be expressed on injured podocytes, and administration of abatacept (modified CTLA4-Ig based on a natural ligand to CD80) has been associated with sustained normalization of urinary protein excretion and maintenance of glomerular filtration rate in experimental and clinical settings. In this report, we describe the rationale for and design of a randomized, placebo-controlled, clinical trial of abatacept in patients with treatment-resistant nephrotic syndrome caused by focal segmental glomerulosclerosis or minimal change disease. The design is a hybrid of a parallel-group and crossover design (switchover) with the primary objectives assessed in the first period of the study and the secondary objectives assessed using data from both periods. All participants will receive the active agent in 1 of the periods. The duration of treatment will be 4 months per period. The primary outcome will be improvement in nephrotic-range proteinuria to subnephrotic range, that is, reduction from baseline to 4 months in urine protein:creatinine ratio ≥ 50% and to a level precision medicine-based approach to this serious kidney condition in which the selection of a therapeutic agent is guided by the underlying disease mechanism operating in individual patients.

  14. Hyperlipidemia in childhood idiopathic nephrotic syndrome during initial remission and relapse.

    Science.gov (United States)

    Mahmud, S; Jahan, S; Hossain, M M

    2011-07-01

    The objective of this study was to correlate hyperlipidemia during initial remission of nephrotic child with relapse. This observational prospective study was carried out among 26 children, between 1 to 8 years age with first attack idiopathic nephrotic syndrome, who was seen at Paediatric Nephrology Department of BSMMU and Paediatric Nephrology Department, NIKDU, from December'2005 to August'2006 and were followed-up for at least 6 months after initial attack. Twenty two age and sex matched hospitalized children, suffering from non-renal diseases, were enrolled as controls. Fasting blood samples for lipid profile were taken at the time of diagnosis of both cases and controls and also during remission of cases only. These patients were divided into two groups based on serum lipid profile during remission. Group-I consisted 16 patients who had normal lipid profile during remission and Group-II consisted 10 patients who had abnormal lipid profile during remission. Both Groups I & II had higher mean levels of serum cholesterol, LDL, TG and Lp(a) than those of controls during initial diagnosis. Between two groups on remission, Group-II patients showed higher mean serum cholesterol (332.9±105.19 mg/dL vs. 183.13±16.89 mg/dL; pchildhood idiopathic nephrotic syndrome.

  15. Acute ischemic stroke associated with nephrotic syndrome: Incidence and significance — Retrospective cohort study

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    Hirotaka Iwaki

    2015-09-01

    Full Text Available We report 10 cases with arterial ischemic stroke (AIS with nephrotic syndrome (NS, and clarified its incidence and clinical characteristics. The patients having albumin less than 3.0 g/dl and serum cholesterol greater than 250 mg/dl at the same time were retrospectively screened from 11,161 cases of stroke. Furthermore, the patients of AIS showing heavy proteinuria were selected. The 10 cases were diagnosed as AIS with NS. Its incidence was 0.09% of all kinds of stroke and 0.12% of AIS. Their subtypes were 6 large-artery atherosclerosis, 3 small-vessel occlusion, and 1 cardioembolism. We carried out a retrospective cohort study to assess the association between NS and atherosclerosis progression in AIS patients. Seven AIS patients with NS due to diabetic nephropathy (cases; NS group were compared with patients with AIS and diabetes mellitus (DM without NS (control group. Control group subjects were matched in a 2:1 ratio to cases by age, sex, use of medications for DM, and hemoglobin A1c (HbA1c level. The NS group had high cerebral artery atherosclerosis scores, especially in the anterior circulation. The NS group demonstrated atherosclerosis of the internal carotid and lower extremity arteries, although there were no statistical differences between the two groups. Study subjects had high serum fibrinogen and D-dimer levels, suggesting that AIS patients with NS have a greater degree of hypercoagulability than AIS patients without NS.

  16. 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

  17. Renoprotection by continuous erythropoietin receptor activator in puromycin aminonucleoside-induced nephrotic syndrome.

    Science.gov (United States)

    Aizawa, Ken; Takeda, Satoshi; Tashiro, Yoshihito; Yorozu, Keigo; Hirata, Michinori; Kanada, Hirotaka; Moriguchi, Yoshiyuki; Endo, Koichi

    2012-01-01

    Recent studies have demonstrated that erythropoiesis-stimulating agents (ESAs) induce a tissue-protective effect in the kidney. In this study, we examined whether continuous erythropoietin receptor activator (CERA), a long-acting ESA, could prevent kidney injury, especially podocyte damage, in a rat model of nephrotic syndrome induced by puromycin aminonucleoside (PAN). Rats were injected with CERA (30 µg/kg) or vehicle 4 h before the injection of PAN (50 mg/kg). Renal function, kidney injury, and podocyte damage were assessed at 7 days. The levels of proteinuria, BUN, and plasma creatinine significantly increased in rats with PAN-induced nephrosis. Treatment with CERA significantly prevented these deteriorations induced by PAN. Glomerular lesions, especially vacuolation of podocytes, and the increase of desmin expression in PAN-treated rats were significantly ameliorated by treatment with CERA. Treatment with CERA also significantly prevented the decrease in the protein productions of nephrin and podocin in the kidneys of PAN-treated rats. We found persistent activation of the Akt signaling pathway in the kidneys of CERA-treated rats. CERA could ameliorate renal dysfunction in PAN-induced nephrosis, which might be due to the amelioration of podocyte injury. CERA inhibited the depletion of nephrin and podocin, key components of the glomerular filtration barrier, and alleviated proteinuria. Activation of the Akt signaling pathway might be involved in the renoprotective effect of CERA. Copyright © 2012 S. Karger AG, Basel.

  18. NPHS2 mutations in children with steroid-resistant nephrotic syndrome.

    Science.gov (United States)

    Otukesh, Hasan; Ghazanfari, Behzad; Fereshtehnejad, Seyed-Mohammad; Bakhshayesh, Masoomeh; Hashemi, Mehrdad; Hoseini, Rozita; Chalian, Majid; Salami, Arezoo; Mehdipor, Leila; Rahiminia, Aysan

    2009-04-01

    Congenital nephrotic syndrome may be caused by mutations in NPHS1 and NPHS2, which encode nephrin and podocin, respectively. Since the identification of the NPHS2 gene, various investigators have demonstrated that its mutation is an important cause of steroid-resistant nephrotic syndrome. We aimed to evaluate frequency and spectrum of podocin mutations in the Iranian children with steroid-resistant nephritic syndrome. We examined 20 children with steroid-resistant nephritic syndrome referred to Ali Asghar Children's Hospital, in Tehran, Iran. Mutations in the 5th and 7th exons of NPHS2 were assessed. The mutational analysis of NPHS2 was performed by DNA sequencing. The mean age at the onset of proteinuria was 6.4 +/- 3.6 years. None of the children had mutations in the exons 5 or 7. Our study suggests that NPHS2 mutations in exons 5 and 7 are not seen in our children. Therefore, we cannot recommend NPHS2 (exons 5 and 7) mutation for screening in Iranian children with steroid-resistant nephritic syndrome. Other exons of podocin or other podocyte proteins in Iranian children may play a role in pathogenesis of steroid-resistant nephritic syndrome.

  19. Factors influencing practice variation in the management of nephrotic syndrome: a qualitative study of pediatric nephrology care providers.

    Science.gov (United States)

    Samuel, Susan M; Flynn, Rachel; Zappitelli, Michael; Dart, Allison; Parekh, Rulan; Pinsk, Maury; Mammen, Cherry; Wade, Andrew; Scott, Shannon D

    2017-06-07

    Treatment protocols for childhood nephrotic syndrome are highly variable between providers and care centres. We conducted a qualitative study to understand the complex multilevel processes that lead to practice variation and influence provider management of nephrotic syndrome. Focus groups with multidisciplinary pediatric nephrology care providers ( n = 67) from 10 Canadian pediatric nephrology centres that had more than 1 pediatric nephrologist were conducted between September 2013 and April 2015. Focus group discussions were guided by the Ottawa Model for Research Use. We used a semistructured interview guide to elicit participants' perspectives regarding 1) the work setting and context of the clinical environment, 2) reasons for variation at the provider level and 3) clinical practice guidelines for nephrotic syndrome. Focus group discussions were transcribed and analyzed concurrently with the use of qualitative content analysis. Emerging themes were grouped into 2 categories: centre-level factors and provider-level factors. At the centre level, the type of care model used, clinic structures and resources, and lack of communication and collaboration within and between Canadian centres influenced care variation. At the provider level, use of experiential knowledge versus empirical knowledge and interpretation of patient characteristics influenced provider management of nephrotic syndrome. Centre- and provider-level factors play an important role in shaping practice differences in the management of childhood nephrotic syndrome. Further research is needed to determine whether variation in care is associated with disparities in outcomes. Copyright 2017, Joule Inc. or its licensors.

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

    NARCIS (Netherlands)

    Wijnhoven, T.J.M.; Geelen, J.M.; Bakker, M.; Lensen, J.F.M.; Rops, A.L.; Kramer, A.B.; Navis, G.J.; Hoven, M.J.W. van den; Vlag, J. van der; Berden, J.H.M.; Wetzels, J.F.M.; Heuvel, L.P.W.J. van den; Monnens, L.A.H.; Kuppevelt, A.H.M.S.M. van

    2007-01-01

    BACKGROUND: 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

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

    NARCIS (Netherlands)

    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.

    2007-01-01

    Background. 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

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

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    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.

  3. Responsiveness of the PROMIS® measures to changes in disease status among pediatric nephrotic syndrome patients: a Midwest pediatric nephrology consortium study.

    Science.gov (United States)

    Selewski, David T; Troost, Jonathan P; Cummings, Danyelle; Massengill, Susan F; Gbadegesin, Rasheed A; Greenbaum, Larry A; Shatat, Ibrahim F; Cai, Yi; Kapur, Gaurav; Hebert, Diane; Somers, Michael J; Trachtman, Howard; Pais, Priya; Seifert, Michael E; Goebel, Jens; Sethna, Christine B; Mahan, John D; Gross, Heather E; Herreshoff, Emily; Liu, Yang; Carlozzi, Noelle E; Reeve, Bryce B; DeWalt, Darren A; Gipson, Debbie S

    2017-08-23

    Nephrotic syndrome represents a condition in pediatric nephrology typified by a relapsing and remitting course, proteinuria and the presence of edema. The PROMIS measures have previously been studied and validated in cross-sectional studies of children with nephrotic syndrome. This study was designed to longitudinally validate the PROMIS measures in pediatric nephrotic syndrome. One hundred twenty seven children with nephrotic syndrome between the ages of 8 and 17 years participated in this prospective cohort study. Patients completed a baseline assessment while their nephrotic syndrome was active, a follow-up assessment at the time of their first complete proteinuria remission or study month 3 if no remission occurred, and a final assessment at study month 12. Participants completed six PROMIS measures (Mobility, Fatigue, Pain Interference, Depressive Symptoms, Anxiety, and Peer Relationships), the PedsQL version 4.0, and two global assessment of change items. Disease status was classified at each assessment: nephrotic syndrome active in 100% at baseline, 33% at month 3, and 46% at month 12. The PROMIS domains of Mobility, Fatigue, Pain Interference, Depressive Symptoms, and Anxiety each showed a significant overall improvement over time (p change, the domains of Mobility, Fatigue, Pain Interference, and Anxiety consistently changed in an expected fashion. With the exception of Pain Interference, change in PROMIS domain scores did not correlate with changes in disease activity. PROMIS domain scores were moderately correlated with analogous PedsQL domain scores. This study demonstrates that the PROMIS Mobility, Fatigue, Pain Interference, and Anxiety domains are sensitive to self-reported changes in disease and overall health status over time in children with nephrotic syndrome. The lack of significant anchoring to clinically defined nephrotic syndrome disease active and remission status may highlight an opportunity to improve the measurement of HRQOL in children

  4. Congenital nephrotic syndrome may respond to cyclosporine A: A case report and review of literature

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    Mulić Bilsana

    2017-01-01

    Full Text Available Introduction. Congenital nephrotic syndrome (CNF is manifested at birth or within the first three months of life. The Finnish-type of CNF is caused by the mutation of the NPHS1 gene, which encodes nephrin in the podocyte slit diaphragm. It is a very severe disease, for which immunosuppressive therapy is not advised. Here we describe a patient with CNF who responded to CsA by partial remission. Case outline. A girl aged 2.5 months presented with severe non-syndromic steroid-resistant nephrotic syndrome. She needed aggressive support including daily albumin infusions and diuretics. Substitution of vitamin D, thyroxin, and anticoagulants were regularly administered. She was also treated with angiotensin converting enzyme inhibitor, without clear benefits regarding proteinuria. In addition, she received intravenous gamma-globulin replacement therapy and antibiotics during frequent infections. While waiting for the results of genetic analyses and faced with many problems related to daily albumin infusions, infections, and thromboembolic complications, cyclosporine A (CsA was introduced as an alternative to early nephrectomy and consequent renal failure. The patient responded by partial remission and CsA treatment continued at home without the albumin infusions. After almost five years since the beginning of the treatment, the patient’s renal function remains unreduced. Conclusion. Our case demonstrates that CsA can induce partial remission in patients with genetic forms of steroid-resistant nephrotic syndrome without influencing the glomerular filtration rate. However, its long-term effect and safety should carefully be monitored. [Project of the Ministry of Education, Science and Technological Development of Republic of Serbia, Grant No. 175079

  5. Intracardiac and intracerebral thrombosis associated with methylenetetrahydrofolate reductase A1298C homozygote mutation in paediatric steroidresistant nephrotic syndrome

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    Rahime Renda

    2016-12-01

    Full Text Available Thromboembolic complications are a significant cause of morbidity and mortality in cases of nephrotic syndrome. Hereditary thrombophilias are also known to increase vascular thrombosis. We present a case that has been followed up for steroid-resistant nephrotic syndrome (NS in which intracardiac and intracranial thrombosis subsequently developed. The patient was found to have a homozygote mutation in the methylenetetrahydrofolate reductase (MTHFR gene as an additional risk factor for recurrent thrombosis. MTHFR mutation with NS was considered to have an important effect on the development of life-threatening thrombosis.

  6. Spectrum of steroid-resistant and congenital nephrotic syndrome in children: the PodoNet registry cohort.

    Science.gov (United States)

    Trautmann, Agnes; Bodria, Monica; Ozaltin, Fatih; Gheisari, Alaleh; Melk, Anette; Azocar, Marta; Anarat, Ali; Caliskan, Salim; Emma, Francesco; Gellermann, Jutta; Oh, Jun; Baskin, Esra; Ksiazek, Joanna; Remuzzi, Giuseppe; Erdogan, Ozlem; Akman, Sema; Dusek, Jiri; Davitaia, Tinatin; Özkaya, Ozan; Papachristou, Fotios; Firszt-Adamczyk, Agnieszka; Urasinski, Tomasz; Testa, Sara; Krmar, Rafael T; Hyla-Klekot, Lidia; Pasini, Andrea; Özcakar, Z Birsin; Sallay, Peter; Cakar, Nilgun; Galanti, Monica; Terzic, Joelle; Aoun, Bilal; Caldas Afonso, Alberto; Szymanik-Grzelak, Hanna; Lipska, Beata S; Schnaidt, Sven; Schaefer, Franz

    2015-04-07

    Steroid-resistant nephrotic syndrome is a rare kidney disease involving either immune-mediated or genetic alterations of podocyte structure and function. The rare nature, heterogeneity, and slow evolution of the disorder are major obstacles to systematic genotype-phenotype, intervention, and outcome studies, hampering the development of evidence-based diagnostic and therapeutic concepts. To overcome these limitations, the PodoNet Consortium has created an international registry for congenital nephrotic syndrome and childhood-onset steroid-resistant nephrotic syndrome. Since August of 2009, clinical, biochemical, genetic, and histopathologic information was collected both retrospectively and prospectively from 1655 patients with childhood-onset steroid-resistant nephrotic syndrome, congenital nephrotic syndrome, or persistent subnephrotic proteinuria of likely genetic origin at 67 centers in 21 countries through an online portal. Steroid-resistant nephrotic syndrome manifested in the first 5 years of life in 64% of the patients. Congenital nephrotic syndrome accounted for 6% of all patients. Extrarenal abnormalities were reported in 17% of patients. The most common histopathologic diagnoses were FSGS (56%), minimal change nephropathy (21%), and mesangioproliferative GN (12%). Mutation screening was performed in 1174 patients, and a genetic disease cause was identified in 23.6% of the screened patients. Among 14 genes with reported mutations, abnormalities in NPHS2 (n=138), WT1 (n=48), and NPHS1 (n=41) were most commonly identified. The proportion of patients with a genetic disease cause decreased with increasing manifestation age: from 66% in congenital nephrotic syndrome to 15%-16% in schoolchildren and adolescents. Among various intensified immunosuppressive therapy protocols, calcineurin inhibitors and rituximab yielded consistently high response rates, with 40%-45% of patients achieving complete remission. Confirmation of a genetic diagnosis but not the

  7. Plasma homocysteine and B vitamins levels in Nigerian children with nephrotic syndrome.

    Science.gov (United States)

    Orimadegun, Bose Etaniamhe; Orimadegun, Adebola Emmanuel; Ademola, Adebowale Dele; Agbedana, Emmanuel Oluyemi

    2014-01-01

    Available data on plasma homocysteine level in patients with nephrotic syndrome (NS) are controversial with increased, decreased and unchanged values reported. Therefore, plasma homocysteine and serum B vitamins in Nigerian children with NS were assessed in this study. Fasting blood samples were analysed for plasma homocysteine, serum folate and B vitamins in 42 children with NS and 42 age and sex-matched healthy controls in this case control study. Data were compared between NS and control using t test and Chi square. Relationships were tested with regression analysis with p set at 0.05. Prevalence of hyperhomocysteinaemia, low folate and cyanocobalamin in NS was 57.1%, 14.3% and 9.5% respectively. The mean homocysteine level was significantly higher in NS than control (11.3±2.6 µmol/L versus 5.5±2.3 µmol/L). Also, NS had lower folate and cyanocobalamin than control: 9.1±3.9 ng/mL versus 11.2±3.1 ng/dL and 268.5±95.7 pg/mL versus 316±117.2 pg/mL respectively. Weak but significant correlation between homocysteine and serum albumin (r = 0.347), folate (r = -0.607) and vitamin B12 (r = -0.185) were found in the NS group. Significant relationship was also found between homocysteine and vitamin B12 (ß = -0.64, 95% CI = -1.20, -0.08) after controlling for folate and vitamin B6 levels. Clinically important hyperhomocysteinaemia and low B vitamins occur in Nigerian children with nephrotic syndrome. This data suggest that potential usefulness of folate and vitamin B supplementation for reducing high homocysteine levels in nephrotic syndrome need to be further investigated.

  8. 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.

  9. Atypical Pneumocystis jiroveci pneumonia with multiple nodular granulomas after rituximab for refractory nephrotic syndrome.

    Science.gov (United States)

    Sato, Mai; Ito, Shuichi; Ogura, Masao; Kamei, Koichi; Miyairi, Isao; Miyata, Ippei; Higuchi, Masataka; Matsuoka, Kentaro

    2013-01-01

    Rituximab, an anti-CD20 antibody that targets B cells, is a promising agent against steroid-dependent and steroid-resistant nephrotic syndrome in children. We report a 3-year-old boy who presented with atypical Pneumocystis jiroveci pneumonia (PCP) following administration of rituximab for refractory nephrotic syndrome. He had received cyclosporine and daily prednisolone for over 1 year. Following rituximab therapy, a hazy shadow was observed on his chest X-ray. Chest-computed tomography revealed multiple nodular lesions in bilateral lungs, although his clinical symptoms were subtle. PCR analysis demonstrated the presence of Pneumocystis DNA in his bronchoalveolar lavage. Lung wedge resection of the nodular lesion exhibited granulomas containing a few cysts of P. jiroveci that primarily consisted of T cells and histiocytes and lacked B cells. A deficiency of B cells following rituximab treatment suggests a dramatic effect on the immune response and, therefore, could result in granulomatous PCP. Nodular granulomatous lesions of PCP comprise an emerging concept previously reported in adults with hematological disease, bone marrow transplant, or treatment with rituximab. We report the first pediatric case of nodular PCP. Granulomatous PCP can be life-threatening. Moreover, bronchoalveolar lavage often fails to demonstrate the presence of P. jiroveci DNA. Wedge biopsy is warranted for definitive diagnosis. Our patient fully recovered with sulfamethoxazole/trimethoprim treatment because of early detection. The indication of rituximab for refractory nephrotic syndrome has increased recently. Therefore, recognition of the risk of atypical PCP is important. Our findings suggest that PCP prophylaxis should be considered following rituximab therapy.

  10. [Childhood steroid-dependent idiopathic nephrotic syndrome: Predictive factors for the need of immunosuppressive treatment].

    Science.gov (United States)

    Golay, M; Douillard, A; Nagot, N; Fila, M; Ichay, L; Dalla Vale, F; Tenenbaum, J; Morin, D

    2017-11-01

    More than half of the children with idiopathic nephrotic syndrome become steroid-dependent (or frequent relapsers) and will later require the use of complementary treatment aiming to reduce steroids' side effects and to limit the number of proteinuria relapses. It appears important to identify these children as early as possible in order to adapt their treatment. The aim of this study was to analyze the population of children, under 18 years of age, diagnosed between 1/01/2000 and 31/05/2015 with an idiopathic nephrotic syndrome and followed at the Montpellier University Hospital to search for criteria predictive of steroid-sparing agent use. In this retrospective study of children with idiopathic nephrotic syndrome, the exclusion criteria were primary steroid resistance and children with no proteinuria relapse after diagnosis. Eighty-four children (54 boys) were included in this study. The mean follow-up duration was 5.5 years (0.75-16). The mean age at diagnosis was 4.6 years. Sixty-five children (77%) received at least one steroid-sparing agent during their follow-up, within a mean 10 months after diagnosis. In these patients, the first relapse of the disease occurred earlier when compared with the children who were maintained on steroid alone (4 months vs 7 months; P<0.001). The use of methylprednisolone pulses to obtain a remission, the cumulative dose of steroid treatment, and the number of proteinuria relapses were also significantly correlated with the use of complementary immunosuppressive therapy. We found no predictive criteria of the use of steroid-sparing agents at diagnosis in our population of children. Nevertheless, with the steroid regimen used, the time within which the first proteinuria relapse occurred appears to be a significant criterion for the secondary use of a steroid-sparing agent. These data should be taken into account when choosing the treatment regimen. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

  11. Three year outcome of childhood idiopathic nephrotic syndrome under a unified immunosuppressive protocol.

    Science.gov (United States)

    Hibino, Satoshi; Uemura, Osamu; Nagai, Takuhito; Yamakawa, Satoshi; Iwata, Naoyuki; Ito, Hidekazu; Nakano, Masaru; Tanaka, Kazuki

    2015-01-01

    This retrospective study was performed to assess the 3 year outcome of a unified protocol for childhood idiopathic nephrotic syndrome. Cyclosporine A (CsA) or CsA plus mycophenolate mofetil (MMF) were used in patients without remission on high-dose steroid therapy. CsA was maintained at an area under the whole blood concentration-time curve up to 4 h after dose (AUC0-4 ) of 1500 and 2000 ng·h/mL in steroid-dependent nephrotic syndrome (SDNS) and steroid-resistant nephrotic syndrome (SRNS), respectively. Ninety-one children were enrolled in the study (SDNS, n = 64; SRNS, n = 18). Patients were divided into minimal change (MC) and focal segmental glomerulosclerosis (FSGS) groups. Three year outcome was evaluated using clinical severity defined as degree of dependence on immunosuppressive therapy for maintenance of remission. In the SDNS group, the numbers of MC and no biopsy were 51 and 13, respectively. No patient had FSGS. Twelve SRNS patients had FSGS and six had MC. In SDNS, 15/64 patients (23%) received no medication. CsA was effective as steroid-sparing agent in 31/38 patients (82%). MMF was effective in all eight patients for whom CsA was unsuccessful. Remission rate in the SRNS group was 14/18 (78%; eight with CsA, and six with a combination of CsA + MMF). Five of the 14 SRNS remission patients received methylprednisolone pulse therapy. Four were resistant to therapy, and had impaired renal function. The clinical severity of MC and FSGS overlapped. Treatment with CsA and combination of CsA plus MMF was useful for SDNS and for remission induction in SRNS. © 2014 Japan Pediatric Society.

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

    Science.gov (United States)

    Bennett, Michael R; Pleasant, LaTawnya; Haffner, Christopher; Ma, Qing; Haffey, Wendy D; Ying, Jun; Wagner, Michael; Greis, Kenneth D; Devarajan, Prasad

    2017-01-01

    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.

  13. Nephrotic Syndrome Secondary to Proliferative Glomerulonephritis with Monoclonal Immunoglobulin Deposits of Lambda Light Chain

    Directory of Open Access Journals (Sweden)

    Seongseok Yun

    2014-01-01

    Full Text Available We describe a rare case of a 46-year-old woman with history of refractory nephrotic syndrome and hypertension who presented with worsening proteinuria and kidney function. Work-up for both autoimmune and infectious diseases and hematologic malignancies including multiple myeloma were negative. Kidney biopsy demonstrated glomerular sclerotic change with lambda light chain deposits in the subendothelial space, which is consistent with proliferative glomerulonephritis with monoclonal immunoglobulin deposit (PGNMID. The patient was treated with bortezomib and dexamethasone without clinical improvement and eventually became hemodialysis dependent.

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

    DEFF Research Database (Denmark)

    Andersen, Mikkel René; Buhl, Kristian B; 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...

  15. Therapy-resistant anaemia in congenital nephrotic syndrome of the Finnish type--implication of EPO, transferrin and transcobalamin losses.

    Science.gov (United States)

    Toubiana, Julie; Schlageter, Marie-Hélène; Aoun, Bilal; Dunand, Olivier; Vitkevic, Renata; Bensman, Albert; Ulinski, Tim

    2009-04-01

    Congenital nephrotic syndrome of the Finnish type (CNF) is due to NPHS1 mutation and is responsible for a variety of urinary protein losses. We report the case of a 4-month-old girl with a particularly severe form (proteinuria approximately 150 g/l) of CNF. She developed severe non-regenerative anaemia requiring bi-monthly blood transfusions despite daily EPO (600 UI/kg) and iron supplementation. Epoetin pharmacokinetics revealed a urinary loss of 27% of the given dose within the first 24 h after IV injection. However, plasma levels remained increased after 24 h (228 UI/l). Plasma transferrin and transcobalamin levels were undetectable. Atransferrinaemia and atranscobalaminaemia seem to be responsible for disturbed erythropoiesis.

  16. Urinary potassium to urinary potassium plus sodium ratio can accurately identify hypovolemia in nephrotic syndrome: a provisional study.

    Science.gov (United States)

    Keenswijk, Werner; Ilias, Mohamad Ikram; Raes, Ann; Donckerwolcke, Raymond; Walle, Johan Vande

    2018-01-01

    There is evidence pointing to a decrease of the glomerular filtration rate (GFR) in a subgroup of nephrotic children, likely secondary to hypovolemia. The aim of this study is to validate the use of urinary potassium to the sum of potassium plus sodium ratio (UK/UK+UNa) as an indicator of hypovolemia in nephrotic syndrome, enabling detection of those patients who will benefit from albumin infusion. We prospectively studied 44 nephrotic children and compared different parameters to a control group (36 children). Renal perfusion and glomerular permeability were assessed by measuring clearance of para-aminohippurate and inulin. Vaso-active hormones and urinary sodium and potassium were also measured. Subjects were grouped into low, normal, and high GFR groups. In the low GFR group, significantly lower renal plasma flow (p = 0.01), filtration fraction (p = 0.01), and higher UK/UK+UNa (p = 0.03) ratio were noted. In addition, non-significant higher plasma renin activity (p = 0.11) and aldosteron (p = 0.09) were also seen in the low GFR group. A subgroup of patients in nephrotic syndrome has a decrease in glomerular filtration, apparently related to hypovolemia which likely can be detected by a urinary potassium to potassium plus sodium ratio > 0.5-0.6 suggesting benefit of albumin infusion in this subgroup. What is Known: • Volume status can be difficult to assess based on clinical parameters in nephrotic syndrome, and albumin infusion can be associated with development of pulmonary edema and fluid overload in these patients. What is New: • Urinary potassium to the sum of urinary potassium plus sodium ratio can accurately detect hypovolemia in nephrotic syndrome and thus identify those children who would probably respond to albumin infusion.

  17. The efficacy of rituximab in treatment of childhood steroid resistant and steroid dependent nephrotic syndrome: a systematic review and Meta-analysis

    Directory of Open Access Journals (Sweden)

    Hamid Mohammadjafar

    2013-05-01

    Full Text Available Corticosteroid resistant and dependent nephrotic syndrome in children is a challenge and there are some difficulties in treating such patients. We reviewed the current studies that evaluated therapeutic role of a relatively new immunosuppressive drug “rituximab” in reducing proteinuria and reduction of relapse rate in less than 16 year old patients with non-responsive or steroid dependent nephrotic syndrome. We searched Medline, Embase, web of science and Cochrane library with appropriate keywords and conducted the complete remission, relapse rate and the mean number of relapses 12 month after therapy on Meta-analysis. We put the data on two different subgroups; steroid resistant nephrotic syndrome and steroid dependent or frequent relapser nephrotic syndrome. In Steroid Resistant Nephrotic syndrome children, the complete remission was 0.27 (0.2- 0.34. In Steroid Dependent Nephrotic syndrome patients, the overall standard mean differences of mean number of relapses 12 mo after treatment in pooled four studies (56 cases was 2.63 (2.03, 3.24. In these dependent patients, the data on relapse rate after treatment pooled on 6 studies (162 cases and yield to the rate of 0.42 (0.15, 0.69 with the range of 0.09 to 0.83. In conclusion, Rituximab is a reasonable therapy for Steroid Dependent and Steroid Resistant Nephrotic syndrome children. In view of paucity of randomized data, we suggest to perform newer controlled multicenter studies

  18. [Thymic disease associated with nephrotic syndrome: a new case with membranous nephropathy and literature review].

    Science.gov (United States)

    Seguier, J; Guillaume-Jugnot, P; Ebbo, M; Daniel, L; Jourde-Chiche, N; Burtey, S; Bernit, E; Thomas, P; Harlé, J-R; Schleinitz, N

    2015-07-01

    Malignant thymoma or thymic hyperplasia is associated with various autoimmune diseases. Renal disease has rarely been reported in this condition. We report a new case with improvement of renal disease after thymectomy. A 77-year-old-women with nephritic syndrome was found to have associated thymic mass. Renal pathology showed membranous nephropathy. The thymic mass pathology showed a B2 type thymoma. After thymectomy the nephrotic syndrome improved. Glomerulopathy can be secondary to an acquired thymic disease. Membranous nephropathy but also other glomerular diseases can be observed often presenting with nephritic syndrome. Despite the rarity of this association this clinical observation underlines that a thymoma should be searched in the presence of a glomerulopathy. The glomerulopathy can be improved by the treatment of the thymoma. Copyright © 2014 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.

  19. Rac1 activation in podocytes induces the spectrum of nephrotic syndrome.

    Science.gov (United States)

    Robins, Richard; Baldwin, Cindy; Aoudjit, Lamine; Côté, Jean-François; Gupta, Indra R; Takano, Tomoko

    2017-08-01

    Hyper-activation of Rac1, a small GTPase, in glomerular podocytes has been implicated in the pathogenesis of familial proteinuric kidney diseases. However, the role of Rac1 in acquired nephrotic syndrome is unknown. To gain direct insights into this, we generated a transgenic mouse model expressing a doxycycline-inducible constitutively active form of Rac1 (CA-Rac1) in podocytes. Regardless of the copy number, proteinuria occurred rapidly within five days, and the histology resembled minimal change disease. The degree and severity of proteinuria were dependent on the transgene copy number. Upon doxycycline withdrawal, proteinuria resolved completely (one copy) or nearly completely (two copy). After one month of doxycycline treatment, two-copy mice developed glomerulosclerosis that resembled focal segmental glomerulosclerosis (FSGS) with urinary shedding of transgene-expressing podocytes. p38 MAPK was activated in podocytes upon CA-Rac1 induction while a p38 inhibitor attenuated proteinuria, podocyte loss, and glomerulosclerosis. Mechanistically, activation of Rac1 in cultured mouse podocytes reduced adhesiveness to laminin and induced redistribution of β1 integrin, and both were partially reversed by the p38 inhibitor. Activation of Rac1 in podocytes was also seen in kidney biopsies from patients with minimal change disease and idiopathic FSGS by immunofluorescence while sera from the same patients activated Rac1 in cultured human podocytes. Thus, activation of Rac1 in podocytes causes a spectrum of disease ranging from minimal change disease to FSGS, due to podocyte detachment from the glomerular basement membrane that is partially dependent on p38 MAPK. Copyright © 2017 International Society of Nephrology. Published by Elsevier Inc. All rights reserved.

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

    Directory of Open Access Journals (Sweden)

    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.

  1. Long-term repeated rituximab treatment for childhood steroid-dependent nephrotic syndrome.

    Science.gov (United States)

    Kim, Ji Hyun; Park, Eujin; Hyun, Hye Sun; Cho, Myung Hyun; Ahn, Yo Han; Choi, Hyun Jin; Kang, Hee Gyung; Ha, Il-Soo; Cheong, Hae Il

    2017-09-01

    Rituximab (RTX) can be used as a rescue therapy for steroid-dependent nephrotic syndrome (SDNS). However, the efficacy and safety of long-term, repeated use of RTX are not established. This study was conducted to assess the efficacy and safety of long-term, repeated RTX treatment in children. Eighteen consecutive child patients with SDNS who were treated with three or more cycles of RTX for one year or longer were recruited, and their medical records were retrospectively reviewed. The patients were followed for 4.7 ± 1.9 years and received 5.2 ± 2.3 cycles of RTX over 2.8 ± 1.1 years. Approximately 70% of the additional RTX cycles were administered due to recovery of B-cells without relapse. The relapse rate decreased from 3.4 ± 2.0 per year initially to 0.4 ± 0.8 per year at the third year after RTX treatment. Approximately 10% of the RTX infusions were accompanied by mild infusion reactions. Eight patients showed sustained remission without any oral medication after the last cycle of RTX, while 10 patients had one or more episodes of relapse after the last cycle of RTX. The relapse rate in the latter group decreased from 2.8 ± 1.5 per year before RTX treatment to 1.3 ± 0.8 per year after cessation of RTX treatment. No significant differences in clinical parameters were found between the two groups. This retrospective study showed that pre-emptive and long-term, repeated RTX treatment is relatively effective and safe in children with SDNS. However, well-designed prospective studies are needed to confirm these findings.

  2. Long-term repeated rituximab treatment for childhood steroid-dependent nephrotic syndrome

    Directory of Open Access Journals (Sweden)

    Ji Hyun Kim

    2017-09-01

    Full Text Available Background: Rituximab (RTX can be used as a rescue therapy for steroid-dependent nephrotic syndrome (SDNS. However, the efficacy and safety of long-term, repeated use of RTX are not established. This study was conducted to assess the efficacy and safety of long-term, repeated RTX treatment in children. Methods: Eighteen consecutive child patients with SDNS who were treated with three or more cycles of RTX for one year or longer were recruited, and their medical records were retrospectively reviewed. Results: The patients were followed for 4.7 ± 1.9 years and received 5.2 ± 2.3 cycles of RTX over 2.8 ± 1.1 years. Approximately 70% of the additional RTX cycles were administered due to recovery of B-cells without relapse. The relapse rate decreased from 3.4 ± 2.0 per year initially to 0.4 ± 0.8 per year at the third year after RTX treatment. Approximately 10% of the RTX infusions were accompanied by mild infusion reactions. Eight patients showed sustained remission without any oral medication after the last cycle of RTX, while 10 patients had one or more episodes of relapse after the last cycle of RTX. The relapse rate in the latter group decreased from 2.8 ± 1.5 per year before RTX treatment to 1.3 ± 0.8 per year after cessation of RTX treatment. No significant differences in clinical parameters were found between the two groups. Conclusion: This retrospective study showed that pre-emptive and long-term, repeated RTX treatment is relatively effective and safe in children with SDNS. However, well-designed prospective studies are needed to confirm these findings.

  3. Nephrotic syndrome in pregnancy wrongly diagnosed and managed ...

    African Journals Online (AJOL)

    The index case which is being presented is a 27 year old multigravid multiparous trader that was eventually diagnosed asnephrotic syndrome in pregnancy which was being managed as preeclampsia. The patient subsequently had a renal biopsy done which showed minimal change nephropathy. However, the patient was ...

  4. The correlation between attention deficit hyperactivity disorder and steroid-dependent nephrotic syndrome

    Directory of Open Access Journals (Sweden)

    Parsa Yousefichaijan

    2015-01-01

    Full Text Available Nephrotic syndrome (NS is characterized by nephritic-range proteinuria and the triad of clinical findings associated with large urinary losses of protein, hypoalbuminemia, edema and hyperlipidemia. More than 80% of children below 13 years of age with primary NS have steroid-responsive forms. There is no identifiable cause of attention-deficit hyperactivity disorder (ADHD. It is likely that the symptoms of ADHD represent a final common pathway of diverse causes, including genetic, organic and environmental etiologies. This case-control study was performed on 130 children aged between 5 and 13 years who were followed-up for two years. Sixty-five children with steroid-dependent nephrotic syndrome (SDNS as the case group and 65 healthy children as the control group were included in the study. Patients with minimal change NS were treated with prednisolone for at least six months. Conner′s Parent Rating Scale - 48 (CPRS-48 was completed by the parents and the children were identified with any form of ADHD. Then, children were referred to an expert psychiatrist. The collected data were analyzed with SPSS software. The result showed that there was no significant relationship between different types of ADHD in both groups. Thus, based on current study, one may conclude that there are no significant differences between prevalence of ADHD in children with SDNS and the control group.

  5. The Correlation of Regulatory T (TReg and Vitamin D3 in Pediatric Nephrotic Syndrome

    Directory of Open Access Journals (Sweden)

    Yunika Nurtyas

    2018-01-01

    Full Text Available Nephrotic syndrome (NS is an autoimmune disease that correlates to the imbalance of regulatory T cells (TReg. This study was aimed to investigate the effect of vitamin D as adjuvant therapy of TReg population in pediatric nephrotic syndrome. This study was designed randomized clinical trial, double blind, with pre- and post-test control groups involving 15 subjects newly diagnosed with NS. Subjects were divided into 2 groups, namely K1 for group treated with prednisone+vitamin D and K2 group for prednisone treatment only. The population of TReg in peripheral blood mononuclear cells (PBMC was analyzed using flowcytometry. Vitamin D serum level was measured through ELISA method. Results showed that there was a significant elevation of TReg (independent t-test, p = 0.010 in K1 group, which was higher than in K2 group. The Pearson test in the K1 group showed that vitamin D level was positively correlated with TReg (p = 0.039, r = 0.779.

  6. The correlation between attention deficit hyperactivity disorder and steroid-dependent nephrotic syndrome.

    Science.gov (United States)

    Yousefichaijan, Parsa; Salehi, Bahman; Rafiei, Mohammad; Dahmardnezhad, Mozhgan; Naziri, Mahdyieh

    2015-11-01

    Nephrotic syndrome (NS) is characterized by nephritic-range proteinuria and the triad of clinical findings associated with large urinary losses of protein, hypoalbuminemia, edema and hyperlipidemia. More than 80% of children below 13 years of age with primary NS have steroid-responsive forms. There is no identifiable cause of attention-deficit hyperactivity disorder (ADHD). It is likely that the symptoms of ADHD represent a final common pathway of diverse causes, including genetic, organic and environmental etiologies. This case-control study was performed on 130 children aged between 5 and 13 years who were followed-up for two years. Sixty-five children with steroid-dependent nephrotic syndrome (SDNS) as the case group and 65 healthy children as the control group were included in the study. Patients with minimal change NS were treated with prednisolone for at least six months. Conner's Parent Rating Scale - 48 (CPRS-48) was completed by the parents and the children were identified with any form of ADHD. Then, children were referred to an expert psychiatrist. The collected data were analyzed with SPSS software. The result showed that there was no significant relationship between different types of ADHD in both groups. Thus, based on current study, one may conclude that there are no significant differences between prevalence of ADHD in children with SDNS and the control group.

  7. [Clinical significance of determining urinary podocalyxin level in children with primary nephrotic syndrome].

    Science.gov (United States)

    Liu, Tao; Zhang, Bi-Li; Li, Li

    2012-05-01

    To study the clinical significance of urinary podocyte marker protein podocalyxin (PCX) in the diagnosis of primary nephrotic syndrome (PNS) in children and the evaluation of disease severity. PCX levels in the first morning urine were measured by turbidimetric 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 curve). Significant increasd levels of urinary PCX were found in children with acute PNS compared with those in the remission stage and the control group (Pnephritic NS group than in the simple NS group (Pnephritic 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 10.28 ng/mL respectively, with a sensitivity and specificity of 81.1% and 93.4% respectively for acute PNS and of 94.1% and 52.8% respectively for nephritic NS. The quantitative detection of urinary PCX is useful in the evaluation of podocyte dynamic changes. It is helpful in the diagnosis of acute PNS and in the differentiation of nephritic NS and simple NS.

  8. Tacrolimus drug level and response to treatment in idiopathic childhood steroid resistant nephrotic syndrome

    International Nuclear Information System (INIS)

    Shah, S.S.; Hafeez, F.; Akhtar, N.

    2015-01-01

    The management of Steroid Resistant Nephrotic Syndrome (SRNS) is an uphill task for paediatric nephrologists as immunosuppressive agents are the mainstay of treatment in these patients. Tacrolimus is used along with steroids. This study is conducted to see the relationship between the tacrolimus dose, drug level and response in the management of SRNS. Methods: This quasi experimental study was conducted at The Childrens Hospital Lahore over a period of one year. Patients with SRNS of either sex and 1-10 years of age were included and those with secondary nephrotic syndrome were excluded. Tacrolimus was given at a dose of 0.05-0.1 mg/kg/day in 2 divided doses along with steroids. The follow-up was done for six months with proteinuria monitoring and tacrolimus drug levels done two weeks after initiation of treatment. Results: Out of 42 patients, 27 (64.3%) were males and 15 (35.7%) were females. The most common histological diagnosis observed was mesangio-proliferative glomerulonephritis in 30 (71.4%) patients. The tacrolimus trough level range was 0.5-15.20 ng/ml with a mean value of 4.68 ng/ml±2.85. Forty-one (97.6%) children showed complete response to treatment while one patient showed partial response. Conclusion: This study suggests that tacrolimus is an effective drug for treatment of SRNS in paediatric patients and there is no linear relationship between the drug dose, response and drug level. (author)

  9. Long-Term Outcome of Steroid-Resistant Nephrotic Syndrome in Children.

    Science.gov (United States)

    Trautmann, Agnes; Schnaidt, Sven; Lipska-Ziętkiewicz, Beata S; Bodria, Monica; Ozaltin, Fatih; Emma, Francesco; Anarat, Ali; Melk, Anette; Azocar, Marta; Oh, Jun; Saeed, Bassam; Gheisari, Alaleh; Caliskan, Salim; Gellermann, Jutta; Higuita, Lina Maria Serna; Jankauskiene, Augustina; Drozdz, Dorota; Mir, Sevgi; Balat, Ayse; Szczepanska, Maria; Paripovic, Dusan; Zurowska, Alexandra; Bogdanovic, Radovan; Yilmaz, Alev; Ranchin, Bruno; Baskin, Esra; Erdogan, Ozlem; Remuzzi, Giuseppe; Firszt-Adamczyk, Agnieszka; Kuzma-Mroczkowska, Elzbieta; Litwin, Mieczyslaw; Murer, Luisa; Tkaczyk, Marcin; Jardim, Helena; Wasilewska, Anna; Printza, Nikoleta; Fidan, Kibriya; Simkova, Eva; Borzecka, Halina; Staude, Hagen; Hees, Katharina; Schaefer, Franz

    2017-10-01

    We investigated the value of genetic, histopathologic, and early treatment response information in prognosing long-term renal outcome in children with primary steroid-resistant nephrotic syndrome. From the PodoNet Registry, we obtained longitudinal clinical information for 1354 patients (disease onset at >3 months and children, respectively, with the highest remission rates achieved with calcineurin inhibitor-based protocols. Ten-year ESRD-free survival rates were 43%, 94%, and 72% in children with IIS resistance, complete remission, and partial remission, respectively; 27% in children with a genetic diagnosis; and 79% and 52% in children with histopathologic findings of minimal change glomerulopathy and FSGS, respectively. Five-year ESRD-free survival rate was 21% for diffuse mesangial sclerosis. IIS responsiveness, presence of a genetic diagnosis, and FSGS or diffuse mesangial sclerosis on initial biopsy as well as age, serum albumin concentration, and CKD stage at onset affected ESRD risk. Our findings suggest that responsiveness to initial IIS and detection of a hereditary podocytopathy are prognostic indicators of favorable and poor long-term outcome, respectively, in children with steroid-resistant nephrotic syndrome. Children with multidrug-resistant sporadic disease show better renal survival than those with genetic disease. Furthermore, histopathologic findings may retain prognostic relevance when a genetic diagnosis is established. Copyright © 2017 by the American Society of Nephrology.

  10. Quantitation of proteinuria in nephrotic syndrome by spot urine protein creatinine ratio estimation in children.

    Science.gov (United States)

    Biswas, A; Kumar, R; Chaterjee, A; Ghosh, J K; Basu, K

    2009-01-01

    In Nephrotic Syndrome the amount of protein excretion is a reflection of activity of disease. Quantitative measurement of proteinuria by a 24-hour urine collection has been the accepted method of evaluation. Recent studies have shown that calculation of protein/creatinine ratio in a spot urine sample correlates well with the 24-hour urine protein (24-HUP) excretion. A study was conducted to compare the accuracy of a spot urinary protein/creatinine ratio (P/C ratio) and urinary dipstick with the 24-hour urine protein. Fifty two samples from 26 patients of nephrotic syndrome were collected. This included a 24-hour urine sample followed by the next voided random spot sample. The protein/creatinine ratio was calculated and dipstick was performed on the spot sample. This was compared with the 24-hour urine protein excretion. The correlation between the three samples was statistically highly significant (pprotein/creatinine ratio in Indian children was also estimated on 50 normal children admitted in the ward without any renal diseases calculated to be 0.053 (SE of mean+/-0.003).

  11. Candida spp. and gingivitis in children with nephrotic syndrome or type 1 diabetes.

    Science.gov (United States)

    Olczak-Kowalczyk, Dorota; Pyrżak, Beata; Dąbkowska, Maria; Pańczyk-Tomaszewska, Małgorzata; Miszkurka, Grażyna; Rogozińska, Izabela; Swoboda-Kopeć, Ewa; Gozdowski, Dariusz; Kalińska, Angelika; Piróg, Anna; Mizerska-Wasiak, Małgorzata; Roszkowska-Blaim, Maria

    2015-05-08

    Diabetes and Nephrotic syndrome (NS) promote plaque-related gingivitis and yeast-like fungal infections. The study assesses the impact of Candida spp. and general disease- or treatment-related factors on plaque-related gingivitis severity in children and adolescents with Nephrotic syndrome /diabetes. Body mass index (BMI), BMI standard deviation score, and oral cavity (Plaque Index--PLI, Gingival Index--GI, mucosa status, presence and Candida enzymatic activity) were assessed in 96 patients (32 with NS: 30- immunosuppressive treatment, 35--type 1 diabetes, and 29 generally healthy), aged; 3-18 years. Laboratory included cholesterol and triglyceride measurements; in diabetic subjects- glycated haemoglobin, in NS: total protein, albumin, creatinine, haemoglobin, haematocrit, white cell count, urinary protein excretion. Medical records supplied information on disease duration and treatment. A statistical analysis was performed; Kendall Tau coefficient, chi-square test, t-test, and multiple regression analysis ( P Gingivitis occurred more frequently in patients with NS/diabetes. Gingivitis severity was correlated with PLI, age, and yeast enzyme activity in NS--to with immunosuppressive treatment with >1 drug, drug doses, treatment duration, lipid disorders, and BMI; in diabetes, with blood glucose and glycated haemoglobin >8%. Poor hygiene control is the main cause of gingivitis. Gingivitis severity is most likely related to age, lipid disorders and increase in body mass. Candida spp., in uncompensated diabetes and in those using immunosuppressive treatment, might intensify plaque-related gingivitis.

  12. Immunoglobulin G and M levels in childhood nephrotic syndrome: two centers Egyptian study.

    Science.gov (United States)

    El Mashad, Ghada Mohamed; El Hady Ibrahim, Soha Abd; Abdelnaby, Sameh Abd Allah

    2017-02-01

    Idiopathic nephrotic syndrome (INS) is the most common glomerular disease in children. Immune cell subsets may play a role in pathogenesis of INS. We aimed to assess immunoglobulin G (IgG) and immunoglobulin M (IgM) levels in children with nephrotic syndrome (NS) to predict prognosis of the disease and response to treatment. This prospective case control study was done in Pediatric Nephrology Units at Minoufia and Benha University Hospitals, during the period from 1st March 2014 to 30th June 2015. Seventy-five children in the active stage of INS and 75 apparently healthy children of matched age and sex were included in this study. Statistical evaluation was performed by SPSS version 18.0 using independent-samples t-test, Chi-square, and Pearson's correlation coefficient (r). Compared with healthy children, IgM level was high, IgG level and IgG/IgM ratio were low (p≤0.05). The IgG level and IgG/IgM ratio decreased more in FRNS than in IFRNS group, and was the lowest in SRNS group. The IgM level increased more in FRNS than in IFRNS group, and was the highest in SRNS group (p<0.05, respectively). Our findings support the idea that IgG level has a prognostic value in NS in children.

  13. Pregnancy in 4 women with childhood-onset steroid-sensitive nephrotic syndrome.

    Science.gov (United States)

    Motoyama, Osamu; Iitaka, Kikuo

    2014-05-01

    Four women with childhood-onset steroid-sensitive nephrotic syndrome (SSNS) had 5 pregnancies. Their age at onset of SSNS was between 4 and 10 years, and age at pregnancy was between 21 and 31 years. Three patients with frequent relapsing nephrotic syndrome (NS) continued to relapse after 20 years of age. Two of them had relapses during 6-32 gestational weeks of pregnancy and were treated with prednisolone (PSL) 10-45 mg/day. One patient delivered a normal baby on 2 pregnancies. Another developed superimposed preeclampsia and her infant showed asymmetrical type of intrauterine growth restriction (IUGR). There was no relapse during pregnancy in 2 patients, including 1 with frequent relapses, who had no relapse over 5 years preceding the pregnancy. In all four patients, normal renal function and complete remission were noted at the last follow-up. Their 5 infants were well at 1-7 years of age. Although hypertension, growth failure of the placenta, and IUGR of the baby may complicate the pregnancy, most pregnancies with SSNS seem to result in normal birth, even when relapses occur during pregnancy and are treated with PSL.

  14. TACROLIMUS DRUG LEVEL AND RESPONSE TO TREATMENT IN IDIOPATHIC CHILDHOOD STEROID RESISTANT NEPHROTIC SYNDROME.

    Science.gov (United States)

    Shah, Syed Sajid Hussain; Hafeez, Farkhanda; Akhtar, Naureen

    2015-01-01

    The management of Steroid Resistant Nephrotic Syndrome (SRNS) is an uphill task for paediatric nephrologists as immunosuppressive agents are the mainstay of treatment in these patients. Tacrolimus is used along with steroids. This study is conducted to see the relationship between the tacrolimus dose, drug level and response in the management of SRNS. This quasi experimental study was conducted at The Children's Hospital Lahore over a period of one year. Patients with SRNS of either sex and 1-10 years of age were included and those with secondary nephrotic syndrome were excluded. Tacrolimus was given at a dose of 0.05-0.1 mg/kg/day in 2 divided doses along with steroids. The follow-up was done for six months with proteinuria monitoring and tacrolimus drug levels done two weeks after initiation of treatment. Out of 42 patients, 27 (64.3%) were males and 15 (35.7%) were females. The most common histological diagnosis observed was mesangio-proliferative glomerulonephritis in 30 (71.4%) patients. The tacrolimus trough level range was 0.5-15.20 ng/ml with a mean value of 4.68 ng/ml ± 2.85. Forty-one (97.6%) children showed complete response to treatment while one patient showed partial response. This study suggests that tacrolimus is an effective drug for treatment of SRNS in paediatric patients and there is no linear relationship between the drug dose, response and drug level.

  15. Clinical significance of determination of changes of plasma vascular endothelial growth factor (VEGF) contents after treatment in pediatric patients with nephrotic syndrome

    International Nuclear Information System (INIS)

    Tu Jingfang

    2004-01-01

    Objective: To investigate the plasma contents of VEGF in pediatric patients with nephrotic syndrome. Methods: Plasma VEGF contents were determined with ELISA in 31 pediatric patients with nephrotic syndrome both before and after treatment as well as in 35 controls. Results: Before treatment, the plasma contents of VEGF in the patients were significantly higher than those in the controls (P<0.01). After one month of treatment, the levels remained significantly higher (P<0.05). Conclusion: Development of nephrotic syndrome in pediatric patients was closely related to the plasma levels of VEGF. (authors)

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

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    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. Long-term outcome of biopsy-proven, frequently relapsing minimal-change nephrotic syndrome in children.

    NARCIS (Netherlands)

    Kyrieleis, H.A.; Lowik, M.M.; Pronk, I.; Cruysberg, J.R.M.; Kremer, J.A.M.; Oyen, W.J.G.; Heuvel, L.P.W.J. van den; Wetzels, J.F.M.; Levtchenko, E.N.

    2009-01-01

    BACKGROUND AND OBJECTIVES: Frequently relapsing and steroid-dependent minimal-change nephrotic syndrome (MCNS) that originates in childhood can persist after puberty in >20% of patients. These patients require immunosuppressive treatment during several decades of their life. We examined long-term

  18. 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

  19. Nephrotic syndrome in The Netherlands: a population-based cohort study and a review of the literature

    NARCIS (Netherlands)

    Bakkali L, el; Rodrigues Pereira, R.; Kuik, D.J.; Ket, J.C.; Wijk, J.A. van

    2011-01-01

    Nephrotic syndrome (NS) is a clinical diagnosis with proteinuria, hypoalbuminaemia and oedema. NS is rare in children, and its incidence in The Netherlands is unknown. The aim of this study was to estimate the incidence of idiopathic NS in the Netherlands. All paediatric patients (age 0-18 years)

  20. Genetic and in vivo determinants of glucocorticoid sensitivity in relation to clinical outcome of childhood nephrotic syndrome

    NARCIS (Netherlands)

    Teeninga, N.; van Holthe, J.E.; van den Akker, E.L.T.; Kersten, M.C.; Boersma, E.; Krabbe, H.G.; Knoers, N.V.A.M.; van der Heijden, A.J.; Koper, J.W.; Nauta, J.

    2014-01-01

    Following initial glucocorticoid treatment, the clinical course in children with nephrotic syndrome is highly variable. Intrinsic sensitivity to glucocorticoids might be a determinant of this variability. Functional polymorphisms of the glucocorticoid receptor gene NR3C1 have been associated with

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

    NARCIS (Netherlands)

    Teeninga, N.; Schreuder, M.F.; Bokenkamp, A.; Waal, HA Delemarre-van d; Wijk, JA van

    2008-01-01

    BACKGROUND: 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 childhood. As it

  2. Comparison of second-line immunosuppressants for childhood refractory nephrotic syndrome: a systematic review and network meta-analysis.

    Science.gov (United States)

    Fu, Hai-Dong; Qian, Gu-Ling; Jiang, Zheng-Yang

    2017-01-01

    Although, most patients respond initially to therapy for nephrotic syndrome, about 70% of patients have a relapse. Currently, there is no consensus about the most appropriate second-line agent in children who continue to suffer a relapse. This network meta-analysis was designed to compare the efficacy and safety of the commonly used immunosuppressive agents in second-line therapeutic agents (ie, cyclophosphamide, cyclosporine, tacrolimus and mycophenolate mofetil) for refractory childhood nephrotic syndrome. MEDLINE, Cochrane, EMBASE and Google Scholar databases were searched until October 17, 2015 using the following search terms: cyclophosphamide, cyclosporine, tacrolimus, mycophenolate mofetil and childhood nephrotic syndrome. Randomized controlled trials, prospective 2-arm studies and cohort studies were included. 7 studies with 391 patients were included. Bayesian network meta-analysis found that treatment with mycophenolate mofetil had the greatest odds of relapse compared with tacrolimus (pooled OR=49.72, 95% credibility interval (CrI) 1.65 to 2483.32), cyclophosphamide (pooled OR=72.05, 95% CrI 1.44 to 13633.33) and cyclosporine (pooled OR=11.42, 95% CrI 1.03 to 131.60). Rank probability analysis found cyclophosphamide was the best treatment with the lowest relapse rate as compared with other treatments (rank probability=0.58), and tacrolimus was ranked as the second best (rank probability=0.38). Our findings support the use of cyclophosphamide and tacrolimus in treating children with relapsing nephrotic syndrome. Copyright © 2016 American Federation for Medical Research.

  3. Evaluation Of Clinical Course, Rirsk Factors For Relapse And Long-Term Outcome Of Children With Primary Nephrotic Syndrome

    Directory of Open Access Journals (Sweden)

    Ataei N

    2003-09-01

    Full Text Available The nephrotic syndrome is the most common chronic renal disease of childhood."nMaterials and Methods: In this study the clinical course, risk factors for relapse and the predictors of long-term outcome of 502 patients (median age 5 yearswith primary nephrotic syndrome were followed for an average of 60 months (3.5 to 240 months from 1981 to 2000."nResults: Among the 502 patients 5 (1% achieved spontaneous remission and 313 children were initial responder. One hundred eighty four patients received at least 1 kidney biopsy (78 prior and 106 after initiation of treatment. Of 104 children with frequently relapsing steroid sensitive and steroid dependent nephrotic syndrome, levamisole induced prolong remission in 33 ( 31.7% of patients. Cyclophosphamid and cyclosporine A induced prolong remission in 49 (50% of 98 and 28 (41.3% of 68 patients respectively. At the time of the final clinical evaluation, 73 patients (14.5% were on remission; 301 (59.9% had relapsing; 43 (8.6% had persistent nephrotic syndrome; 33 (6.6% of patients evolving to end-stage renal disease (ESRD and 6 (1.2% of them with chronic renal failure died (infection and cardio respiratory were the cause of death in 5 and 1 patient respectively. Young age (1-5 y at onset of disease and atopy were identified as an independent risk factors for relapse (P0.05. Patients with steroid dependent nephrotic syndrome (SDNS or MCNS had better response to cyclophosphamide or cyclosporin than children with steroid resistance nephrotic syndrome (SRNS or FSGS (P0.05. Persistent proteinuria, hypertension, microscopic or macroscopic hematuia, glucosuria were associated with progression to chronic renal failure (PO.05."nConclusion: Steroid dependency and histopathology of MCNS in patients with nephrotic syndrome were significantly associated with good long-term prognosis. In contrast persistent proteinuria, histopatholoy of FSGS, hypertension, macroscopic or microscopic hematuria, and glucosoria were

  4. ADVERSE EFFECTS OF LONG-TERM CORTICOSTEROID THERAPY OF NEPHROTIC SYNDROME IN CHILDREN

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    T. L. Nastausheva

    2017-01-01

    Full Text Available Background: Long-term corticosteroid therapy in children leads to plenty of adverse effects with negative influence on health. Objective: analysis of adverse effects of corticosteroids in children with steroid-sensitive  nephrotic syndrome and development of recommendations of their early detection.Methods: A retrospective study is conducted on children with this syndrome aged 3–18 who applied to Voronezh Regional Children’s Clinical Hospital № 1 in 2011–2014.  Complications  of corticosteroid  therapy revealed during clinical examination of children were taken into account. Data on 118 healthy children examined in 2012–2014 were used to calculate integral index.Results: The study analyses treatment results of 18 children who received glucocorticosteroids  during 6 months before hospitalization and 13 children who were withdrawn from glucocorticosteroids  for 6 months or more before hospitalization. Among adverse reactions in group 1 there prevailed overweight/obesity  (78%, reactive pancreatitis (72%, leukemoid reactions (67%, liver damage (61%, Cushingoid syndrome (44%, chronic gastroduodenitis  (33%. Hyperglycemia (11%, hypertension (6% and infection (6% were less common. In group 2 only 2 (15% patients had chronic gastroduodenitis,  other complications were not documented. Indices that change in children with nephrotic syndrome during corticosteroid treatment (body mass index, blood serum glucose and amylase were measured by a single scale using modifications coefficients. Average value of the coefficients is suggested to be a new diagnostic criterion (metabolic reaction index which allows to reveal corticosteroid adverse effects before any clinical manifestations.Conclusion: Most adverse reactions of glucocorticosteroids are short-term and continue after 6 months in a small number of patients.

  5. [Treatment of children with steroid-dependent nephrotic syndrome with rituximab].

    Science.gov (United States)

    Huang, Jianping; Du, Juan; Wang, Shuo; Xiao, Lili; Zhao, Xiaoyan

    2014-07-01

    To investigate the effects of rituximab (RTX) in children with steroid-dependent nephrotic syndrome. Five cases of children with steroid-dependent nephrotic syndrome seen from May 2012 to February 2013 in whom only steroid plus calcineurin inhibitor was effective and the disease recurred on reduction of dose were enrolled into this study, including 3 males and 2 females. Calcineurin inhibitors were stopped and steroids was changed to full dose. After the general condition improved, RTX was given at a dose of 375 mg/m(2), once a week for a total of three times for one course. After urine protein became negative for five days, the dose of steroid was changed to 2 mg/kg every other day, thereafter the dose was reduced by 5 mg per every 2 weeks, until discontinuation. After regular monitoring, when peripheral blood B cells were ≥ 3%, a second RTX was added. Urine protein was negative in 2-7 days in 5 patients after the first RTX treatment. Before treatment B lymphocytes in peripheral blood was 7.8% to 13.0% and after the first course of RTX treatment decreased to 0 in the first 6 to 8 months at the beginning of recovery, while in the first 7 to 10 months to 3.3%-6.1%, after a second RTX was given, B lymphocytes were reduced to 0, but in two cases (cases 1 and 3) B lymphocytes rose again at 16 and 17 months, in the first 17 and 18 months rose to 4.16% and 4.17%, RTX was given once again respectively. B lymphocytes were reduced to 0 again. Currently the 5 patients continued to be negative for urine protein, maintaining remission for 12 to 20 months.RTX infusion had no significant side effects, and side effects of steroid and calcineurin inhibitor disappeared. In children with steroid-dependent and only calcineurin inhibitor effective nephritic syndrome, relapse may still occur after improvement of nephrotic syndrome, after the first RTX treatment, regular monitoring of B lymphocytes, RTX supplementary treatment in advance can help discontinuation of steroids and

  6. 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

  7. A Case of Compartment Syndrome in the Scrotum and Inguinal Area Complicating Idiopathic Nephrotic Syndrome of Childhood.

    Science.gov (United States)

    Saqan, Rola S; Alazab, Rami S; Allouh, Mohammed

    2017-11-01

    Idiopathic nephrotic syndrome (INS) is a kidney disease characterized by massive proteinuria. Protein loss leads to decreased oncotic pressure shifting the fluids into the interstitial space causing edema, complications such as infections and thromboembolism occur. We report a 7-year-old, diagnosed with NS presenting with a relapse. He developed ascites and scrotal edema followed by severe scrotal pain and redness, progressing rapidly to ecchymosis in the inguinal areas not in continuity with the scrotum. Ultrasound with color Doppler was inconclusive, scrotal exploration was done along with skin incision in the inguinal areas and scrotum, which appeared to relieve the condition rapidly.

  8. Pharmacokinetics of once daily prolonged-release formulation of tacrolimus in children with primary nephrotic syndrome.

    Science.gov (United States)

    Han, Y; DU, S Q; Xiao, H J; Zhou, Y; Ding, J; Ding, J J; Cui, Y M

    2017-10-18

    Tacrolimus prolonged-release(PR) formulation is a new once-daily formulation of the calcineurin inhibitor tacrolimus, which is currently used in adult liver or kidney transplant patients,and is also gradually widely used in children with nephrotic syndrome.The present study was undertaken to preliminarily investigate the pharmacokinetic characteristics of tacrolimus PR in pediatric nephrotic syndrome recipients. This single-center open-label prospective study was performed in pediatric nephrotic syndrome recipients. Pharmacokinetic samples were collected from eight pediatric subjects with nephrotic syndrome from Department of Pediatric Nephrology in Peking University First Hospital between June and August 2011. They followed administration of single oral doses of tacrolimus PR formulation at 0.02 mg/kg (n=2), 0.05 mg/kg (n=2) and 0.10 mg/kg (n=4). Blood samples were taken before the dose and 1, 2, 4, 6, 8, 10, 12 and 24 h after drug intake. No other medicines or interacting food or drinks were taken during the study period. Blood concentrations were measured using an enzyme multiplied immunoassay technique. Pharmacokinetic analysis was performed using WinNolin Phoenix software Version 6.0(Pharsight, Cary, NC,USA). The pharmacokinetic data were best described by a non-compartment model. Pharmacokinetic parameters of tacrolimus PR formulation in the 3 ascending doses groups (0.02 mg/kg,0.05 mg/kg and 0.10 mg/kg) were as follows: the maximum drug concentrations (C max /D) were (1.7±1.0) μg/L, (3.1±1.9) μg/L, (8.0±3.5) μg/L, respectively; Areas under the drug concentration-time curve(AUC 0-∞ /D) were (47.2±47.1) h×μg/L, (84.0±13.1) h×μg/L, (175.6±107.1) h×μg/L, respectively; Oral clearance rates were (0.8±0.9) L/(h×kg), (0.4±0.1) L/(h×kg), (1.9±1.3) L/(h×kg), respectively; Body weight normalized distribution volumes were (7.0±3.4) L/kg, (12.4±8.4) L/kg and (73.6±68.6) L/kg, respectively. Both mean C max normalized level for the administered

  9. 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.

  10. HLA-DR, -DQB typing of steroid-sensitive idiopathic nephrotic syndrome children in Taiwan.

    Science.gov (United States)

    Huang, Yuan-Yuan; Lin, Fang-Ju; Fu, Lin-Shien; Lan, Joung-Liang

    2009-01-01

    The association between human leukocyte antigens (HLA) and idiopathic nephrotic syndrome (INS) in children has been discussed in various studies. In this study, 59 Chinese children with steroid-sensitive nephritic syndrome (SSNS) in Taiwan were enrolled, and 33 patients underwent renal biopsy. The frequency of HLA-DR11 was found to be significantly higher and HLA-DR14 was lower in SSNS patients as compared with the healthy control group. In frequent relapsers, HLA-DR4 was more frequent, while HLA-DQB1*0602 was less frequent, as compared with infrequent relapsers. However, there was no significant difference between steroid-dependent and non-steroid-dependent patients. In patients who showed a response to levamisole, the HLA-DR9 and -DQB1*0303 alleles were more frequent, while HLA-DR13 was less frequent. In a comparison of HLA in different INS pathologies, HLA-DQB1*0401 was significantly frequent in IgM nephropathy. Even though we compared each group with the control group separately, the HLA distributions of both groups still differed from each other. Our data suggest that the immunogenic characteristics of children with SSNS in Taiwan are different from those of other populations. The common motif between different HLA alleles and the three-dimensional crystal structures may offer an explanation for the varying results from different populations. According to HLA typing, the results support the hypothesis that IgM nephropathy is a different entity from minimal change nephrotic syndrome. Copyright (c) 2009 S. Karger AG, Basel.

  11. Acquired Resistance to Corticotropin Therapy in Nephrotic Syndrome: Role of De Novo Neutralizing Antibody.

    Science.gov (United States)

    Wang, Pei; Zhang, Yan; Wang, Yu; Brem, Andrew S; Liu, Zhangsuo; Gong, Rujun

    2017-07-01

    There is increasing evidence supporting the use of corticotropin as an alternative treatment of refractory proteinuric glomerulopathies. The efficacy of short-acting corticotropin, however, remains unknown and was tested here in an adolescent with steroid-dependent nephrotic syndrome caused by minimal change disease. After developing Cushing syndrome and recently being afflicted with severe cellulitis, the patient was weaned off all immunosuppressants, including corticosteroids. This resulted in a relapse of generalized anasarca, associated with massive proteinuria and hypoalbuminemia. Subsequently, mono-therapy with short-acting animal-derived natural corticotropin was initiated and resulted in a rapid response, marked by substantial diuresis, reduction in body weight, and partial remission of proteinuria. Ten days later, the patient developed mild skin rash and subcutaneous nodules at injection sites. A relapse followed despite doubling the dose of corticotropin, consistent with delayed-onset resistance to treatment. Immunoblot-based antibody assay revealed de novo formation of antibodies in the patient's serum that were reactive to the natural corticotropin. In cultured melanoma cells known to express abundant melanocortin receptors, addition of the patient's serum strikingly mitigated dendritogenesis and cell signaling triggered by natural corticotropin, denoting neutralizing properties of the newly formed antibodies. Collectively, short-acting natural corticotropin seems effective in steroid-dependent nephrotic syndrome. De novo formation of neutralizing antibodies is likely responsible for acquired resistance to corticotropin therapy. The proof of concept protocols established in this study to examine the anticorticotropin neutralizing antibodies may aid in determining the cause of resistance to corticotropin therapy in future studies. Copyright © 2017 by the American Academy of Pediatrics.

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

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    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.

  13. 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

    (p = 0.048). CONCLUSION: The vitamin D status was insufficient in 93% of the patients. We suggest that vitamin D status in children with NS be measured routinely at the time of diagnosis so that an individual treatment strategy for vitamin D defi-ciency can be given. Further studies are needed......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...

  14. 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

    (p = 0.048). CONCLUSION: The vitamin D status was insufficient in 93% of the patients. We suggest that vitamin D status in children with NS be measured routinely at the time of diagnosis so that an individual treatment strategy for vitamin D deficiency can be given. Further studies are needed......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...

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

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    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) 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

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

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    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.

  17. 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

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

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    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.

  19. Effect of cesarean section on relapse of childhood idiopathic nephrotic syndrome.

    Science.gov (United States)

    Kimata, Takahisa; Kino, Jiro; Yamanouchi, Sohsaku; Suruda, Chikushi; Tsuji, Shoji; Kaneko, Kazunari

    2017-10-01

    In parallel with the increase in the prevalence of childhood chronic diseases, the rate of cesarean delivery has risen during the past decades. This study tested the hypothesis that children delivered by cesarean section (CS) have a higher risk of relapse of idiopathic nephrotic syndrome (INS). Fifty-six children with INS were categorized into three groups. Group A consisted of patients with INS who had no relapses after the onset of INS; group B consisted of patients with INS who had infrequent relapse; and group C consisted of patients with INS who had frequent relapse. The number of enrolled patients in groups A, B, and C was 10, 14, and 32, respectively. The ratio of neonates delivered via CS was significantly higher in group C (37.5%, P childhood INS. © 2017 Japan Pediatric Society.

  20. Immunosupressive therapy in children with steroid-resistant nephrotic syndrome: single center experience.

    Science.gov (United States)

    Echeverri, Catalina Velez; Valencia, Gustavo Adolfo Zuluaga; Higuita, Lina Maria Serna; Gayubo, Ana Katherina Serrano; Ochoa, Carolina Lucia; Rosas, Luisa Fernanda Rojas; Muñoz, Laura Carolina; Sierra, Javier; Zuleta, Jhon Jairo; Ruiz, Juan José Vanegas

    2013-01-01

    [corrected] Nephrotic syndrome is one of the most frequent glomerular diseases among children, and steroid therapy remains as the treatment choice. In spite of this, 10 to 15% of the patients are steroidresistant, and the best therapy for such cases has never been defined. Mycophenolate acid (MA) is one of the treatments used in such situations. To describe the clinical behavior of children diagnosed with steroid-resistant nephrotic syndrome (SRNS) and to assess the therapeutic response to MA. This was a retrospective and descriptive study. 26 clinical records of patients with SRNS; 70% male and 30% female. All patients underwent kidney biopsies, which showed a predominance of focal segmental glomerulosclerosis (FSGS). The immunosuppresive drugs used were: Mycophenolate mofetil (MMF) 100%, Cyclosporine 69.2%, Cyclophosphamide 23.1%, and Rituximab 23%. One month after treatment initiation with MMF 61.5% achieved remission. The median of relapses per year for the patients was 3 (p25: 2.75 - p75: 4). This median became 1 (p25: 1 - p75: 3.25) after using this medication (p = 0.08). Furthermore, prior to the start of the MMF treatment, the median of the steroid dose was 1 (p25: 0.5- p75: 1.62) mg/k/day. After using MMF, this median became 0.07 (p25: 0 - p75: 0.55) mg/k/day (p < 0.001), in 8 patients prednisolone was stopped. In our experience, treatment with MMF showed positive results such as decrease in the frequency of relapses, less proteinuria, and reduction in the dose of steroids administered without deterioration of glomerular filtration rates. However, more studies are needed to assess efficacy, safety, and optimal dosage.

  1. Immunosupressive therapy in children with steroid-resistant nephrotic syndrome: single center experience

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    Catalina Velez Echeverri

    2013-09-01

    Full Text Available INTODUCTION: Nephrotic syndrome is one of the most frequent glomerular diseases among children, and steroid therapy remains as the treatment choice. In spite of this, 10 to 15% of the patients are steroidresistant, and the best therapy for such cases has never been defined. Mycophenolate acid (MA is one of the treatments used in such situations. OBJECTIVE: To describe the clinical behavior of children diagnosed with steroid-resistant nephrotic syndrome (SRNS and to assess the therapeutic response to MA. METHODS: This was a retrospective and descriptive study. RESULTS: 26 clinical records of patients with SRNS; 70% male and 30% female. All patients underwent kidney biopsies, which showed a predominance of focal segmental glomerulosclerosis (FSGS. The immunosuppresive drugs used were: Mycophenolate mofetil (MMF 100%, Cyclosporine 69.2%, Cyclophosphamide 23.1%, and Rituximab 23%. One month after treatment initiation with MMF 61.5% achieved remission. The median of relapses per year for the patients was 3 (p25: 2.75 - p75: 4. This median became 1 (p25: 1 - p75: 3.25 after using this medication (p = 0.08. Furthermore, prior to the start of the MMF treatment, the median of the steroid dose was 1 (p25: 0.5- p75: 1.62 mg/k/day. After using MMF, this median became 0.07 (p25: 0 - p75: 0.55 mg/k/day (p < 0.001, in 8 patients prednisolone was stopped. CONCLUSION: In our experience, treatment with MMF showed positive results such as decrease in the frequency of relapses, less proteinuria, and reduction in the dose of steroids administered without deterioration of glomerular filtration rates. However, more studies are needed to assess efficacy, safety, and optimal dosage.

  2. Mutation spectrum of genes associated with steroid-resistant nephrotic syndrome in Chinese children.

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    Wang, Ying; Dang, Xiqiang; He, Qingnan; Zhen, Yan; He, Xiaoxie; Yi, Zhuwen; Zhu, Kuichun

    2017-08-20

    Approximately 20% of children with idiopathic nephrotic syndrome do not respond to steroid therapy. More than 30 genes have been identified as disease-causing genes for the steroid-resistant nephrotic syndrome (SRNS). Few reports were from the Chinese population. The coding regions of genes commonly associated with SRNS were analyzed to characterize the gene mutation spectrum in children with SRNS in central China. The first phase study involved 38 children with five genes (NPHS1, NPHS2, PLCE1, WT1, and TRPC6) by Sanger sequencing. The second phase study involved 33 children with 17 genes by next generation DNA sequencing (NGS. 22 new patients, and 11 patients from first phase study but without positive findings). Overall deleterious or putatively deleterious gene variants were identified in 19 patients (31.7%), including four NPHS1 variants among five patients and three PLCE1 variants among four other patients. Variants in COL4A3, COL4A4, or COL4A5 were found in six patients. Eight novel variants were identified, including two in NPHS1, two in PLCE1, one in NPHS2, LAMB2, COL4A3, and COL4A4, respectively. 55.6% of the children with variants failed to respond to immunosuppressive agent therapy, while the resistance rate in children without variants was 44.4%. Our results show that screening for deleterious variants in some common genes in children clinically suspected with SRNS might be helpful for disease diagnosis as well as prediction of treatment efficacy and prognosis. Copyright © 2017 Elsevier B.V. All rights reserved.

  3. Correlation between lipid profile and C-reactive protein in children with nephrotic syndrome

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    Kurnia Dwi Astuti

    2015-03-01

    Full Text Available Background Nephrotic syndrome (NS causes dyslipidemia in children, which can be long term or intermittent. Dyslipidemia has long been established as a risk factor for atherosclerosis. An early sign of atherosclerosis is elevated high sensitivity C-reactive protien (hsCRP. Atherosclerosis early in life, especially in childhood, warrants an assessment for NS. Study on a correlation between lipid profile and hsCRP, as a marker of atherosclerosis, in pediatric NS patients has been limited. Objective To assess for a correlation between lipid profile and hsCRP in childhood nephrotic syndrome. Methods This cross-sectional study was undertaken on 29 children with NS in Dr. Kariadi Hospital. Serum hsCRP, total cholesterol, low-density lipoprotein (LDL, and high-density lipoprotein (HDL were examined in the active phase. Spearman’s test was used to analyze a possible correlation between total cholesterol, LDL, HDL and hsCRP levels. Results Mean levels of total cholesterol (454 mg/dL and LDL (288 mg/dL in this study were high, while the HDL level (55 mg/dL was normal, according to US Department of Health and Human Services classifications. The median hsCRP level was 0.33 mg/L and 9 (31% subjects had high hsCRP levels of more than 1 mg/L. There was a positive correlation between LDL level and hsCRP (r=0.423; P<0.05. Conclusions There is a weak positive correlation between LDL and hsCRP levels in children with NS.

  4. Nephrotic Syndrome

    Science.gov (United States)

    ... of thickening membranes within the glomeruli. The exact cause of the thickening isn't known, but it's sometimes associated with other medical conditions, such as hepatitis B, malaria, lupus and cancer. Systemic lupus erythematosus. This chronic ...

  5. Nephrotic Syndrome

    Science.gov (United States)

    ... returning chemicals the body needs (such as sodium, phosphorus, and potassium) back into the bloodstream. The extra water combines with other waste to become urine (pee). The main functional units of the kidneys, ...

  6. Lessons from genetics: is it time to revise the therapeutic approach to children with steroid-resistant nephrotic syndrome?

    Science.gov (United States)

    Becherucci, Francesca; Mazzinghi, Benedetta; Provenzano, Aldesia; Murer, Luisa; Giglio, Sabrina; Romagnani, Paola

    2016-08-01

    Primitive nephrotic syndrome is one of the most common glomerular diseases in childhood and represents the clinical manifestation of various pathologic changes in the kidney. In children, nephrotic syndrome is classified based on the initial response to empiric corticosteroid treatment, which is considered as the best predictor of patients' final outcome. The advent of next-generation sequencing technology showed that genetic alterations in structural genes of the podocyte can be recognized in a significant proportion of not only familial or syndromic patients with steroid-resistant nephrotic syndrome (SRNS), but also of sporadic cases, raising the question of whether it is time to update current protocols of patient care. In this review, we discuss the implications derived from several studies describing a high prevalence in children with SRNS of pathogenic mutations in a group of genes and their unresponsiveness to immunosuppressive therapy. We propose a diagnostic and therapeutic algorithm to reduce the exposure to immunosuppressants in individuals with unresponsive forms of the disease, sparing patients the untoward side effects of prolonged ineffective treatments, and at the same time guaranteeing the optimal immunosuppressive or other new therapy in potentially responsive patients.

  7. Urine Neutrophil Gelatinase Associated Lipocalin to Creatinine Ratio: A Novel Index for Steroid Response in Idiopathic Nephrotic Syndrome.

    Science.gov (United States)

    Nickavar, Azar; Safaeian, Baranak; Sadeghi-Bojd, Simin; Lahouti Harah dashti, Arash

    2016-01-01

    To find the value of urine neutrophil gelatinase associated lipocalin (NGAL) in differentiating steroid response in children with idiopathic nephrotic syndrome (INS). A total of 52 children with INS (n = 27, steroid resistant; n = 25, steroid responsive) aged 1-16 y, along with 18 healthy control children were enrolled in this study. Urine NGAL as well as urine protein, and serum creatinine were analyzed during active phase of INS. Serum creatinine (P 0.032), and urine NGAL/Cr (P 0.001) were significantly higher in steroid resistant than steroid sensitive patients. The optimal cutoff value for urine NGAL/Cr with the highest sensitivity and specificity was 0.46 ng/mg and cut off value of 0.01 and 1.15 ng/mg had maximum sensitivity and specificity, respectively. Urine NGAL/Cr could be considered as a marker of steroid resistance in children with idiopathic nephrotic syndrome.

  8. Steroid-resistant nephrotic syndrome as the initial presentation of nail-patella syndrome: a case of a de novo LMX1B mutation.

    Science.gov (United States)

    Nakata, Tomohiro; Ishida, Ryo; Mihara, Yuu; Fujii, Atsuko; Inoue, Yoshimoto; Kusaba, Tetsuro; Isojima, Tsuyoshi; Harita, Yutaka; Kanda, Chiaki; Kitanaka, Sachiko; Tamagaki, Keiichi

    2017-03-23

    Nail-patella syndrome (NPS) is an autosomal dominant disorder caused by mutations in the LMX1B gene and is characterized by nail dysplasia, skeletal abnormalities, and nephropathy. We herein report a case of steroid-resistant nephrotic syndrome (SRNS) prior to overt orthopedic symptoms in a patient with NPS. A 24-year-old woman presented to our hospital with knee pain. She had poorly developed nails, hypoplastic patellas, dislocation of the elbows, and iliac horns in the pelvis. At the age of 7, she developed nephrotic syndrome and was diagnosed with primary focal segmental glomerulosclerosis by renal biopsy. She received long-term corticosteroid therapy with no obvious response. Her clinical course and orthopedic manifestations indicated NPS, and a genetic analysis showed a de novo mutation in the LMX1B gene (c.819 + 1G > A). Nephropathy in this case was considered to be associated with NPS. Therefore, we discontinued corticosteroids without the exacerbation of nephrotic syndrome. Patients with NPS may develop nephrotic syndrome prior to overt orthopedic symptoms and only show non-specific findings in renal biopsy at an early stage of NPS nephropathy. Hereditary nephrotic syndrome, often presenting as childhood-onset SRNS, may also be difficult to diagnose in patients with the following conditions: renal symptoms prior to overt extrarenal symptoms, de novo mutations, and non-specific findings in renal biopsy. Therefore, in the management of SRNS in children, we need to reconsider the possibility of hereditary diseases such as NPS even without a family history.

  9. 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

  10. Cyclosporine in the treatment of childhood idiopathic steroid resistant nephrotic syndrome: a single centre experience in Nigeria.

    Science.gov (United States)

    Ladapo, Taiwo Augustina; Esezobor, Christopher Imokhuede; Lesi, Foluso Ebunoluwa

    2016-01-01

    Children with steroid resistant nephrotic syndrome usually require treatment with second-line agents and calcineurin inhibitors such as cyclosporine are now recommended as initial therapy. These agents only recently become available in our environment and their impact on care is unknown. We reviewed the short-term treatment outcomes of their use in comparison with previous outcomes. Medical records of children managed for idiopathic steroid resistant nephrotic syndrome over a 5 year period were reviewed. Remission rates and improvement in renal function following use of various agents were compared. Of 103 children with idiopathic nephrotic syndrome, 25(24.3%) were steroid resistant, of whom 17 received additional medications. Full remission rate for cyclosporine was 70% (7/10). Remission rates prior to the availability of cyclosporine were 40% (2/5) for cyclophosphamide and 66% (2/3), (partial remission only) with enalapril, an angiotensin converting enzyme inhibitor used in combination with alternate day prednisolone. One child with cyclophosphamide resistance subsequently achieved remission with cyclosporine. Remission was not related to sex (p=0.96) , age (p=0.54) , serum albumin (p=0.37) or hypertension (p=0.43) but to serum cholesterol (p= 0.02) . The estimated glomerular filteration rate (eGFR) among children treated with cyclosporine ranged from 30-167 ml/min/1.73m 2 as follows: >90 (5); 60-89 (3); 30-59 (2) while the mean pre and post treatment eGFR in those with eGFR nephrotic syndrome.

  11. 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.

  12. Proteomic profiling of nephrotic syndrome in serum using magnetic bead based sample fractionation & MALDI-TOF MS

    Science.gov (United States)

    Sui, Weiguo; Dai, Yong; Zhang, Yue; Chen, Jiejing; Liu, Huaying; Huang, He

    2012-01-01

    Background & objectives: At present, the diagnosis of nephrotic syndrome (NS) requires a renal biopsy which is an invasive procedure. We undertook this pilot study to develop an alternative method and potential new biomarkers for diagnosis, and validated a set of well-integrated tools called ClinProt to investigate serum petidome in NS patients. Methods: The fasting blood samples from 49 patients diagnosed with NS by renal biopsy, including 17 mesangial proliferative glomerulonephritis (MsPGN), 12 minimal change nephrotic syndrome (MCNS), 10 focal segmental glomerulosclerosis (FSGS) and 10 membranous nephropathy (MN), were collected and screened to describe their variability of the serum peptidome. The results in NS group were compared with those in 10 control healthy individuals. Specimens were purified with magnetic beads-based weak cation exchange chromatography and analyzed in a MALDI-TOF MS. Results: The results showed 43, 61, 45 and 19 differential peptide peaks in MsPGN, MCNS, MN and FSGS groups, respectively. A Genetic Algorithm was used to set up the classification models. Cross validation of healthy controls from MsPGN, MCNS, MN and FSGS was 96.18, 100, 98.53 and 94.12 per cent, respectively. The recognition capabilities were 100 per cent. Interpretation & conclusions: Our results showed that proteomic analysis of serum with MALDI-TOF MS is a fast and reproducible approach, which may give an early idea of the pathology of nephrotic syndrome. PMID:22561615

  13. Genetic and in vivo determinants of glucocorticoid sensitivity in relation to clinical outcome of childhood nephrotic syndrome.

    Science.gov (United States)

    Teeninga, Nynke; Kist-van Holthe, Joana E; van den Akker, Erica L T; Kersten, Marie C; Boersma, Eric; Krabbe, Hans G; Knoers, Nine V A M; van der Heijden, Albert J; Koper, Jan W; Nauta, Jeroen

    2014-06-01

    Following initial glucocorticoid treatment, the clinical course in children with nephrotic syndrome is highly variable. Intrinsic sensitivity to glucocorticoids might be a determinant of this variability. Functional polymorphisms of the glucocorticoid receptor gene NR3C1 have been associated with either relatively impaired (GR-9β) or increased (BclI) glucocorticoid sensitivity. Here, in a prospective, well-defined cohort of children with nephrotic syndrome, we evaluated both carriage of GR-9β+TthIII-1 and BclI haplotypes in 113 children and a dexamethasone suppression test in 90 children in relation to their clinical outcome over a median follow-up of 4.4 years. Carriers of GR-9β+TthIII-1 had a significantly higher incidence of steroid dependence 13/25 (52%) compared with noncarriers 19/75 (25%) with a hazard ratio adjusted for gender, age, and descent of 3.04 with 95% confidence interval 1.37-6.74. Both first and frequent relapses happened significantly more often in GR-9β+TthIII-1 carriers than in noncarriers. There were no significant differences in therapeutic outcomes between carriers and noncarriers of the BclI haplotype. Results of the dexamethasone test showed no associations with clinical outcome. Thus, the GR-9β+TthIII-1 haplotype of the glucocorticoid receptor gene offers new insights into the clinical course of children with nephrotic syndrome.

  14. [A prospective, randomized, controlled clinical study of Huai Qi Huang granules in treatment of childhood primary nephrotic syndrome].

    Science.gov (United States)

    Geng, Hai-Yun; Cao, Li; Wang, Wei; Chen, Da-Kun; Chen, Chao-Ying; Chu, Mei; Ji, Li-Na; Wang, Jing-Jing; Tu, Juan; Li, Jian-Guo; Li, Hua-Rong; Du, Pei-Wei

    2015-01-01

    To study the efficacy of Huai Qi Huang granules in the treatment of childhood primary nephrotic syndrome. Between July 2009 and December 2011, patients who were admitted and diagnosed for the first time as childhood primary nephrotic syndrome were randomized into a treatment group (Huai Qi Huang granules plus glucocorticoid; n=23) and a control group (glucocorticoid alone; n=19) for a prospective study. The two groups were compared for regression time of edema, time to urinary protein clearance, relapse rate, incidence of infection, dosage of glucocorticoid, and humoral and cellular immunological indicators. There were no significant differences in regression time of edema, time to urinary protein clearance, and relapse rate between the treatment and control groups (P>0.05). The treatment group had significantly lower incidence of infection and daily dose of glucocorticoid (at month 6) than the control group (P0.05). No Huai Qi Huang-related adverse events were observed in this study. Huai Qi Huang granules treatment can reduce the dose of glucocorticoid and the incidence of infection in children with primary nephrotic syndrome and has a favourable safety.

  15. 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.

  16. Effects of steroids therapy and ACTH therapy on the central nervous system evaluated by CT. Nephrotic syndrome and infantile spasms

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    Xano, E.; Yamashita, F.; Aoki, N.i; Matsuishi, T. (Kurume Univ., Fukuoka (Japan). School of Medicine); Yamamoto, M.

    1981-05-01

    CT scanning revealed cerebral atrophy of various degrees in 12 of 15 cases of nephrotic syndrome in which ACTH therapy was given and also in all the 8 cases of infantile spasms in which ACTH therapy was given. CT findings of cerebral atrophy were analysed by computor and expressed in terms of cerebrospinal fluid (CSF)-space/intracranial space ratio (%). The ratio was 6.6 +- 3.9% in nephrotic syndrome, 16.4 +- 7.8% in infantile spasms, and 1.6 +- 0.8% in control, showing statistically significant differences among them. Abnormal CT findings of 12 cases of nephrotic syndrome returned to normal after withdrawal of steroids in 9 cases (75%). CT findings in 8 cases of infantile spasms also improved in all the cases after withdrawal of ACTH therapy. The results suggested that cerebral atrophy on CT scan is a reversible change. However, the effect of steroids and ACTH on the central nervous system is not sufficiently known yet. We should be very careful about long-term administration of great amount of steroids or ACTH to infants and children.

  17. Role of PCSK9 and IDOL in the pathogenesis of acquired LDL receptor deficiency and hypercholesterolemia in nephrotic syndrome.

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    Liu, Shuman; Vaziri, Nosratola D

    2014-03-01

    Nephrotic syndrome (NS) leads to elevation of serum total and LDL cholesterol. This is largely due to impaired LDL clearance, which is caused by hepatic LDL receptor (LDLR) deficiency despite normal LDLR mRNA expression, pointing to a post-transcriptional process. The mechanism(s) by which NS causes LDLR deficiency is not known. By promoting degradation of LDLR, Proprotein Convertase Subtilisin/Kexin type 9 (PCSK9) and inducible degrader of the LDL receptor (IDOL) play a major role in post-translational regulation of LDLR. We, therefore, tested the hypothesis that LDLR deficiency despite its normal gene expression in NS may be due to upregulation of hepatic PCSK9 and IDOL. LDLR, IDOL and PCSK9 expressions and nuclear translocation of liver X receptor (LXR) that regulates IDOL expression were determined in the liver of rats with puromycin-induced NS and control (CTL) rats. Compared with the CTLs, the NS rats showed marked elevation of serum total and LDL cholesterol and a significant reduction in hepatic LDLR protein expression. This was accompanied by marked upregulation of hepatic PCSK9 and IDOL expressions and heightened LXR activation. LDLR deficiency, hypercholesterolemia and elevated plasma LDL in NS are associated with upregulation of PCSK9 and IDOL. Interventions targeting these pathways may be effective in the management of hypercholesterolemia and the associated cardiovascular and other complications of NS.

  18. The Clinical Course of Minimal Change Nephrotic Syndrome With Onset in Adulthood or Late Adolescence: A Case Series.

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    Maas, Rutger J; Deegens, Jeroen K; Beukhof, Johan R; Reichert, Louis J; Ten Dam, Marc A; Beutler, Jaap J; van den Wall Bake, A Warmold L; Rensma, Pieter L; Konings, Constantijn J; Geerse, Daniel A; Feith, Geert W; Van Kuijk, Willi H; Wetzels, Jack F

    2017-05-01

    Few studies have examined the treatment and outcome of adult-onset minimal change nephrotic syndrome (MCNS). We retrospectively studied 125 patients who had MCNS with onset in either adulthood or late adolescence. Presenting characteristics, duration of initial treatment and response to treatment, relapse patterns, complications, and long-term outcome were studied. Case series. Patients with new-onset nephrotic syndrome 16 years or older and a histologic diagnosis of MCNS in 1985 to 2011 were identified from pathology records of 10 participating centers. Partial and complete remission, treatment resistance, relapse, complications, renal survival. Corticosteroids were given as initial treatment in 105 (84%) patients. After 16 weeks of corticosteroid treatment, 92 (88%) of these patients had reached remission. Median time to remission was 4 (IQR, 2-7) weeks. 7 (6%) patients initially received cyclophosphamide with or without corticosteroids, and all attained remission after a median of 4 (IQR, 3-11) weeks. 13 (10%) patients reached remission without immunosuppressive treatment. One or more relapses were observed in 57 (54%) patients who received initial corticosteroid treatment. Second-line cyclophosphamide resulted in stable remission in 57% of patients with relapsing MCNS. Acute kidney injury was observed in 50 (40%) patients. Recovery of kidney function occurred almost without exception. Arterial or venous thrombosis occurred in 11 (9%) patients. At the last follow-up, 113 (90%) patients were in remission and had preserved kidney function. 3 patients with steroid-resistant MCNS progressed to end-stage renal disease, which was associated with focal segmental glomerulosclerosis lesions on repeat biopsy. Retrospective design, variable treatment protocols. The large majority of patients who had MCNS with onset in adulthood or late adolescence were treated with corticosteroids and reached remission, but many had relapses. Cyclophosphamide resulted in stable remission

  19. Mycophenolate mofetil in treatment of childhood steroid-dependent nephrotic syndrome.

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    Jellouli, Manel; Fitouhi, Sonia; Abidi, Kamel; Hammi, Yousra; Naija, Ouns; Zarrouk, Chokri; Gargah, Tahar

    2016-03-01

    To establish the efficacy of mycophenolate mofetil (MMF) in steroid dependent nephrotic syndrome and to determine the predictive factors for a good response. retrospective hospital-based cohort study in the department of pediatric of Charles Nicolle hospital, between 2005 and 2012 included 30 children with steroid-dependent nephritic syndrome who were treated with MMF. A total of 30 patients (20 boys and 10 girls) were included. The mean age at the time of diagnosis was 5.45 years and treatment with MMF was performed at a mean age of 10.84 years. Side effects of steroid were found in 17 patients. Four patients had renal impairment (ciclotoxicity). The evolution of the disease was 5.45 years. The average rate of relapse was 1.75 relapses / year. The minimum dose of corticosteroids was 0.74 mg / kg / day. During MMF therapy, the average rate of relapse was 0.45 relapses / year (p<0.0001). The average residual steroid dose was 0.2 mg/kg/ day. Responding patients were younger at the onset of MMF (8.57 versus 12.83, p=0.009), had a short development period (3.75 vs 7.03 years, p=0.05), had not received cyclosporine (p=0.02). MMF allows steroid sparing and reduces the number of relapse. It is more effective than the patients are young, with short disease outcome and had not previously been treated with cyclosporine.

  20. An inducible mouse model of podocin-mutation-related nephrotic syndrome.

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    Mansoureh Tabatabaeifar

    Full Text Available Mutations in the NPHS2 gene, encoding podocin, cause hereditary nephrotic syndrome. The most common podocin mutation, R138Q, is associated with early disease onset and rapid progression to end-stage renal disease. Knock-in mice carrying a R140Q mutation, the mouse analogue of human R138Q, show developmental arrest of podocytes and lethal renal failure at neonatal age. Here we created a conditional podocin knock-in model named NPHS2 R140Q/-, using a tamoxifen-inducible Cre recombinase, which permits to study the effects of the mutation in postnatal life. Within the first week of R140Q hemizygosity induction the animals developed proteinuria, which peaked after 4-5 weeks. Subsequently the animals developed progressive renal failure, with a median survival time of 12 (95% CI: 11-13 weeks. Foot process fusion was observed within one week, progressing to severe and global effacement in the course of the disease. The number of podocytes per glomerulus gradually diminished to 18% compared to healthy controls 12-16 weeks after induction. The fraction of segmentally sclerosed glomeruli was 25%, 85% and 97% at 2, 4 and 8 weeks, respectively. Severe tubulointerstitial fibrosis was present at later disease stage and was correlated quantitatively with the level of proteinuria at early disease stages. While R140Q podocin mRNA expression was elevated, protein abundance was reduced by more than 50% within one week following induction. Whereas miRNA21 expression persistently increased during the first 4 weeks, miRNA-193a expression peaked 2 weeks after induction. In conclusion, the inducible R140Q-podocin mouse model is an auspicious model of the most common genetic cause of human nephrotic syndrome, with a spontaneous disease course strongly reminiscent of the human disorder. This model constitutes a valuable tool to test the efficacy of novel pharmacological interventions aimed to improve podocyte function and viability and attenuate proteinuria

  1. [Idiopathic nephrotic syndrome (INS) in children in Dakar: about 40 cases].

    Science.gov (United States)

    Keita, Younoussa; Lemrabott, Ahmed Tall; Sylla, Assane; Niang, Babacar; Ka, El Hadji Fary; Dial, Chérif Mohamed; Ndongo, Aliou Abdoulaye; Sow, Amadou; Moreira, Claude; Niang, Abdou; Ndiaye, Ousmane; Diouf, Boucar; Sall, Mouhamadou Guélaye

    2017-01-01

    This study aimed to analyze the diagnostic, therapeutic, and evolutionary features of nephrosis in children in a pediatric department in Dakar. The study was carried out in the Department of Pediatrics at the Aristide Le Dantec Hospital. We conducted a retrospective study over a period of 3 years from 1 January 2012 to 31 December 2014. All patients aged 2-12 years with idiopathic nephrotic syndrome were included in the study. Forty cases of nephrosis were collected, that is to say a prevalence of 23% among patients with kidney disease treated in the Department of Pediatrics. The average age was 7.11 ± 3.14 years. 72.5% (n=29) of patients suffered from pure nephrotic syndrome. Lower limb edema was present in 100% of patients, oliguria in 55% (n=22) and high blood pressure (HBP) in 5% (n=2) of cases. Median proteinuria was 145,05 ± 85,54 mg/kg/24 hours. Median protidemia was 46,42 ±7.88 g/L and median albumin was 17.90 ± 7.15 g/L. Thirty nine patients were treated with prednisone-based corticosteroid therapy. Corticosensitivity was retained in 77% (n=30) patients and corticoresistance in 13% (n=5) of cases. The factor of poor response after corticosteroid therapy was initial proteinuria greater than 150 mg/kg/day (p = 0.024). Renal biopsy was performed in 18% (n=7) of patients which showed focal and segmental hyalinosis in 57.2% (n=4). Cyclophosphamide and azathioprine were associated with corticosteroids in 10% (n=4) of cases respectively. The overall remission rate was 89.8%. The evolution toward chronic renal failure was observed in three patients. Nephrosis accounted for almost one quarter of all cases of kidney disease treated in our Department. It has high overall remission rate. The only factor contributing to poor response after corticosteroid therapy was high levels of initial proteinuria. Focal and segmental hyalinosis was the most frequently found lesion diagnosed by renal biopsy.

  2. Childhood idiopathic steroid-resistant nephrotic syndrome at a Single Center in Khartoum.

    Science.gov (United States)

    Ali, El-Tigani M A; Makki, Hanna F K; Abdelraheem, Mohamed B; Makke, Salwa O; Allidir, Rashid A

    2017-01-01

    Prevalence, clinicopathological features, and outcome of childhood idiopathic steroid-resistant nephrotic syndrome (ISRNS) vary in different countries. We report on these parameters in a single center in Khartoum. We retrospectively reviewed all the records of children with idiopathic nephrotic syndrome (INS) followed up in the pediatric renal unit, Soba Hospital, Khartoum between 2001 and 2012. ISRNS was defined as no remission within four weeks of daily prednisolone at a dose of 60 mg/m2. In 430 children with INS 130 (28%) had SRNS with a mean age of 7.7 ± 4.12 years. Males were 78 (60%). At presentation, hematuria was recorded in 57%, hypertension was recorded in 48%, and renal impairment in 15%. Histopathology showed focal segmental glomerulosclerosis in 40.8%, mesangioproliferative glomerulonephritis (22.3%), mesangiocapillary glomerulonephritis (16.9%), minimal change disease (MCD) (16.2%), and membranous glomerulonephritis (3.8%). Therapy included cyclosporine in 38.5%, additional therapy with cyclophosphamide, mycophenolate mofetil or tacrolimus in 20.8%, and steroids ± diuretics ± angiotensin converting enzyme (ACE) inhibitors in 40.7%. After 3.64 ± 2.84 years, 26.8% had complete remission (CR), 18.6% partial remission (PR), 26.8% were unremitting, 21.5% had chronic kidney disease (CKD), 1.6% died, and 4.6% were lost to follow-up. Non-MCD cases had significantly lower CR and higher CKD rates than MCD (P = 0.047 and 0.041, respectively). Cyclosporine ± additional therapy was significantly associated with higher rate of CR than steroids ± ACE inhibitors ± diuretics (P = 0.001), but the prevalence of CKD between the two groups was not significantly different (P = 0.604). Impaired renal function and hypertension at presentation were risk factors for CKD (P = 0.001 and 0.001, respectively). In Sudanese children with ISRNS, non-MCD lesions were the most common lesions. This pattern in addition to the lack of adequate therapy may explain the relatively

  3. Childhood idiopathic steroid-resistant nephrotic syndrome at a Single Center in Khartoum

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    El-Tigani M. A Ali

    2017-01-01

    Full Text Available Prevalence, clinicopathological features, and outcome of childhood idiopathic steroid-resistant nephrotic syndrome (ISRNS vary in different countries. We report on these parameters in a single center in Khartoum. We retrospectively reviewed all the records of children with idiopathic nephrotic syndrome (INS followed up in the pediatric renal unit, Soba Hospital, Khartoum between 2001 and 2012. ISRNS was defined as no remission within four weeks of daily prednisolone at a dose of 60 mg/m2. In 430 children with INS 130 (28% had SRNS with a mean age of 7.7 ± 4.12 years. Males were 78 (60%. At presentation, hematuria was recorded in 57%, hypertension was recorded in 48%, and renal impairment in 15%. Histopathology showed focal segmental glomerulosclerosis in 40.8%, mesangioproliferative glomerulonephritis (22.3%, mesangiocapillary glomerulonephritis (16.9%, minimal change disease (MCD (16.2%, and membranous glomerulonephritis (3.8%. Therapy included cyclosporine in 38.5%, additional therapy with cyclophosphamide, mycophenolate mofetil or tacrolimus in 20.8%, and steroids ± diuretics ± angiotensin converting enzyme (ACE inhibitors in 40.7%. After 3.64 ± 2.84 years, 26.8% had complete remission (CR, 18.6% partial remission (PR, 26.8% were unremitting, 21.5% had chronic kidney disease (CKD, 1.6% died, and 4.6% were lost to follow-up. Non-MCD cases had significantly lower CR and higher CKD rates than MCD (P = 0.047 and 0.041, respectively. Cyclosporine ± additional therapy was significantly associated with higher rate of CR than steroids ± ACE inhibitors ± diuretics (P = 0.001, but the prevalence of CKD between the two groups was not significantly different (P = 0.604. Impaired renal function and hypertension at presentation were risk factors for CKD (P = 0.001 and 0.001, respectively. In Sudanese children with ISRNS, non-MCD lesions were the most common lesions. This pattern in addition to the lack of adequate therapy may explain the relatively

  4. Biallelic Mutations in Nuclear Pore Complex Subunit NUP107 Cause Early-Childhood-Onset Steroid-Resistant Nephrotic Syndrome.

    Science.gov (United States)

    Miyake, Noriko; Tsukaguchi, Hiroyasu; Koshimizu, Eriko; Shono, Akemi; Matsunaga, Satoko; Shiina, Masaaki; Mimura, Yasuhiro; Imamura, Shintaro; Hirose, Tomonori; Okudela, Koji; Nozu, Kandai; Akioka, Yuko; Hattori, Motoshi; Yoshikawa, Norishige; Kitamura, Akiko; Cheong, Hae Il; Kagami, Shoji; Yamashita, Michiaki; Fujita, Atsushi; Miyatake, Satoko; Tsurusaki, Yoshinori; Nakashima, Mitsuko; Saitsu, Hirotomo; Ohashi, Kenichi; Imamoto, Naoko; Ryo, Akihide; Ogata, Kazuhiro; Iijima, Kazumoto; Matsumoto, Naomichi

    2015-10-01

    The nuclear pore complex (NPC) is a huge protein complex embedded in the nuclear envelope. It has central functions in nucleocytoplasmic transport, nuclear framework, and gene regulation. Nucleoporin 107 kDa (NUP107) is a component of the NPC central scaffold and is an essential protein in all eukaryotic cells. Here, we report on biallelic NUP107 mutations in nine affected individuals who are from five unrelated families and show early-onset steroid-resistant nephrotic syndrome (SRNS). These individuals have pathologically focal segmental glomerulosclerosis, a condition that leads to end-stage renal disease with high frequency. NUP107 is ubiquitously expressed, including in glomerular podocytes. Three of four NUP107 mutations detected in the affected individuals hamper NUP107 binding to NUP133 (nucleoporin 133 kDa) and NUP107 incorporation into NPCs in vitro. Zebrafish with nup107 knockdown generated by morpholino oligonucleotides displayed hypoplastic glomerulus structures and abnormal podocyte foot processes, thereby mimicking the pathological changes seen in the kidneys of the SRNS individuals with NUP107 mutations. Considering the unique properties of the podocyte (highly differentiated foot-process architecture and slit membrane and the inability to regenerate), we propose a "podocyte-injury model" as the pathomechanism for SRNS due to biallelic NUP107 mutations. Copyright © 2015 The American Society of Human Genetics. Published by Elsevier Inc. All rights reserved.

  5. Urinary Plasmin Inhibits TRPV5 in Nephrotic-Range Proteinuria

    NARCIS (Netherlands)

    Tudpor, Kukiat; Lainez, Sergio; Kwakernaak, Arjan J.; Kovalevskaya, Nadezda V.; Verkaart, Sjoerd; van Genesen, Siebe; van der Kemp, Annemiete; Navis, Gerjan; Bindels, Rene J. M.; Hoenderop, Joost G. J.

    2012-01-01

    Urinary proteins that leak through the abnormal glomerulus in nephrotic syndrome may affect tubular transport by interacting with membrane transporters on the luminal side of tubular epithelial cells. Patients with nephrotic syndrome can develop nephrocalcinosis, which animal models suggest may

  6. 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.

  7. [Dermatoglyphics differences among children with nephrotic syndrome according to steroid response].

    Science.gov (United States)

    Corona-Rivera, Jorge Román; Pérez-Cortés, Gustavo; Osuna-Osuna, Julieta; Garay-Cortés, Marcela Guadalupe; Pérez-Molina, J Jesús; Ramírez-Godínez, Santa; Peña-Padilla, Christian; Rivera-Vargas, Jehú; Bobadilla-Morales, Lucina

    2016-01-01

    Although the association between the type of idiopathic nephrotic syndrome (INS) and a peculiar pattern of fingerprints digital would suggest the presence of genetic factors related to both, this has not been previously studied. This study aimed to evaluate if there are fingerprints patterns differences between children with steroid-resistant INS (SRNS) and those with steroid-sensitive INS (SSNS). The frequencies distribution of arches, ulnar loops, radial loops, and whorls was studied in 60 children with SRNS, and 60 children with SSNS. Bivariate analysis to detect the relationship between each fingerprint pattern with the study groups was performed by chi-square test and to evaluate its possible association, the odds ratios (OR) were calculated with 95% confidence's intervals (95%CI). The patients with SRNS had a higher frequency of digital whorls compared with that of patients with SSNS (46.7% vs. 30.7%, p = 0.005). Additional comparisons using a "whorls excesses" definition obtained from normative data in our population (≥ 7 whorls in females or ≥ 8 in males) were associated with increased odds for SRNS (OR 2.96, 95% CI 1.15-7.61). Our findings indicate that there are differences between children with SRNS and SSNS at the level of digital dermatoglyphics, but further studies are needed to confirm this association and its possible implications.

  8. Pioglitazone Enhances the Beneficial Effects of Glucocorticoids in Experimental Nephrotic Syndrome.

    Science.gov (United States)

    Agrawal, S; Chanley, M A; Westbrook, D; Nie, X; Kitao, T; Guess, A J; Benndorf, R; Hidalgo, G; Smoyer, W E

    2016-05-04

    Glucocorticoids are the primary therapy for nephrotic syndrome (NS), but have serious side effects and are ineffective in ~20-50% of patients. Thiazolidinediones have recently been suggested to be renoprotective, and to modulate podocyte glucocorticoid-mediated nuclear receptor signaling. We hypothesized that thiazolidinediones could enhance glucocorticoid efficacy in NS. We found that puromycin aminonucleoside-induced proteinuria in rats was significantly reduced by both high-dose glucocorticoids (79%) and pioglitazone (61%), but not low-dose glucocorticoids (25%). Remarkably, pioglitazone + low-dose glucocorticoids also reduced proteinuria (63%) comparably to high-dose glucocorticoids, whereas pioglitazone + high-dose glucocorticoids reduced proteinuria to almost control levels (97%). Molecular analysis revealed that both glucocorticoids and pioglitazone enhanced glomerular synaptopodin and nephrin expression, and reduced COX-2 expression, after injury. Furthermore, the glomerular phosphorylation of glucocorticoid receptor and Akt, but not PPARγ, correlated with treatment-induced reductions in proteinuria. Notably, clinical translation of these findings to a child with refractory NS by the addition of pioglitazone to the treatment correlated with marked reductions in both proteinuria (80%) and overall immunosuppression (64%). These findings together suggest that repurposing pioglitazone could potentially enhance the proteinuria-reducing effects of glucocorticoids during NS treatment.

  9. Nephrotic syndrome induced by dibasic sodium phosphate injections for twenty-eight days in rats.

    Science.gov (United States)

    Tsuchiya, Noriko; Torii, Mikinori; Narama, Isao; Matsui, Takane

    2009-04-01

    Sprague-Dawley rats received once daily tail-vein injections of 360 mM dibasic sodium phosphate solution at 8 mL/kg for fourteen or twenty-eight days. Clinical examination revealed persistent proteinuria from three days after the first dosing and thereafter severe proteinuria from eight days or later in the phosphate-treated groups. Proteinuria developed without remission even after fourteen-day withdrawal in the fourteen-day dosed group. Phosphate-treated animals developed lipemia, hypercholesterolemia, anemia, higher serum fibrinogen levels, and lower serum albumin/globulin ratios on day 29. Renal weight increased significantly compared with control animals, and the kidneys appeared pale and enlarged with a rough surface. Histopathologically, glomerular changes consisted of mineralization in whole glomeruli, glomerular capillary dilatation, partial adhesion of glomerular tufts to Bowman's capsule, and mesangiolysis. Ultrastructural lesions such as an increased number of microvilli, effacement of foot processes, and thickening of the glomerular basement membrane, and immunocytochemical changes in podocytes, mainly decreased podoplanin-positive cells and increased desmin expression, were also conspicuous in the phosphate-treated rats for twenty-eight days. Marked tubulointerstitial lesions were tubular regeneration and dilatation, protein casts, mineralization in the basement membrane, focal interstitial inflammation, and fibrosis in the cortex. These clinical and morphological changes were similar to features of human nephrotic syndrome.

  10. Non-Autoimmune Subclinical and Overt Hypothyroidism in Idiopathic Steroid-resistant Nephrotic Syndrome in Children.

    Science.gov (United States)

    Marimuthu, Vidhya; Krishnamurthy, Sriram; Rajappa, Medha

    2017-11-15

    To evaluate the frequency of non-autoimmune subclinical and overt hypothyroidism in children with idiopathic steroid-resistant nephrotic syndrome (SRNS). This cross-sectional study recruited 30 children (age 1-18 y) with idiopathic SRNS; and 30 healthy controls. Serum T3, T4 and TSH were performed in cases as well as controls. Anti-thyroid peroxidase and anti-thyroglobulin antibody tests were performed in all cases. Non-autoimmune subclinical or overt hypothyroidism was detected in 10 out of 30 children with idiopathic SRNS; 2 had overt hypothyroidism, while 8 patients had subclinical hypothyroidism. Children with SRNS had a mean (SD) TSH value 4.55 (4.64) mIU/L that was higher as compared to controls (1.88 (1.04) mIU/L) (Phypothyroidism (2 cases) and grade III subclinical hypothyroidism (1 case) were subsequently started on levothyroxine therapy. The prevalence of subclinical and overt hypothyroidism seems to be high in idiopathic SRNS, with almost one-third of children having overt or subclinical non-autoimmune hypothyroidism.

  11. Substantial practice variation exists in the management of childhood nephrotic syndrome.

    Science.gov (United States)

    Samuel, Susan; Morgan, Catherine J; Bitzan, Martin; Mammen, Cherry; Dart, Allison B; Manns, Braden J; Alexander, R Todd; Erickson, Robin L; Grisaru, Silviu; Wade, Andrew W; Blydt-Hansen, Tom; Feber, Janusz; Arora, Steven; Licht, Christoph; Zappitelli, Michael

    2013-12-01

    Practice variation is common for nephrotic syndrome (NS) treatment. A cross-sectional, web-based survey on NS treatment was administered to 58 Canadian pediatric nephrologists with the aim to document existing practice variation and compare practice with the recommendations of the Kidney Disease Improving Global Outcomes Clinical Practice Guideline for NS. Of the 58 nephrologists asked to participate in the survey, 40 (69 %) responded. Among these, 62 % prescribed initial daily glucocorticoid (GC) therapy for 6 weeks, 26 % for 4 weeks by 26 %, and 10 % prescribed 'other'. Alternate-day GC was continued for 6 weeks by 63 % of respondents and for >6 and <6 weeks by 32 and 6 %, respectively. For biopsy-confirmed minimal change disease, 65 and 46 % of respondents chose oral cyclophosphamide for frequently relapsing and steroid-dependent phenotypes, respectively; calcineurin inhibitors or mycophenolate were the second most popular choices. Kidney biopsy was 'always' performed by 16, 39, and 97 % of respondents for frequently relapsing, steroid-dependent, and steroid-resistant patients, respectively. Rituximab had been administered by 60 % of respondents; 22, 56, and 72 % reported that they would consider rituximab for frequently relapsing, steroid-dependent, and steroid-resistant patients, respectively. Most notable differences between practice and Guideline recommendations were first presentation GC duration, GC-sparing agent choices in frequently relapsing and steroid-dependent patients, and biopsy practices. There is substantial Canadian practice variation in NS treatment. Assessment of factors driving variation and strategies to implement Guideline recommendations are needed.

  12. Childhood Nephrotic Syndrome Management and Outcome: A Single Center Retrospective Analysis

    Directory of Open Access Journals (Sweden)

    Chia-shi Wang

    2017-01-01

    Full Text Available There is a paucity of information on outpatient management and risk factors for hospitalization and complications in childhood nephrotic syndrome (NS. We described the management, patient adherence, and inpatient and outpatient usage of 87 pediatric NS patients diagnosed between 2006 and 2012 in the Atlanta Metropolitan Statistical Area. Multivariable analyses were performed to examine the associations between patient characteristics and disease outcome. We found that 51% of the patients were treated with two or more immunosuppressants. Approximately half of the patients were noted to be nonadherent to medications and urine protein monitoring. The majority (71% of patients were hospitalized at least once, with a median rate of 0.5 hospitalizations per patient year. Mean hospital length of stay was 4.0 (3.8 days. Fourteen percent of patients experienced at least one serious disease complication. Black race, frequently relapsing/steroid-dependent and steroid-resistant disease, and the first year following diagnosis were associated with higher hospitalization rates. The presence of comorbidities was associated with longer hospital length of stay and increased risk of serious disease complications. Our results highlight the high morbidity and burden of NS and point to particular patient subgroups that may be at increased risk for poor outcome.

  13. Patterns of Childhood Steroid-Sensitive and Steroid-Resistant Nephrotic Syndrome in Saudi Children.

    Science.gov (United States)

    Alharthi, Abdulla A

    2017-02-01

    Nephrotic syndrome (NS) is one of the most common causes of chronic kidney disease in children. Mostly, NS is controlled by steroids. In spite of this, 10% to 20% of the patients have steroid resistant NS (SRNS), and the rest have steroid sensitive NS (SSNS). Eighty-seven children with NS (66 SSNS; 21SRNS) were retrospectively studied within the past 10 years in Taif region of Saudi Arabia. Regarding outcome, 66 (76%) patient responded to the steroid therapy, while 21 (24%) patients characterize as SRNS. Out of 66 SSNS, 25 children revealed complete remission, 26 were diagnosed as steroid dependent, and 15 were identified as frequent relapses. Among 21 SRNS biopsied patients, focal segmental glomerulosclerosis was the most common histological patterns of NS followed by the membranoproliferative glomerulonephritis pattern, which is significantly different from other studies performed in the Kingdom of Saudi Arabia. Out of 21 SRNS patients, 13 developed chronic kidney disease, 4 got end-stage renal disease, and 4 patients died.

  14. Nephrotic Syndrome Complicated by Life-Threatening Pulmonary Embolism in an Adult Patient

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    Szu-Chia Chen

    2010-02-01

    Full Text Available We report a 23-year-old male presenting with edema. He was originally admitted for an elective renal biopsy for diagnosis of renal pathology. Unfortunately, because of acute abdominal pain an exploratory laparotomy was done. Progressive azotemia and oliguria then developed, and he required temporary hemodialysis. However, he suffered from sudden-onset severe respiratory distress, and blood gas analysis showed profound hypoxemia with a marked arterial–alveolar oxygen difference. Assessment of a pulmonary embolism by radioisotope imaging was not possible because of his dependence on mechanical ventilation. Subcutaneous low molecular weight heparin and intravenous methylprednisolone were given to treat the presumed pulmonary embolism and the underlying nephrotic syndrome. His partial oxygen level gradually increased after continuous heparin and steroid administration. Complete obliteration of one major pulmonary artery and partial obliteration of other smaller arteries were revealed by magnetic resonance angiography. He was discharged and followed-up as an outpatient, and was given oral warfarin and prednisolone. Follow-up magnetic resonance angiography 5 months later showed a normal pulmonary tree with no residual lesions.

  15. Genotyping Rs2274625 Marker in NPHS2 Gene Associated with Nephrotic Syndrome in Isfahan Population

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    L Esmaili Chamgordani

    2015-12-01

    Full Text Available Introduction: Nephrotic syndrome (NS is a genetic disease belonging to a heterogeneous group of glomerular disorders, which mainly occurs within the children. Linkage analysis using single nucleotide polymorphisms (SNP is used as an indirect method in molecular diagnosis of the disease. A large number of SNP markers have been introduced in NPHS2gene in the available electronic databases. Method: In the present study, the genotype and informative status of rs2274625 marker in NPHS2 genewas investigated in 120 unrelated healthy individuals using Tetra-primer ARMS PCR technique and newly designed primers. Allelic frequency and presence of Hardy Weinberg Equilibrium (HWE was estimated using GenePop website. Furthermore, PowerMarker software was utilized in order to compute the index of polymorphism information content (PIC. Results: The study results indicated allele frequency of 97% and 3% for C and T alleles, respectively, in regard with rs2274625 marker within Isfahan population. Moreover, the PIC for the rs2274625 marker was 0.5%, and HWE revealed the equilibruim of the study population in regard with the related marker. Conclusion: As the study findings indicated, rs2274625 could be introduced as an SNP marker in the linkage analysis in order to molecularly trace NPHS2 gene mutations in molecular NS diagnosis in Isfahan population as a representative sample of the Iranian population.

  16. Association between Mean Platelet Volume and Treatment Duration in Children with Nephrotic Syndrome

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    Fethiye İnan Damar

    2012-03-01

    Full Text Available Introduction: The aim of our study was to determine the relationship between mean platelet volume (MPV and duration of response to treatment in children with steroid-sensitive nephrotic syndrome (SSNS. Patients and Methods: This is a retrospective study which included 32 children with SSNS. The control group consisted of 32 age- and gender-matched healthy children. Results: The mean age of the children with SDNS (21 males was 6.45±3.71 years and that of controls (20 males 6.81±1.97 years. The duration of response to steroid therapy of the patients was 14.7±6.8 days. While there were no relationship between duration of treatment and the MPV, duration of treatment was inversely correlated with red blood cell count (r=-0.484, p=0.005. Conclusion: There was not an association between the MPV and the duration of response to treatment. However, the response time to treatment was increased with a decrease in number of erythrocytes. We may interpret this situation as follows: a decrease in red blood cell count may result in reduced delivery of oxygen to tissues. (The Me di cal Bul le tin of Ha se ki 2012; 50: 6-9

  17. Clinical significance of measurement of plasma leptin and serum IL-6, IL-18 levels after treatment in patients with children nephrotic syndrome

    International Nuclear Information System (INIS)

    Wang Xiaoyan

    2011-01-01

    Objective: To explore the clinical significance of changes of plasma leptin and serum IL-6, IL-18 levels after treatment in patients with children nephrotic syndrome. Methods: Plasma leptin (with RIA) serum IL-6, IL-18 (with ELISA) levels were measured in 31 patients with children nephrotic syndrome both before and after treatment as well as in 30 controls. Results: Before treatment,the plasma leptin and serum IL-6, IL-18 levels were significantly higher than those in controls(P <0.01). After treatment for 3 months, the levels in patients though dropped markedly remained significantly higher than those in controls (P<0.05). Plasma leptin levels were positively correlated with IL-6, IL-18 levels (r=0.6138, 0.5784, P<0.01). Conclusion: Changes of plasma leptin and serum IL-6, IL-18 levels after treatment might be of prognostic importance in patients with children nephrotic syndrome. (authors)

  18. Benign acute childhood myositis complicating influenza B infection in a boy with idiopathic nephrotic syndrome.

    Science.gov (United States)

    Skrzypczyk, Piotr; Przychodzień, Joanna; Pańczyk-Tomaszewska, Małgorzata

    2016-01-01

    Benign acute childhood myositis (BACM) is an acute complication of an infection characterized by calf pain, limitation of lower limb mobility, an increase in serum creatine kinase, and a self-limiting course. No reports of BACM in children with idiopathic nephrotic syndrome (INS) can be found in the literature. A 5-year-old boy with steroid-sensitive INS presented with fever, leg pain, and problems with walking. Physical examination showed pharyngeal erythema, preserved movements in all joints, and weakness of leg muscles. Laboratory tests showed white blood cell count 3900/µl, albumin 2.3 g/dl, urea 25 mg/dl, creatinine 0.3 mg/dl, increased transaminases (AspAT 440 U/l, AlAT 100 U/l) and creatine kinase (10 817 U/l), and proteinuria 3500 mg/dl. The boy was diagnosed with an INS bout and BACM. Testing for infective causes of myositis showed evidence of an influenza B virus infection. Treatment included prednisone and oseltamivir. A rapid improvement of motor function was observed, with normalization of serum creatine kinase and transaminases, and resolution of proteinuria. 1. As influenza virus infection in a child with INS is a risk factor for complications and a disease bout, these patients should be vaccinated against influenza. 2. Differential diagnosis of leg pain and mobility limitation in a child with INS should include lower limb deep venous thrombosis, arthritis, post-infectious neurological complications (including Guillain-Barré syndrome), and BACM. 3. Serum creatine kinase level should be measured in all cases of motor disturbances in a child with symptoms of respiratory tract infection.

  19. Clinical genetic testing using a custom-designed steroid-resistant nephrotic syndrome gene panel: analysis and recommendations.

    Science.gov (United States)

    Sen, Ethan S; Dean, Philip; Yarram-Smith, Laura; Bierzynska, Agnieszka; Woodward, Geoff; Buxton, Chris; Dennis, Gemma; Welsh, Gavin I; Williams, Maggie; Saleem, Moin A

    2017-12-01

    There are many single-gene causes of steroid-resistant nephrotic syndrome (SRNS) and the list continues to grow rapidly. Prompt comprehensive diagnostic testing is key to realising the clinical benefits of a genetic diagnosis. This report describes a bespoke-designed, targeted next-generation sequencing (NGS) diagnostic gene panel assay to detect variants in 37 genes including the ability to identify copy number variants (CNVs). This study reports results of 302 patients referred for SRNS diagnostic gene panel analysis. Phenotype and clinical impact data were collected using a standard proforma. Candidate variants detected by NGS were confirmed by Sanger sequencing/Multiplex Ligation-dependent Probe Amplification with subsequent family segregation analysis where possible. Clinical presentation was nephrotic syndrome in 267 patients and suspected Alport syndrome (AS) in 35. NGS panel testing determined a likely genetic cause of disease in 44/220 (20.0%) paediatric and 10/47 (21.3%) adult nephrotic cases, and 17/35 (48.6%) of haematuria/AS patients. Of 71 patients with genetic disease, 32 had novel pathogenic variants without a previous disease association including two with deletions of one or more exons of NPHS1 or NPHS2 . Gene panel testing provides a genetic diagnosis in a significant number of patients presenting with SRNS or suspected AS. It should be undertaken at an early stage of the care pathway and include the ability to detect CNVs as an emerging mechanism for genes associated with this condition. Use of clinical genetic testing after diagnosis of SRNS has the potential to stratify patients and assist decision-making regarding management. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

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

    DEFF Research Database (Denmark)

    Stæhr, Mette; Buhl, Kristian Bergholt; Andersen, René F

    2015-01-01

    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...... with aberrant filtration of uPA across the injured glomerular barrier. Amiloride inhibits urine uPA activity which attenuates plasminogen activation and urine protease activity in vivo. Urine uPA is a relevant target for amiloride in vivo....

  1. Anesthetic management of a child with nephrotic syndrome undergoing open heart surgery: Report of a rare case

    Directory of Open Access Journals (Sweden)

    Vishnu Datt

    2012-01-01

    Full Text Available The congenital nephrotic syndrome (NS in infancy and childhood is an important entity but combination with acyanotic congenital heart disease is uncommon. Anesthesia in such cases is challenging because of associated problems like hypo-protienemia, anti-thrombin III deficiency, edema, hyperlipidemia, coagulopathy, cardiomyopathy, immunodeficiency, increased lung water etc. We describe anesthetic management of a patient with childhood NS and sinus venosus atrial septal defect (ASD undergoing open heart surgery. We also suggest guidelines for safe conduct of anesthesia and CPB in such patients.

  2. [NPHS2 Mutation analysis study in children with steroid-resistant nephrotic syndrome].

    Science.gov (United States)

    Azocar, Marta; Vega, Álvaro; Farfán, Mauricio; Cano, Francisco

    2016-01-01

    Podocin is a protein located in the glomerular slit diaphragm where it takes part in the regulation of glomerular filtration. Mutations of the NPHS2 gene that codes podocin are the main cause of autosomal recessive steroid resistant nephrotic syndrome (SRNS). To identify the NPHS2 mutations in Chilean children with SRNS, and to determine the prevalence of the most common variants in a group of healthy adults. Mutation analysis of NPHS2 in 34 Chilean children with SRNS. Once the two most common variants of NPHS2 were identified, screening for these mutations was performed on 233 healthy adults. The mutation analysis was performed by the direct sequencing of the eight coding exons by polymerase chain reaction amplification. The DNA sequencing was performed using a fluorometric method, and then evaluated with SeqPilot software. The relationship between the presence of NPHS2 variants and SRNS was calculated by comparing the allele frequency between patients with SRNS and those of the healthy volunteers using the exact Fisher test. A P<.05 was considered significant. Pathogenic NPHS2 mutations were detected in 7 (21%) of the 34 patients studied, of which 6 were heterozygotes for p.R229Q and p.A284V. The presence of p.R229Q was 2.46% in the healthy volunteers. This study shows that p.R229Q and p.A284V are the most frequent variants in Chilean children with SRNS. It is the first time that this relationship has been reported in Chilean children. Based on this, a screening strategy is proposed for the genetic study in patients with SRNS and their families. A parallel or sequential search strategy for p.R229Q and p.A284V in these patients is proposed. Copyright © 2015 Sociedad Chilena de Pediatría. Publicado por Elsevier España, S.L.U. All rights reserved.

  3. Case series of thromboembolic complications in childhood nephrotic syndrome: Hacettepe experience.

    Science.gov (United States)

    Tavil, Betul; Kara, Fehime; Topaloglu, Rezan; Aytac, Selin; Unal, Sule; Kuskonmaz, Baris; Cetin, Mualla; Besbas, Nesrin; Gumruk, Fatma

    2015-06-01

    The aim of this study was to describe the incidence and contributory risk factors for thromboembolic complications in children with nephrotic syndrome (NS) and thrombosis. Among 188 children with the diagnosis of NS (80 girls; mean age: 12.6 ± 5.4 years) followed up in our hospital for the last 5 years, 17 (9.0 %) children (16 boys) identified as having thromboembolic complications. All 17 children with NS and thrombosis were screened for laboratory risk factors for thrombosis. The diagnosis was confirmed by cranial magnetic resonance imaging, doppler ultrasonography, and echocardiography. Among 17 children with thrombosis, 14 (82.3 %) were found to have focal segmental glomerulosclerosis (FSGS) as underlying pathology by renal biopsy. The mean age of the thrombotic children was 4.5 ± 3.2 years at the diagnosis of NS and that was 7.1 ± 4.9 years at the time of thrombosis. The mean time from NS diagnosis to the first thrombosis development was 2.6 ± 2.3 years. Thrombosis occurred during the first year of NS in 9/17 (52.9 %) children. Most of the children (88.2 %) had venous thrombosis. Among the screened risk factors, high factor VIII level (64.7 %) was the leading factor followed by decreased antithrombin III level (29.4 %). Furthermore, 4 children had central venous catheters and 2 had infection as clinical risk factors for thrombosis. In this case series, subtype of FSGS, active disease state of NS, central venous catheters, and some inherited and acquired thrombotic risk factors have been identified as contributory factors for the development of thrombosis in children with NS.

  4. Uteroglobin gene polymorphism (G38A) may be a risk factor in childhood idiopathic nephrotic syndrome.

    Science.gov (United States)

    Demircioglu Kılıc, Beltinge; Buyukcelik, Mithat; Oguzkan Balcı, Sibel; Pehlivan, Sacide; Kul, Seval; Col, Nilgun; Balat, Ayse

    2018-02-01

    Uteroglobin (UG) is a multifunctional protein with anti-inflammatory properties. The aim of this study was to first evaluate the role of UG gene G38A polymorphism in childhood idiopathic nephrotic syndrome (INS), and determine whether this variation may be related to the occurrence of INS or a steroid response. One hundred and thirty-six children diagnosed with INS in Gaziantep University, Department of Pediatric Nephrology, and 70 healthy volunteers were included. Children with INS were divided into two groups: steroid-sensitive (n = 84), and steroid-resistant (n = 52). Samples were examined using the polymerase chain reaction restriction fragment length polymorphism (PCR-RFLP) enzyme method. The distributions of AA, GG, and AG genotypes of UG gene G38A (G/A) were 16.9%, 44.9%, and 38.2% in the all-INS group, whereas they were 14.3%, 48.8%, and 36.9% in the steroid-sensitive INS (SSINS) group compared with 21.1%, 38.5%, and 40.4% in steroid-resistant INS (SRINS), and 5.7%, 41.4%, and 52.9% in controls. The risk of INS was increased almost 4-fold in children with the AA genotype (p = 0.016). The risk of having SSINS was increased 3.5-fold (p = 0.042) whereas the risk of SRINS was increased 4.8-fold in the same genotype (p = 0.014). The uteroglobin gene may play an important role in the development of INS, and the AA genotype of UG gene G38A polymorphism was found more frequently in those children. Further studies evaluating all polymorphisms in larger patient groups are needed to exactly determine the effect of UG gene on the development of INS and steroid response in children.

  5. Toll-like receptor 3 expression and function in childhood idiopathic nephrotic syndrome.

    Science.gov (United States)

    Jamin, A; Dehoux, L; Dossier, C; Fila, M; Heming, N; Monteiro, R C; Deschênes, G

    2015-12-01

    The efficacy of steroids and immunosuppressive treatments in idiopathic nephrotic syndrome (INS) hints at the implication of immune cells in the pathophysiology of the disease. Toll-like receptor (TLR) dysfunctions are involved in many kidney diseases of immune origin, but remain little described in INS. We investigated the expression and function of TLRs in peripheral blood mononuclear cells (PBMC) of INS children, including 28 in relapse, 23 in remission and 40 controls. No child had any sign of infection, but a higher Epstein-Barr virus viral load was measured in the PBMC of relapsing patients. TLR-3 expression was increased in B cells only during INS remission. There was a negative correlation between proteinuria and TLR-3 expression in total and the main subsets of PBMC from INS patients. The expression of TLR-8 was also increased in both CD4(+) T cells and B cells in INS remission. There was a negative correlation between proteinuria and TLR-8 expression in total PBMC, CD4(+) T cells and B cells of INS patients. Nevertheless, TLR-3 and TLR-8 expression was normalized in all PBMC subsets in an additional group of 15 INS patients in remission with B cell repletion after rituximab therapy. Paradoxically, interferon (IFN) regulatory factor 3 transactivation was increased in PBMC of all INS patients. In-vitro secretion of IFN-α and interleukin 6 were increased spontaneously in PBMC of INS remission patients, whereas PBMC from all INS patients displayed an impaired IFN-α secretion after TLR-3 stimulation. Thus, TLR-3 pathway dysfunctions may be closely involved in INS pathogenesis. © 2015 British Society for Immunology.

  6. Zinc supplementation as an adjunct to standard therapy in childhood nephrotic syndrome - a systematic review.

    Science.gov (United States)

    Bhatt, Girish Chandra; Jain, Shikha; Das, Rashmi Ranjan

    2016-11-08

    To evaluate the role of zinc as add on treatment to the "recommended treatment" of nephrotic syndrome (NS) in children. All the published literature through the major databases including Medline/Pubmed, Embase, and Google Scholar were searched till 31 st December 2015. Reference lists from the articles were reviewed to identify additional pertinent articles. Retrieved papers concerning the role of zinc in childhood NS were reviewed by the authors, and the data were extracted using a standardized data collection tool. Randomized trials (RCTs) comparing zinc vs placebo was included. Effect of zinc was studied in both steroid sensitive and steroid dependent/frequent relapsing NS. The primary outcome measure was the risk of relapse in 12 mo. The secondary outcome measures were mean relapse rate per patient in 12 mo, mean relapse rate per patient in 6 mo, risk of infection associated relapse in 12 mo, cumulative dose of steroids in two groups, mean length of time to next relapse, adverse effects of therapy, and change in serum zinc levels. Of 54 citations retrieved, a total of 6 RCTs were included. Zinc was used at a dose of 10-20 mg/d, for the duration that varied from 6-12 mo. Compared to placebo, zinc reduced the frequency of relapses, induced sustained remission/no relapse, reduced the proportion of infection episodes associated with relapse with a mild adverse event in the form of metallic taste. The GRADE evidence generated was of "very low-quality". Zinc may be a useful additive in the treatment of childhood NS. The evidence generated mostly was of "very low-quality". We need more good quality RCTs in different country setting as well different subgroups of children before any firm recommendation can be made.

  7. NUP107 mutations in children with steroid-resistant nephrotic syndrome.

    Science.gov (United States)

    Park, Eujin; Ahn, Yo Han; Kang, Hee Gyung; Miyake, Noriko; Tsukaguchi, Hiroyasu; Cheong, Hae Il

    2017-06-01

    NUP107 is a novel gene associated with autosomal recessive steroid-resistant nephrotic syndrome (SRNS) with focal segmental glomerulosclerosis (FSGS) in children. The frequency of NUP107 mutations in children with SR-FSGS remains unknown. Nine families with two siblings affected by childhood-onset SRNS or proteinuria were recruited. FSGS was confirmed by a kidney biopsy in at least one affected sibling in all families. Additionally, 69 sporadic pediatric cases with biopsy-proven SR-FSGS who had not responded to any treatment were included. All coding exons with flanking introns of the NUP107 gene were amplified using polymerase chain reaction and directly sequenced. Biallelic NUP107 mutations were detected in four pairs (44.4%) of siblings from the familial cases and three (4.3%) sporadic cases. All affected patients harbored the p.Asp831Ala mutation in one allele and a truncating or abnormal splicing mutation in the other allele. NUP107 mutation-positive patients showed an earlier onset age (39.4 ± 13.1 versus 76.8 ± 50.0 months, P= 0.027) and more rapid progression to end-stage renal disease (at the ages of 58.9 ± 23.4 versus 123.1 ± 62.7 months, P < 0.001) compared with mutation-negative patients. None of the eight mutation-positive cases, who underwent kidney transplantation, showed recurrence of FSGS in the graft kidney, while 35.3% of mutation-negative cases showed recurrence of FSGS. An unexpectedly high incidence of NUP107 mutations was observed in Korean children with SR-FSGS. Initial genetic screening of children with SR-FSGS should include the NUP107 gene, at least in Korea. Further studies are necessary to determine the incidences of NUP107 mutations in other countries. © The Author 2016. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved.

  8. [Association between angiotensin-converting enzyme 2 gene polymorphisms and childhood primary nephrotic syndrome].

    Science.gov (United States)

    Qiu, Ming-Yu; Xie, Qin-Fang; Wang, Li-Na; Yu, Li

    2015-03-01

    Angiotensin-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. The 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. Allele frequency and genotype distribution showed no significant difference between the PNS and control groups whether in female or in male children (P>0.05). The PNS group was classified 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). There 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.

  9. Rapid detection of monogenic causes of childhood-onset steroid-resistant nephrotic syndrome.

    Science.gov (United States)

    Lovric, Svjetlana; Fang, Humphrey; Vega-Warner, Virginia; Sadowski, Carolin E; Gee, Heon Yung; Halbritter, Jan; Ashraf, Shazia; Saisawat, Pawaree; Soliman, Neveen A; Kari, Jameela A; Otto, Edgar A; Hildebrandt, Friedhelm

    2014-06-06

    In steroid-resistant nephrotic syndrome (SRNS), >21 single-gene causes are known. However, mutation analysis of all known SRNS genes is time and cost intensive. This report describes a new high-throughput method of mutation analysis using a PCR-based microfluidic technology that allows rapid simultaneous mutation analysis of 21 single-gene causes of SRNS in a large number of individuals. This study screened individuals with SRNS; samples were submitted for mutation analysis from international sources between 1996 and 2012. For proof of principle, a pilot cohort of 48 individuals who harbored known mutations in known SRNS genes was evaluated. After improvements to the method, 48 individuals with an unknown cause of SRNS were then examined in a subsequent diagnostic study. The analysis included 16 recessive SRNS genes and 5 dominant SRNS genes. A 10-fold primer multiplexing was applied, allowing PCR-based amplification of 474 amplicons in 21 genes for 48 DNA samples simultaneously. Forty-eight individuals were indexed in a barcode PCR, and high-throughput sequencing was performed. All disease-causing variants were confirmed via Sanger sequencing. The pilot study identified the genetic cause of disease in 42 of 48 (87.5%) of the affected individuals. The diagnostic study detected the genetic cause of disease in 16 of 48 (33%) of the affected individuals with a previously unknown cause of SRNS. Seven novel disease-causing mutations in PLCE1 (n=5), NPHS1 (n=1), and LAMB2 (n=1) were identified in childhood-onset SRNS cases. Copyright © 2014 by the American Society of Nephrology.

  10. T-lymphocyte-expressing inflammatory cytokines underlie persistence of proteinuria in children with idiopathic nephrotic syndrome.

    Science.gov (United States)

    Guimarães, Fábio Tadeu Lourenço; Melo, Gustavo Eustáquio Brito Alvim de; Cordeiro, Thiago Macedo; Feracin, Victor; Vieira, Etel Rocha; Pereira, Wagner de Fátima; Pinheiro, Sérgio Veloso Brant; Miranda, Aline Silva; Simões-E-Silva, Ana Cristina

    2017-09-28

    There is evidence of an important role of immune system changes in the triggering and maintenance of idiopathic nephrotic syndrome (INS). The aim of this study was to investigate the expression of cytokines in lymphocyte populations of patients with INS in comparison to healthy individuals, according to proteinuria. This cross-sectional study included 44 patients with INS and eight healthy children, matched for age and sex (controls). Patients were subdivided according to proteinuria: persistent proteinuria or partial remission (PP≥300mg/24h, n=17) and low proteinuria or complete remission (LP<300mg/24h, n=27). Ex vivo analysis of peripheral blood leukocytes by flow cytometry was performed using surface markers for T-lymphocytes, TCD4, TCD8, natural killer (NK) cells, NKT, and B-lymphocytes. Frequencies of intracellular cytokines were analyzed in these cells. The frequencies of B-lymphocytes, NK cells, and NKT cells were lower in INS than in controls, whereas INS patients had a higher frequency of CD4 + tumor necrosis factor (TNF)-α + cells than controls. Cytotoxic-T-lymphocytes expressing IFN-γ were lower in INS than in controls. Patients with PP showed higher frequencies of CD4-T-lymphocytes expressing IFN-γ and TNF-α than controls. CD8-lymphocytes expressing TNF-α were increased in PP group when compared with LP and controls, while CD8 + interferon (IFN)-γ + cells were lower than in LP and in controls. Regardless the level of proteinuria, INS patients had increased expression of TNF-α in CD4-lymphocytes and reduced expression of IFN-γ in CD8-lymphocytes. Persistence of proteinuria was associated with higher levels of inflammatory markers. Copyright © 2017 Sociedade Brasileira de Pediatria. Published by Elsevier Editora Ltda. All rights reserved.

  11. 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.

  12. Effect of Astragalus injection on the red blood cell, reticulocyte and platelet parameters in patients with primary nephrotic syndrome

    Directory of Open Access Journals (Sweden)

    Lei Zhang

    2016-01-01

    Full Text Available Objective: To study the effect of Astragalus injection on the red blood cell, reticulocyte and platelet parameters in patients with primary nephrotic syndrome. Methods: A total of 88 patients were selected and randomly divided into two groups. Patients in the control group were treated with prednisone. Patients in the observation group were treated with Astragalus injection on the basis of prednisone treatment. Fourteen days referred to a course of treatment. After two courses of treatments, the changes of indexes and clinical therapeutic effect of red blood cells (hematocrit, erythrocyte aggregation index, reticulocyte (Retic, reticulocyte percentage (Retic%, reticulocyte hemoglobin content (CHr, thrombocytocrit (PCT, platelet volume distribution width (PDW, mean platelet volume (MPV, platelet parameters (PLT of the two groups were observed. Results: Two groups showed statistical difference after comparison towards clinical total effective rate. They showed obvious decreased trend in hematocrit and erythrocyte aggregation index after treatment. Comparing with those of the control group, the reticulocyte percentage, reticulocyte and reticulocyte hemoglobin content also significantly decreased and showed statistically significant. The comparison of PLT, MPV, PDW, PCT between two groups showed significant differences. Conclusions: Astragalus injection can improve the blood viscosity state and related clinical symptoms by reducing the red blood cell, reticulocyte and platelet parameters in the treatment of primary nephrotic syndrome.

  13. Systematic biomarker discovery and coordinative validation for different primary nephrotic syndromes using gas chromatography-mass spectrometry.

    Science.gov (United States)

    Lee, Jung-Eun; Lee, Yu Ho; Kim, Se-Yun; Kim, Yang Gyun; Moon, Ju-Young; Jeong, Kyung-Hwan; Lee, Tae Won; Ihm, Chun-Gyoo; Kim, Sooah; Kim, Kyoung Heon; Kim, Dong Ki; Kim, Yon Su; Kim, Chan-Duck; Park, Cheol Whee; Lee, Do Yup; Lee, Sang-Ho

    2016-07-01

    The goal of this study is to identify systematic biomarker panel for primary nephrotic syndromes from urine samples by applying a non-target metabolite profiling, and to validate their utility in independent sampling and analysis by multiplex statistical approaches. Nephrotic syndrome (NS) is a nonspecific kidney disorder, which is mostly represented by minimal change disease (MCD), focal segmental glomerulosclerosis (FSGS), and membranous glomerulonephritis (MGN). Since urine metabolites may mirror disease-specific functional perturbations in kidney injury, we examined urine samples for distinctive metabolic changes to identify biomarkers for clinical applications. We developed unbiased multi-component covarianced models from a discovery set with 48 samples (12 healthy controls, 12 MCD, 12 FSGS, and 12 MGN). To extensively validate their diagnostic potential, new batch from 54 patients with primary NS were independently examined a year after. In the independent validation set, the model including citric acid, pyruvic acid, fructose, ethanolamine, and cysteine effectively discriminated each NS using receiver operating characteristic (ROC) analysis except MCD-MGN comparison; nonetheless an additional metabolite multi-composite greatly improved the discrimination power between MCD and MGN. Finally, we proposed the re-constructed metabolic network distinctively dysregulated by the different NSs that may deepen comprehensive understanding of the disease mechanistic, and help the enhanced identification of NS and therapeutic plans for future. Copyright © 2016 Elsevier B.V. All rights reserved.

  14. NOX-mediated impairment of PDGF-induced DNA synthesis in peripheral blood lymphocytes of children with idiopathic nephrotic syndrome.

    Science.gov (United States)

    Al-Eisa, Amal; Dhaunsi, Gursev S

    2017-10-01

    BackgroundCellular oxidative stress, inflammatory responses, and immunogenic events are involved in pathogenesis of idiopathic nephrotic syndrome (INS); however, the exact mechanism remains unknown. We examined NADPH oxidase (NOX) activity and platelet-derived growth factor (PDGF)-induced DNA synthesis in peripheral blood lymphocytes (PBL) of patients with INS.MethodsPBL from 15 patients with INS and 15 age- and gender-matched controls were isolated, and enzyme activities of NOX, catalase, and superoxide dismutase (SOD) were measured along with the assay of malondialdehyde levels and bromo-deoxyuridine incorporation. Protein expression of NOX-1 was measured using western blot analysis.ResultsPatients with INS had significantly (PNOX activity and increased protein expression of NOX-1 in PBL as compared with controls. Catalase and SOD activities were markedly lower with lipid peroxide levels significantly (PNOX, markedly corrected impairment in growth factor-induced BrdU incorporation.ConclusionsThese results show that NOX activation might have a role in regulation of lymphocytic activity in patients with INS through the impairment of PDGF mitogenic function and might contribute toward pathogenesis of nephrotic syndrome.

  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. A randomized clinical trial indicates that levamisole increases the time to relapse in children with steroid-sensitive idiopathic nephrotic syndrome

    NARCIS (Netherlands)

    Gruppen, Mariken P.; Bouts, Antonia H.; Jansen-van der Weide, Marijke C.; Merkus, Maruschka P.; Zurowska, Aleksandra; Maternik, Michal; Massella, Laura; Emma, Francesco; Niaudet, Patrick; Cornelissen, Elisabeth A. M.; Schurmans, Thierry; Raes, Ann; van de Walle, Johan; van Dyck, Mieke; Gulati, Ashima; Bagga, Arvind; Davin, Jean-Claude

    2018-01-01

    Levamisole has been considered the least toxic and least expensive steroid-sparing drug for preventing relapses of steroid-sensitive idiopathic nephrotic syndrome (SSINS). However, evidence for this is limited as previous randomized clinical trials were found to have methodological limitations.

  17. REducing STEroids in Relapsing Nephrotic syndrome: the RESTERN study- protocol of a national, double-blind, randomised, placebo-controlled, non-inferiority intervention study

    NARCIS (Netherlands)

    Schijvens, A. M.; Dorresteijn, E. M.; Roeleveld, N.; ter Heine, R.; van Wijk, J. A. E.; Bouts, A. H. M.; Keijzer-Veen, M. G.; van de Kar, N. C. A. J.; van den Heuvel, L. P. W. J.; Schreuder, M. F.

    2017-01-01

    Introduction Oral corticosteroids are the first-line treatment for idiopathic childhood nephrotic syndrome. Most children experience several relapses, needing repeated courses of corticosteroid therapy. This exposes them to side effects and long-term complications. For most patients, long-term

  18. REducing STEroids in Relapsing Nephrotic syndrome : the RESTERN study- protocol of a national, double-blind, randomised, placebo-controlled, non-inferiority intervention study

    NARCIS (Netherlands)

    Schijvens, A M; Dorresteijn, Eiske M.; Roeleveld, N.; ter Heine, R.; van Wijk, Joanna A E; Bouts, Antonia H M; Keijzer-Veen, M G; van de Kar, Nicole C A J; van den Heuvel, L P W J; Schreuder, M F

    2017-01-01

    INTRODUCTION: Oral corticosteroids are the first-line treatment for idiopathic childhood nephrotic syndrome. Most children experience several relapses, needing repeated courses of corticosteroid therapy. This exposes them to side effects and long-term complications. For most patients, long-term

  19. REducing STEroids in Relapsing Nephrotic syndrome: the RESTERN study- protocol of a national, double-blind, randomised, placebo-controlled, non-inferiority intervention study

    NARCIS (Netherlands)

    Schijvens, A.M.; Dorresteijn, E.M.; Roeleveld, N.; Heine, R. ter; Wijk, J.A. van; Bouts, A.H.M.; Keijzer-Veen, M.G.; Kar, N.C.A.J. van de; Heuvel, L.P.W.J. van den; Schreuder, M.F.

    2017-01-01

    INTRODUCTION: Oral corticosteroids are the first-line treatment for idiopathic childhood nephrotic syndrome. Most children experience several relapses, needing repeated courses of corticosteroid therapy. This exposes them to side effects and long-term complications. For most patients, long-term

  20. Long-term outcome of childhood-onset complicated nephrotic syndrome after a multicenter, double-blind, randomized, placebo-controlled trial of rituximab.

    Science.gov (United States)

    Kamei, Koichi; Ishikura, Kenji; Sako, Mayumi; Aya, Kunihiko; Tanaka, Ryojiro; Nozu, Kandai; Kaito, Hiroshi; Nakanishi, Koichi; Ohtomo, Yoshiyuki; Miura, Kenichiro; Takahashi, Shori; Morimoto, Tetsuji; Kubota, Wataru; Ito, Shuichi; Nakamura, Hidefumi; Iijima, Kazumoto

    2017-11-01

    Although rituximab effectively prevents relapses of complicated frequently relapsing nephrotic syndrome (FRNS) and steroid-dependent nephrotic syndrome (SDNS), data of long-term outcomes and safety are limited. Fifty-one patients (age, 3-38 years) with childhood-onset complicated FRNS or SDNS, who received rituximab in investigator-initiated multicenter prospective trials were enrolled. Rituximab was administered at 375 mg/m 2 once weekly for 4 weeks, and immunosuppressive agents were discontinued according to the study protocol. We investigated relapses, re-administration of immunosuppressive agents, additional rituximab treatment, body height, renal function, and late adverse events during the observation period. Forty-eight patients (94%) developed relapses during the observation period (median, 59 months) and the 50% relapse-free survival was 261 days. Thirty patients (59%) developed SDNS, 44 (86%) required re-administration of immunosuppressive agents, and 22 (43%) received additional rituximab treatment. All patients who were receiving immunosuppressive agents at rituximab treatment required either immunosuppressive agents or additional rituximab treatment. On the contrary, 5 of the 13 patients without immunosuppressive agents at rituximab treatment required neither immunosuppressive agents nor additional rituximab treatment and 3 of them did not develop relapse during observation period. Growth failure due to steroid toxicity did not progress and none of the patients developed chronic renal insufficiency. None of the patients suffered from rituximab-related late adverse events. As most patients suffer from relapses after B-cell recovery, long-term immunosuppressive agents or additional rituximab treatment is necessary. However, some patients who can discontinue immunosuppressive agents before rituximab treatment may achieve long-term remission after rituximab treatment without immunosuppressive agents.

  1. A multicenter randomized trial indicates initial prednisolone treatment for childhood nephrotic syndrome for two months is not inferior to six-month treatment.

    Science.gov (United States)

    Yoshikawa, Norishige; Nakanishi, Koichi; Sako, Mayumi; Oba, Mari S; Mori, Rintaro; Ota, Erika; Ishikura, Kenji; Hataya, Hiroshi; Honda, Masataka; Ito, Shuichi; Shima, Yuko; Kaito, Hiroshi; Nozu, Kandai; Nakamura, Hidefumi; Igarashi, Takashi; Ohashi, Yasuo; Iijima, Kazumoto

    2015-01-01

    In this multicenter, open-label, randomized controlled trial, we determined whether 2-month prednisolone therapy for steroid-sensitive nephrotic syndrome was inferior or not to 6-month therapy despite significantly less steroid exposure. The primary end point was time from start of initial treatment to start of frequently relapsing nephrotic syndrome. The pre-specified non-inferiority margin was a hazard ratio of 1.3 with one-sided significance of 5%. We randomly assigned 255 children with an initial episode of steroid-sensitive nephrotic syndrome to either 2 - or 6-month treatment of which 246 were eligible for final analysis. The total prednisolone exposure counted both initial and relapse prednisolone treatment administered over 24 months. Median follow-up in months was 36.7 in the 2-month and 38.2 in the 6-month treatment group. Time to frequent relaps was similar in both groups; however, the median was reached only in the 6-month group (799 days). The hazard ratio was 0.86 (90% confidence interval, 0.64-1.16) and met the non-inferior margin. Time to first relapse was also similar in both groups: median day 242 (2-month) and 243 (6-month). Frequency and severity of adverse events were similar in both groups. Most adverse events were transient and occurred during initial or relapse therapy. Thus, 2 months of initial prednisolone therapy for steroid-sensitive nephrotic syndrome, despite less prednisolone exposure, is not inferior to 6 months of initial therapy in terms of time to onset of frequently relapsing nephrotic syndrome.

  2. GLUCOCORTICOSTEROID THERAPY AND PHYSICAL DEVELOPMENTOF CHILDREN WITH STEROID-SENSITIVE NEPHROTIC SYNDROME: A RETROSPECTIVE STUDY

    Directory of Open Access Journals (Sweden)

    Olga A. Zhdanova

    2017-01-01

    Full Text Available Background. Body weight gain and growth retardation are common side effects of prolonged glucocorticosteroid  therapy in children. Time for the appearance  and elimination of glucocorticosteroid  obesity  as well as growth disorders  require further investigations.Objective.  Our aim was to study the relationship between glucocorticosteroid  therapy and changes in physical development indices of children with steroid-sensitive nephrotic syndrome (SSNS.Methods. We carried out a retrospective study of case records of patients with SSNS hospitalized in 2011–2014.  Treatment of children was carried out in accordance with the Federal Clinical Guidelines. The Z-score (ANTHRO Plus was determined for body length (height, body weight, body mass index and correlation of physical development indices with a cumulative dose and duration of glucocorticosteroid  therapy.Results.  We analyzed data on the treatment of 31 children, 18 of them received glucocorticosteroids  for 6 months (Group 1, 13 of them did not receive glucocorticosteroids       6 months (Group 2. The Z-score  of body weight in children in these groups was 1.64 ± 1.54 and 0.05 ± 1.19 (p = 0.004, Z-score  of body mass index was 1.85 ± 1.64 and -0.54 ± 1.14, respectively (p < 0.001. Excess body weight and obesity were only observed in children of Group 1 (in 6 and 9, respectively.  The Z-score  of the body length of patients in groups 1 and 2 were comparable and did not differ from normal values (0.34 ± 1.08 and 0.52 ± 1.12, respectively, p = 0.655. Correlation of Z-score values of the body length and cumulative doses of glucocorticosteroids was noted (r = -0.87, p < 0.001.Conclusion. Long-term (at least 6 months glucocorticosteroid intake is associated with the development of overweight and obesity in most children with SSNS. In patients who did not use hormonal drugs for 6 months, normal body weight values were recorded. The height of children with SSNS was within

  3. Regulatory and effector T cells changes in remission and resistant state of childhood nephrotic syndrome

    Directory of Open Access Journals (Sweden)

    A Jaiswal

    2014-01-01

    Full Text Available Idiopathic minimal change disease is a disorder of T-cell dysfunction. The relative predominance of regulatory T cells (Tregs, Th1, and Th2 cells in nephrotic syndrome (NS remains controversial. Imbalance in peripheral blood regulatory and effector T cells (Teff are linked to cell mediated immune response and may be associated with steroid response in NS. Peripheral blood CD4 + CD25 + FoxP3 + (Tregs, CD4 + IFN-γ + (Th1, and CD4 + IL-4 + (Th2 lymphocytes were analyzed in 22 steroid-sensitive NS (SSNS patients in sustained remission, 21 steroid-resistant NS (SRNS and 14 healthy controls. The absolute percentage values and ratio of Th1/Tregs, Th2/Tregs, and Th1/Th2 were compared between SSNS, SRNS and control subjects. The percentage of Tregs was lower in SRNS patients (P = 0.001 compared with that of SSNS and healthy control. The percentage of Th1 cells was higher in SRNS (P = 0.001 compared to that of SSNS patients; however, it was similar to healthy controls (P = 1.00. The percentage of Th2 cells in SRNS (P = 0.001 was higher as compared to SSNS and controls. The ratio of Th1/Treg cells in SRNS (P = 0.001 was higher as compared to SSNS patients and controls. The ratio of Th2/Treg was also higher in SRNS as compared to SSNS and controls. The ratio of Th1/Th2 cells in SSNS, SRNS, and healthy controls were similar. The cytokines secretion complemented the change in different T-cell subtypes in SSNS, SRNS and healthy controls. However, the IFN-γ secretion in healthy controles was low inspite of similar percentage of Th1 cells among SRNS cases. We conclude that greater ratio of Tregs compared to that Th1 and Th2 favor steroid sensitivity and reverse ratio results in to SRNS. The difference in ratio is related to pathogenesis or it can be used as marker to predict steroid responsiveness needs further evaluation.

  4. 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. © The Author(s) 2016.

  5. 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. Copyright © 2016 International Society of Nephrology. Published by Elsevier Inc. All rights reserved.

  6. HLA-DQA1 and PLCG2 Are Candidate Risk Loci for Childhood-Onset Steroid-Sensitive Nephrotic Syndrome.

    Science.gov (United States)

    Gbadegesin, Rasheed A; Adeyemo, Adebowale; Webb, Nicholas J A; Greenbaum, Larry A; Abeyagunawardena, Asiri; Thalgahagoda, Shenal; Kale, Arundhati; Gipson, Debbie; Srivastava, Tarak; Lin, Jen-Jar; Chand, Deepa; Hunley, Tracy E; Brophy, Patrick D; Bagga, Arvind; Sinha, Aditi; Rheault, Michelle N; Ghali, Joanna; Nicholls, Kathy; Abraham, Elizabeth; Janjua, Halima S; Omoloja, Abiodun; Barletta, Gina-Marie; Cai, Yi; Milford, David D; O'Brien, Catherine; Awan, Atif; Belostotsky, Vladimir; Smoyer, William E; Homstad, Alison; Hall, Gentzon; Wu, Guanghong; Nagaraj, Shashi; Wigfall, Delbert; Foreman, John; Winn, Michelle P

    2015-07-01

    Steroid-sensitive nephrotic syndrome (SSNS) accounts for >80% of cases of nephrotic syndrome in childhood. However, the etiology and pathogenesis of SSNS remain obscure. Hypothesizing that coding variation may underlie SSNS risk, we conducted an exome array association study of SSNS. We enrolled a discovery set of 363 persons (214 South Asian children with SSNS and 149 controls) and genotyped them using the Illumina HumanExome Beadchip. Four common single nucleotide polymorphisms (SNPs) in HLA-DQA1 and HLA-DQB1 (rs1129740, rs9273349, rs1071630, and rs1140343) were significantly associated with SSNS at or near the Bonferroni-adjusted P value for the number of single variants that were tested (odds ratio, 2.11; 95% confidence interval, 1.56 to 2.86; P=1.68×10(-6) (Fisher exact test). Two of these SNPs-the missense variants C34Y (rs1129740) and F41S (rs1071630) in HLA-DQA1-were replicated in an independent cohort of children of white European ancestry with SSNS (100 cases and ≤589 controls; P=1.42×10(-17)). In the rare variant gene set-based analysis, the best signal was found in PLCG2 (P=7.825×10(-5)). In conclusion, this exome array study identified HLA-DQA1 and PLCG2 missense coding variants as candidate loci for SSNS. The finding of a MHC class II locus underlying SSNS risk suggests a major role for immune response in the pathogenesis of SSNS. Copyright © 2015 by the American Society of Nephrology.

  7. NPHS1 gene mutations confirm congenital nephrotic syndrome in four Brazilian cases: A novel mutation is described.

    Science.gov (United States)

    Guaragna, Mara S; Cleto, Thaís Lira; Souza, Marcela Lopes; Lutaif, Anna Cristina G B; de Castro, Luiz Cláudio Gonçalves; Penido, Maria Goretti Moreira Guimarães; Maciel-Guerra, Andréa T; Belangero, Vera M S; Guerra-Junior, Gil; De Mello, Maricilda P

    2016-09-01

    Autosomal recessive mutations in NPHS1 gene are a common cause of congenital nephrotic syndrome (CNS). The disorder is characterized by massive proteinuria that manifests in utero or in the neonatal period during the first 3 months of life. NPHS1 encodes nephrin, a member of the immunoglobulin family of cell adhesion molecules and the main protein expressed at the renal slit diaphragm. Currently, there are approximately 250 mutations described in the NPHS1 gene distributed among all nephrin domains. The main objective of this study was to perform the analysis of the NPHS1 gene in patients with congenital nephrotic syndrome in order to determine the molecular cause of the disease. Direct sequencing of NPHS1 gene in four children was performed. Each patient was heterozygous for two pathogenic mutations disclosing the molecular cause of the disease in 100% of the cases. We identified six different mutations, consisting of one in-frame deletion, one frameshift, and four missense substitutions. The p.Val736Met mutation that is described here for the first time was considered pathogenic by different mutation predictive algorithms. Regardless of the type of mutation, three patients had a bad outcome and died Despite the small size of the cohort, this study contributed to the increasing number of deleterious mutations in the NPHS1 gene by describing a new mutation. Also, since we identified NPHS1 pathogenic mutations as the cause of the disease in all cases analyzed, it might be a frequent cause of CNS in the South Eastern region of Brazil, although the analysis of a larger sample is required to obtain more indicative epidemiological data. © 2015 Asian Pacific Society of Nephrology.

  8. Report of novel genetic variation in NPHS2 gene associated with idiopathic nephrotic syndrome in South Indian children.

    Science.gov (United States)

    Dhandapani, Mohanapriya Chinambedu; Venkatesan, Vettriselvi; Rengaswamy, Nammalwar Bollam; Gowrishankar, Kalpana; Ekambaram, Sudha; Sengutavan, Prabha; Perumal, Venkatachalam

    2017-02-01

    Steroid-resistant nephrotic syndrome (SRNS) is found in 10-20 % of children with idiopathic nephrotic syndrome (INS). In SRNS patients, common histopathological subtypes are Focal segmental glomerulosclerosis (FSGS) (53 %) and minimal change disease (MCD) (27 %). Familial forms of FSGS constitute podocyte diseases with varying severity and age of onset. Podocin gene (NPHS2) mutations cause childhood-onset steroid-resistant FSGS and MCD to adult-onset FSGS. In view of genetic variations and susceptibility to the disease, the present investigation was undertaken to study the pattern of genetic mutation in children from South India. Mutation analysis was carried out by direct sequencing of the entire NPHS2 gene (eight exons) using specific primers in 200 INS (100 SRNS and 100 steroid sensitive) children and 100 healthy controls. The allele and genotype frequencies of NPHS2 gene were calculated for both cases and controls as per Hardy-Weinberg equilibrium. Among the SRNS patients, 18 % revealed both heterozygous and homozygous mutations. Out of 12 mutations, 8 were homozygous and 4 were heterozygous. Interestingly, we found two novel SNPs in exon 4 of NPHS2 gene, which are documented and submitted to dbsnp database (Ref rs12401711 and rs12401708). Mutational analysis of NPHS2 would be advisable at the start of treatment. The genetic variations detected in the study would serve as the important molecular marker in treating the children's at early stage, which also enables to detect carriers, prenatal diagnosis and provide genetic counseling to couples at risk.

  9. Multi-drug resistance-1 gene polymorphisms in nephrotic syndrome: impact on susceptibility and response to steroids.

    Science.gov (United States)

    Youssef, Doaa M; Attia, Tarek A; El-Shal, Amal S; Abduelometty, Fawzya A

    2013-11-10

    Role of multidrug resistance-1 (MDR-1) gene polymorphisms has not been clarified in nephrotic syndrome (NS). Additionally, researchers studied several genetic polymorphisms to explain their influence on different patients' responses to steroid; however the data were inconsistent. Therefore, we aimed to investigate the association of MDR-1 gene polymorphisms [C1236T, G2677T/A, C3435T] and haplotypes with susceptibility to childhood nephrotic syndrome, and whether they influence steroid response. We detected MDR-1 gene polymorphisms using polymerase chain reaction restriction fragment length polymorphism (PCR-RFLP) in 138 NS patients and 140 age and sex matched healthy children. The frequencies of MDR1 G2677T/A GT, GA, TT+AA genotypes or T allele, MDR1 C3435T TT genotype, and T allele genotype frequencies were significantly increased in NS group. While no significant differences were observed in distributions of C1236T genotypes or allele between NS patients and healthy children. Moreover, steroid non-responder NS patients had significantly higher frequencies of MDR1 G2677T/A GT, GA, and TT+AA genotypes than steroid responsive NS patients. We observed also that NS patients with age less than 6 years old had increased frequencies of MDR1 G2677T/A GT, GA, TT+AA genotypes or T allele MDR1 C3435T CT, TT genotypes and T allele. Interestingly the frequency of the TGC haplotype of MDR1 was lower in the initial steroid responders than in non-responders NS patients. On the contrary, there were no any association between the MDR1 haplotypes with NS susceptibility and they did not influence renal pathological findings. Our data suggested that MDR1 C3435T or G2677T/A gene polymorphisms are risk factors of increased susceptibility, earlier onset of NS, and steroid resistance. © 2013 Elsevier B.V. All rights reserved.

  10. Rituximab in steroid-dependent idiopathic nephrotic syndrome in childhood--follow-up after CD19 recovery.

    Science.gov (United States)

    Sellier-Leclerc, Anne-Laure; Baudouin, Véronique; Kwon, Thérésa; Macher, Marie-Alice; Guérin, Valérie; Lapillonne, Hélène; Deschênes, Georges; Ulinski, Tim

    2012-03-01

    Rituximab (RTX) is a new treatment strategy in high-degree steroid-dependent idiopathic nephrotic syndrome (SDNS) in childhood. Thirty patients (nine girls) with SDNS with steroid side effects and previously treated with immunosuppressive drugs, mostly calcineurin inhibitors, were treated with RTX and included in this non-controlled single-centre study. Patient age at first RTX infusion was 12.9 ± 0.7 years. Our aim was to evaluate disease outcome after a minimum CD19 depletion period of 15 months obtained by repeated RTX infusion. Minimum follow-up after initial CD19 depletion was 24 months. During the RTX treatment period, seven patients had nephrotic syndrome relapses, six among them at the time of an intermittent CD19 recovery and one patient relapsed under CD19 depletion. The risk for these patients to relapse after the RTX treatment period was higher than in those without intermittent relapses. After definitive CD19 recovery over a follow-up of 17.4 ± 1.9 months, 19 patients (63%) did not relapse and 11 (37%) relapsed 4.3 ± 1 months after defininitive CD19 recovery. Among these 11 patients, 6 already had intermittent relapses during the RTX treatment period. Steroid and immunosuppressive treatment could be discontinued in all patients during CD19 depletion and was re-introduced in two after CD19 recovery. Fourteen patients had mostly benign and transitory side effects, which did not require RTX discontinuation. In conclusion, RTX treatment with a 15-month CD19 depletion period induced long-term remission after definitive CD 19 recovery in almost two-thirds the of patients without oral immunosuppressive drugs.

  11. α-asarone reduce proteinuria by restoring antioxidant enzymes activities and regulating necrosis factor κB signaling pathway in doxorubicin-induced nephrotic syndrome.

    Science.gov (United States)

    Sutariya, Brijesh; Saraf, Madhusudan

    2018-02-01

    α-asarone is natural bioactive compound that has been reported to have many benefits and medicinal properties. The present study aimed to assess the protective effect of α-asarone against doxorubicin (DOX) induced nephrotic syndrome in rats. An experimental nephrotic syndrome was induced by single intravenous injection of DOX (7 mg/kg) in rats. Animals were orally administered α-asarone (10 and 20 mg kg -1  d -1 ) for 4 weeks. Blood, urine and kidney tissues were collected for analyses at the end of the study. Treatment with α-asarone significantly improved kidney function by significantly inhibiting proteinuria, hypoalbuminemia, dyslipidemia, and restored antioxidant enzyme activities in kidney tissue. Furthermore, α-asarone ameliorated mRNA and protein expression of NF-κB, TNF-α, IL-6, and podocin in the kidney. Histopathological evidence also confirmed the protective effects of α-asarone against DOX-induced nephrotic syndrome. In conclusion, α-asarone has an anti-nephritic effect that might be attributed to its antioxidant, hypolipidaemic and anti-inflammatory activities. Copyright © 2017. Published by Elsevier Masson SAS.

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

    Directory of Open Access Journals (Sweden)

    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.

  13. REducing STEroids in Relapsing Nephrotic syndrome: the RESTERN study— protocol of a national, double-blind, randomised, placebo-controlled, non-inferiority intervention study

    Science.gov (United States)

    Schijvens, A M; Dorresteijn, E M; Roeleveld, N; ter Heine, R; van Wijk, J A E; Bouts, A H M; Keijzer-Veen, M G; van de Kar, N C A J; van den Heuvel, L P W J; Schreuder, M F

    2017-01-01

    Introduction Oral corticosteroids are the first-line treatment for idiopathic childhood nephrotic syndrome. Most children experience several relapses, needing repeated courses of corticosteroid therapy. This exposes them to side effects and long-term complications. For most patients, long-term prognosis is for complete resolution of the disease over time and maintenance of normal kidney function. Therefore, it is vital to focus on minimising adverse events of the disease and its therapy. Unfortunately, no randomised controlled trials are available to determine the optimal corticosteroid treatment of an infrequent relapse of nephrotic syndrome. Recent studies show that treatment schedules for the first episode can safely be shortened to 2 months. The hypothesis of the REducing STEroids in Relapsing Nephrotic syndrome (RESTERN) study is that a 4-week reduction of alternate-day steroids after inducing remission is effective and safe, reduces steroid exposure by 35% on average and is therefore preferable. Methods and analysis The RESTERN study is a nationwide, double-blind, randomised, placebo-controlled, non-inferiority intervention study. Children aged 1–18 years with a relapse of steroid-sensitive nephrotic syndrome are eligible for this study. Study subjects (n=144) will be randomly assigned to either current standard therapy in the Netherlands or a reduced prednisolone schedule. The primary outcome of the RESTERN study is the time to first relapse after the final prednisolone dose. The secondary outcomes are the number or relapses, progression to frequent relapsing or steroid dependent nephrotic syndrome and the cumulative dosage of prednisolone during the study period. Ethics and dissemination This non-inferiority trial will be performed in accordance with the Declaration of Helsinki and has been approved by the medical ethical committee of Arnhem-Nijmegen and the Dutch Competent Authority (Central Committee on Research Involving Human Subjects, CCMO

  14. REducing STEroids in Relapsing Nephrotic syndrome: the RESTERN study- protocol of a national, double-blind, randomised, placebo-controlled, non-inferiority intervention study.

    Science.gov (United States)

    Schijvens, A M; Dorresteijn, E M; Roeleveld, N; Ter Heine, R; van Wijk, J A E; Bouts, A H M; Keijzer-Veen, M G; van de Kar, N C A J; van den Heuvel, L P W J; Schreuder, M F

    2017-09-27

    Oral corticosteroids are the first-line treatment for idiopathic childhood nephrotic syndrome. Most children experience several relapses, needing repeated courses of corticosteroid therapy. This exposes them to side effects and long-term complications. For most patients, long-term prognosis is for complete resolution of the disease over time and maintenance of normal kidney function. Therefore, it is vital to focus on minimising adverse events of the disease and its therapy. Unfortunately, no randomised controlled trials are available to determine the optimal corticosteroid treatment of an infrequent relapse of nephrotic syndrome. Recent studies show that treatment schedules for the first episode can safely be shortened to 2 months. The hypothesis of the REducing STEroids in Relapsing Nephrotic syndrome (RESTERN) study is that a 4-week reduction of alternate-day steroids after inducing remission is effective and safe, reduces steroid exposure by 35% on average and is therefore preferable. The RESTERN study is a nationwide, double-blind, randomised, placebo-controlled, non-inferiority intervention study. Children aged 1-18 years with a relapse of steroid-sensitive nephrotic syndrome are eligible for this study. Study subjects (n=144) will be randomly assigned to either current standard therapy in the Netherlands or a reduced prednisolone schedule. The primary outcome of the RESTERN study is the time to first relapse after the final prednisolone dose. The secondary outcomes are the number or relapses, progression to frequent relapsing or steroid dependent nephrotic syndrome and the cumulative dosage of prednisolone during the study period. This non-inferiority trial will be performed in accordance with the Declaration of Helsinki and has been approved by the medical ethical committee of Arnhem-Nijmegen and the Dutch Competent Authority (Central Committee on Research Involving Human Subjects, CCMO). After completion of this study, results will be published in

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

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    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

  16. Parental Attitudes to Genetic Testing Differ by Ethnicity and Immigration in Childhood Nephrotic Syndrome: A Cross-Sectional Study

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    Karlota Borges

    2016-03-01

    Full Text Available Background: Studies in the USA report differences in opinion among parents of different ethnic groups toward genetic testing for their child; however, there are no studies that address this issue in the diverse ethnic and immigrant population in Canada. Objective: This study aims to determine whether ethnicity and immigration status influences parental interest in clinical genetic testing for a potentially progressive kidney disease. Design: This is a cross-sectional study. Setting: Participants were recruited from the Greater Toronto Area, Canada. Participants: The study included 320 parents of children ages 1–18 years with nephrotic syndrome enrolled in the Insight into Nephrotic Syndrome: Investigating Genes, Health and Therapeutics (INSIGHT observational cohort study. Measurements: Demographic, ethnicity, immigration, and child specific factors as well as interest in genetic testing were collected through self-reported questionnaires administered at baseline study visit. Methods: Logistic regression models were used to examine association of ethnicity and immigration status with interest in genetic testing. Results: The majority of parents (85 % were interested in genetic testing for their child. South Asian and East/Southeast Asian parents had 74 and 76 % lower odds of agreeing to genetic testing when compared to Europeans (odds ratio (OR 0.26, 95 % confidence interval (CI 0.10–0.68; OR 0.24, 95 % CI 0.07–0.79, respectively after controlling for age and sex of child, age and education level of parent, initial steroid resistance, and duration of time in Canada. Immigrants to Canada also had significantly lower odds (OR 0.29, 95 % CI 0.12–0.72 of agreeing to genetic testing after similar adjustment. Higher education level was not associated with greater interest in genetic testing (OR 1.24, 95 % CI 0.64–2.42. Limitations: Participants have already agreed to aggregate genetic testing for research purposes as part of enrolment in

  17. Parental attitudes to genetic testing differ by ethnicity and immigration in childhood nephrotic syndrome: a cross-sectional study.

    Science.gov (United States)

    Borges, Karlota; Vasilevska-Ristovska, Jovanka; Hussain-Shamsy, Neesha; Patel, Viral; Banh, Tonny; Hebert, Diane; Pearl, Rachel J; Radhakrishnan, Seetha; Piscione, Tino D; Licht, Christoph P B; Langlois, Valerie; Levin, Leo; Strug, Lisa; Parekh, Rulan S

    2016-01-01

    Studies in the USA report differences in opinion among parents of different ethnic groups toward genetic testing for their child; however, there are no studies that address this issue in the diverse ethnic and immigrant population in Canada. This study aims to determine whether ethnicity and immigration status influences parental interest in clinical genetic testing for a potentially progressive kidney disease. This is a cross-sectional study. Participants were recruited from the Greater Toronto Area, Canada. The study included 320 parents of children ages 1-18 years with nephrotic syndrome enrolled in the Insight into Nephrotic Syndrome: Investigating Genes, Health and Therapeutics (INSIGHT) observational cohort study. Demographic, ethnicity, immigration, and child specific factors as well as interest in genetic testing were collected through self-reported questionnaires administered at baseline study visit. Logistic regression models were used to examine association of ethnicity and immigration status with interest in genetic testing. The majority of parents (85 %) were interested in genetic testing for their child. South Asian and East/Southeast Asian parents had 74 and 76 % lower odds of agreeing to genetic testing when compared to Europeans (odds ratio (OR) 0.26, 95 % confidence interval (CI) 0.10-0.68; OR 0.24, 95 % CI 0.07-0.79, respectively) after controlling for age and sex of child, age and education level of parent, initial steroid resistance, and duration of time in Canada. Immigrants to Canada also had significantly lower odds (OR 0.29, 95 % CI 0.12-0.72) of agreeing to genetic testing after similar adjustment. Higher education level was not associated with greater interest in genetic testing (OR 1.24, 95 % CI 0.64-2.42). Participants have already agreed to aggregate genetic testing for research purposes as part of enrolment in INSIGHT study. While majority of parents were interested in genetic testing for their child, immigrants, particularly

  18. Trends in the histopathology of childhood nephrotic syndrome in Ibadan Nigeria: preponderance of idiopathic focal segmental glomerulosclerosis.

    Science.gov (United States)

    Asinobi, Adanze O; Ademola, Adebowale D; Okolo, Clement A; Yaria, Joseph O

    2015-12-15

    Reports on the histopathology of childhood nephrotic syndrome (NS) had emanated from our Centre since the 1960s and by the late 1980s and early 1990s, a change was observed and reported. Taking into consideration the worldwide changing trend in the histopathology of the NS and our Unit policy change in the indications for renal biopsy, a change was envisaged. We therefore evaluated the current histologic pattern of childhood NS in Ibadan with the view to highlighting any variations from the past and comparing the findings with regional and global trends. We reviewed our database and analyzed the renal biopsy findings in patients who were biopsied before treatment was administered between 1997 and 2001 and those with mostly idiopathic steroid resistant NS (SRNS) and secondary NS, managed between 2006 and 2013. A comparative analysis of the findings from the present study was carried out with two previous reports from our Unit in the 1970s and early 1990s and also with reports from other Centres. A total of 78 patients had successful biopsies done during the study period in children aged between 2 ½ and 16 years. In both pre-treatment biopsy era (1997-2001) and post-treatment biopsy era (2006-2013), focal segmental glomerulosclerosis (FSGS) predominated. 75 % of the patients had idiopathic NS and among the patients that had idiopathic steroid resistant NS, FSGS was the most common followed by MPGN. For secondary NS, MCD was the most common but could be the early stages of either membranous nephropathy (MN) or FSGS. Chronic pyelonephritis and chronic interstitial nephritis occurred in 25 % of the study population but they were more prevalent in secondary nephrotic syndrome. FSGS is the most common histopathology in children requiring renal biopsy in Ibadan presently. FSGS is also the most common histopathology in idiopathic SRNS, which is in keeping with reports from most parts of the world. There has been a transition from the preponderance of Quartan Malarial

  19. Vitamin D receptor gene TaqI and Apal polymorphisms and steroid responsiveness in childhood idiopathic nephrotic syndrome

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    Al-Eisa AA

    2016-08-01

    Full Text Available Amal A Al-Eisa, Mohammad Z Haider Department of Pediatrics, Faculty of Medicine, Kuwait University, Safat, Kuwait Background: Vitamin D activity is controlled by vitamin D receptors (VDRs, which are affected by different genetic polymorphisms, including TaqI and Apal restriction fragment length polymorphisms (RFLPs, which have been reported to be associated with several diseases. The aim of this study was to determine the frequency and the association of VDR gene polymorphisms with idiopathic nephrotic syndrome (INS and steroid responsiveness in Kuwaiti children. Subjects and methods: Genotypes of the VDR TaqI gene polymorphism and the Apal gene polymorphism were analyzed using polymerase chain reaction-RFLP in 78 INS patients and 56 matched controls. Results: A total of 78 INS (62 steroid sensitive [SS] and 16 steroid resistant [SR] patients with a mean age of 6.5±3.1 years were studied. Male:female ratio was 2:1. The TT genotype of VDR–TaqI polymorphism was detected in 41% of the INS patients compared to 42% of the controls (P=0.816. The heterozygous TC genotype was detected in 33% of INS patients compared to 46% of the controls (P=0.462. The CC genotype was detected in 25.6% of INS patients and 21% of the controls (P=0.719. The C-allele frequency, in its homozygous and heterozygous forms, was 71% in INS patients compared to 63% in the controls (P=0.342. Similarly, no significant difference was detected in terms of VDR–Apal polymorphism in INS patients compared to the controls for all the three genotypes (P=0.76, P=0.207, and P=0.364, respectively, for GG, GT, and TT genotypes. The T-allele frequency, in its homozygous and heterozygous forms, was 89% in INS patients compared to 93% in the controls (P=0.076. No significant difference was found in any of the allele frequencies between SS and SR subgroups when compared with each other or when compared to the controls. Conclusion: Our data do not support the use of VDR–TaqI or

  20. The significance of determination of renal tubular markers before and after treatment in the primary nephrotic syndrome

    International Nuclear Information System (INIS)

    Xie Bing; Jiang Liping

    2011-01-01

    Objective: To evaluate the damage of renal tubule of patients with primary nephrotic syndrome (PNS) by detecting renal tubule markers and investigate the significance of different therapeutic effects. Methods: Serum levels of interleukin-6(IL-6), ET-1, α 1 -microglobulin(α 1 -m), β 2 -microglobulin(β 2 -m) and plasma level of ET-1 were determined with RIA, fibrinogen degradation product (FDP) with ELISA, automatic biochemistry analysis N-acetyl-β-D-glucosaminidase(NAG), CH 2 O was determined with physico-method respectively. Results: The concentrations of IL-6, ET-1, α 1 -m, β 2 -m, FDP, NAG were significantly decreased in cases of complete remission after therapy (P 2 O excepted (P>0.05), the decrease of IL-6, ET-1, α 1 -m, FDP were no significant in cases of invalid (P>0.05), the concentrations of renal tubule markers in cases of partial remission and invalid were higher than those in cases of complete and significant remission. Conclusion: The determination of several renal tubule markers can be used for diagnose, monitor and judge the therapeutic effects of PNS. (authors)

  1. A novel approach to the histological diagnosis of pediatric nephrotic syndrome by low vacuum scanning electron microscopy.

    Science.gov (United States)

    Okada, Shinichi; Inaga, Sumire; Kawaba, Yasuo; Hanada, Takuya; Hayashi, Atsushi; Nakane, Hironobu; Naguro, Tomonori; Kaidoh, Toshiyuki; Kanzaki, Susumu

    2014-01-01

    Despite intensive treatment, steroid-resistant nephrotic syndrome (NS) often progresses to endstage renal disease. Therefore, a more accurate and quick histological diagnosis is required to properly treat such patients. The aim of this study was to introduce a novel approach to the histological diagnosis of pediatric NS by low vacuum scanning electron microscopy (LVSEM) and to describe the morphological differences in glomeruli between steroid-sensitive and steroid-resistant NS specimens. The subjects were three patients with steroid-sensitive NS and four patients with steroid-resistant NS. Conventional renal biopsy paraffin sections were stained with platinum-blue (Pt-blue) or periodic acid methenamine silver (PAM) and directly observed under LVSEM at magnifications between ×50 and ×10,000. The Pt-blue-stained sections showed three-dimensional structural alterations in glomerular podocytes and foot processes. PAM-stained sections showed changes in the structure and thickness of the glomerular basement membrane (GBM). Consequently, many round-shaped podocytes and elongated primary foot processes were exclusively recognized in steroid-resistant NS, although irregularities in foot process interdigitation, fusions, effacements, and microvillus transformations were observed in both steroid-sensitive and steroidresistant NS. Irregularities in thickness and the wrinkling of GBMs were clearly detected in steroid-resistant NS. The evaluation by LVSEM is probably useful for the renal histological diagnosis of pediatric NS.

  2. The relationship between renal P-glycoprotein expression and response to steroid therapy in childhood nephrotic syndrome.

    Science.gov (United States)

    Türkmen, Mehmet; Torun Bayram, Meral; Soylu, Alper; Özer, Erdener; Sarıoğlu, Sülen; Kavukçu, Salih

    2013-01-01

    In this study, we aimed to investigate the relationship between renal P-glycoprotein (rP-gp) expression and response to corticosteroid therapy in childhood nephrotic syndrome (NS). Expression of rP-gp was evaluated prior to non-steroid immunosuppressive therapy in children with NS (Group 1), prior to any treatment in children with immunoglobulin (Ig)A nephropathy (Group 2), and during renal donation in healthy adults (Group 3); mesangial proliferation was evaluated in Groups 1 and 2. Total dosage of steroid was calculated in Group 1. The ratio of rP-gp (+) glomeruli was higher in Group 1 than Group 3 (33.8 ± 27.1% vs 4.7 ± 4.6%, p=0.000). There were no rP-gp (+) glomeruli in Group 2. The rate of mesangial proliferation was similar in Groups 1 and 2. There was no statistically significant correlation between rP-gp expression and total steroid dosage (r=0.455, p=0.160). Our findings showed that rP-gp expression is increased in patients receiving steroid therapy for NS regardless of the cumulative steroid dosage and mesangial cell proliferation.

  3. 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.

  4. Mesenchymal Stem Cells May Ameliorate Nephrotic Syndrome Post-Allogeneic Hematopoietic Stem Cell Transplantation-Case Report

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    Xin Zhang

    2017-08-01

    Full Text Available IntroductionBecause of their immunomodulatory and anti-inflammatory effects, mesenchymal stem cells (MSCs have been considered as potential therapeutic agents for treating immune-related or autoimmune diseases, such as graft-versus-host disease (GVHD. Nephrotic syndrome (NS after allogeneic hematopoietic stem cell transplantation (allo-HSCT is an uncommon complication with unclear etiology and pathogenesis. It may be an immune disorder involving immune complex deposition, B cells, regulatory T cells (Tregs, and Th1 cytokines and be a manifestation of chronic GVHD. Corticosteroids and calcium antagonists, alone or in combination, are the most common therapeutic agents in this setting. Rituximab is commonly administered as salvage treatment. However, treatment failure and progressive renal function deterioration has been reported to occur in approximately 20% of patients in a particular cohort.Case presentationWe present a patient who developed NS 10 months after allo-HSCT. After treatment failure with cyclosporine A, prednisone, and rituximab, she achieved a complete response with MSC treatment. The clinical improvement of this patient was accompanied by a decreased B cell population together with an increased frequency of regulatory B cells (Bregs and Tregs after MSC treatment.ConclusionMSCs could modulate NS after allo-HSCT by suppressing B cell proliferation, inducing Tregs and Bregs, and inhibiting inflammatory cytokine production by monocytes and NK cells. Among all these, Bregs might play an important role in ameliorating the NS of this patient.

  5. Association of neutrophil gelatinase associated lipocalin and cystatin-c with kidney function in children with nephrotic syndrome

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    Alaleh Gheissari

    2013-01-01

    Full Text Available Background: Nephrotic syndrome (NS is a major clinical concern in human health, especially in children. Despite of the etiology, the prediction of remission in different treatment regimens based on suitable biomarkers is under development. The goal of this evaluation was the demonstration of correlation between serum level of Neutrophil gelatinase associated lipocalin (NGAL and cystatin-C with kidney function in patients with NS. Methods: During the period between September 2008 and December 2011, 52 patients admitted to St. Al Zahra University Hospital were selected for evaluation. The measured parameters consisted of NGAL, cystatin-C, creatinine, albumin, blood urea nitrogen, urine protein, glomerular filtration rate. Demographic data were collected and considered in comparisons. Comparison between variables and their correlations were examined. Results: Means of serum NGAL and cystatin-C were significantly higher in case than the control group, P < 0.05. The mean of serum NGAL in patients without remission and who achieved remission were 23.09 (standard deviation [SD] ±10.11 and 36.26 (SD ± 20.10 ng/ml respectively; P < 0.05. Serum NGAL levels had a correlation with the following factors: Systolic blood pressure, diastolic blood pressure (DBP, cystatin-C, remission. Linear regression analysis showed a significant correlation between cystatin-C and systolic and DBP. Conclusions: Based on the results, serum NGAL can be used as a prognostic marker for remission. In addition, NGAL and cystatin-C are biomarkers of kidney injury in NS.

  6. A relationship between proteinuria and acute tubulointerstitial disease in rats with experimental nephrotic syndrome

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    Eddy, A.A.; McCulloch, L.; Liu, E.; Adams, J. (Hospital for Sick Children, Toronto, ON (Canada))

    1991-05-01

    The relationship between tubulointerstitial nephritis and proteinuria was characterized in experimental nephrosis in rats. In one group, proteinuria induced by aminonucleoside of puromycin (PAN) was reduced by using an 8% protein diet and adding the angiotensin I-converting enzyme (ACE) inhibitor enalapril to the drinking water. Two control groups were injected with saline and PAN, respectively, and fed a 27% protein diet. The first group had significantly reduced albuminuria and a definite attenuation of tubular cell injury. There was a strong positive correlation between the number of interstitial macrophages and albuminuria. The beneficial effect was reproduced by dietary-protein restriction alone, whereas ACE inhibition alone had an insignificant effect on the degree of proteinuria. Depletion of circulating T lymphocytes in one group of nephrotic rats eliminated interstitial lymphocytes but did not affect interstitial macrophage influx. Inhibition of the in situ proliferation of resident interstitial macrophages by unilateral kidney irradiation failed to change the intensity of the macrophage infiltration. Treatment of rats with sodium maleate produced proximal tubular cell toxicity but interstitial inflammation did not develop, suggesting that the latter is not a nonspecific response to tubular injury. These studies demonstrate a strong relationship between tubulointerstitial nephritis and the severity of proteinuria in experimental nephrosis.

  7. Serum and urinary lipoproteins in the human nephrotic syndrome: evidence for renal catabolism of lipoproteins

    Energy Technology Data Exchange (ETDEWEB)

    Shore, V.G.; Forte, T.; Licht, H.; Lewis, S.B.

    1982-03-01

    The urinary excretion of lipoproteins and the possibility of catabolic alterations on glomerular filtration were investigated in four nephrotic subjects difering in etiology, serum lipoprotein profile, and 24 hr urinary output of protein and lipids. The apolipoproteins and lipoproteins of urine were compared with those of serum with respect to distribution profile, physical properties, and composition. As expected from molecular sieving effects during glomerular filtration, the urinary HDL were more abundant than the lower density lipoproteins even when the plasma LDL was elevated markedly. Intact apolipoproteins were not found in the concentrated urinary fraction isolated by ultrafiltration between the limits of 10/sup 4/ and 5 x 10/sup 4/ daltons. On the basis of immunoreactivity, gel electrophoresis, and amino acid composition, apolipoproteins B and AI are the major and minor proteins, respectively, of urinary LDL, and apo B is the major protein of the urinary IDL and VLDL. Apolipoproteins AI, AII, CI, CIII, and possibly AIV were isolated from the urinary HDL. As much as 20% of the protein moiety of the urinary HDL appeared to be large apolipoprotien fragments with molecular weights and isoelectric points similar to those of apo CII and apo CIII. The lower density classes of urinary lipoproteins also appeared to have lost apo E and apo C's and to have undergone partial proteolysis.

  8. [Small-cell lung cancer and rapidly fatal nephritic syndrome].

    Science.gov (United States)

    Yangui, I; Msaad, S; Smaoui, M; Makni, S; Kammoun, K; Khébir, A; Boudawara, T; Ayoub, A

    2007-10-01

    Nephrotic syndrome due to membranous glomerulonephritis is observed in 1 to 3% of patients with lung cancer. The nephrotic syndrome usually precedes the discovery of the causal tumor, but diagnosis can be concomitant or during the disease course. We describe a case of small-cell carcinoma of the lung without metastases revealed by a paraneoplastic nephrotic syndrome. Complete remission of the tumor was achieved with chemotherapy and radiotherapy with resolution of the nephrotic syndrome, but tumor progression occurred together with rapidly fatal renal failure. In this case, and the review of the literature, illustrate the association between paraneoplastic nephrotic syndrome and lung cancer, as well as the disease course and prognosis of the lung cancer and the accompanying glomerulopathy.

  9. EFFICACY OF THE IMMUNOSUPPRESSIVE THERAPY AGAINST THE NEPHROTIC SYNDROME AMONG CHILDREN

    OpenAIRE

    A.G. Timofeeva; T.V. Sergeeva; T.V. Margieva; T.S. Voznesenskaya; O.V. Komarova; A.N. Tsygin

    2007-01-01

    The researchers evaluated the efficacy of different immunosuppressive medications in case of steroid dependent and steroid resistant variants of the nephrtic syndrome among children: traditional alkylating agents (cyclophosphamide, chlorbutin), cyclosporine and mycophenolate mofetil. Cyclosporine proved to be most efficient in treatment against steroid dependent nephritic syndrome, as the researchers were more often able to cancel the steroids, while the recurrence of the nephritic syndrome d...

  10. Paraneoplastic Cushing Syndrome Due To Wilm's Tumor.

    Science.gov (United States)

    Faizan, Mahwish; Manzoor, Jaida; Saleem, Muhammad; Anwar, Saadia; Mehmood, Qaiser; Hameed, Ambreen; Ali, Agha Shabbir

    2017-05-01

    Paraneoplastic syndromes are rare disorders that are triggered by an altered immune system response to neoplasm. Paraneoplastic syndromes may be the first or the most prominent manifestations of cancer. Wilm's tumor is the most frequent pediatric renal malignancy and usually presents with abdominal mass. Unusual presentations like acquired von Willebrand disease, sudden death due to pulmonary embolism and Cushing syndrome have been described in the literature. Cushing syndrome, as the presenting symptom of a malignant renal tumor in children, is a very rare entity. Few case reports are available in the literature exploring the option of preoperative chemotherapy as well as upfront nephrectomy. We report a rare case of paraneoplastic Cushing syndrome due to a Wilm's tumor. Based on gradual decrease of postoperative weight, blood pressure, serum adrenocorticotropic hormone, and plasma cortisol levels, along with histological confirmation of Wilm's tumor, paraneoplastic Cushing syndrome due to Wilm's tumor was confirmed.

  11. [Value of determination of haptoglobin and α1-antitrypsin in predicting response to glucocorticoid therapy in children with primary nephrotic syndrome].

    Science.gov (United States)

    Yang, Juan; Zhang, Bi-Li

    2015-03-01

    To study the value of the determination of serum and urine haptoglobin (HP) and alpha 1-antitrypsin (AAT) in predicting the response to glucocorticoid therapy in children with primary nephrotic syndrome (PNS). A total of 84 children with PNS were classified to steroid-sensitive nephrotic syndrome (SSNS) (n=58) and steroid-resistant nephrotic syndrome (SRNS) groups (n=26). Forty healthy children were randomly selected for the control group. HP and AAT levels in blood and urinary samples were determined using ELISA. The efficiency of HP and AAT in predicting the response to glucocorticoid treatment of PNS was evaluated by the receiver operating characteristic (ROC) curve. Compared with the control group, both the SSNS and SRNS groups had significantly higher serum HP concentrations and urine AAT/Cr ratio before treatment (Pratio before treatment and after one week and four weeks of treatment (Phighest efficiency in predicting the response to glucocorticoid treatment of PNS at the concentration of 37.935 mg/mL, with the sensitivity and specificity being 92.3% and 86.2% respectively. Urine AAT/Cr ratio had the highest prediction efficiency at 0.0696, with the sensitivity and specificity being 100% and 79.3% respectively. ROC curve analysis of serum HP combined with urine AAT/Cr ratio showed a better prediction efficiency, with the sensitivity and specificity being 92.3% and 96.6% respectively. The increase in serum HP level or urine AAT/Cr ratio may indicate glucocorticoid resistance in the early stage of PNS. A combination of the two can achieve better efficiency in the prediction of SRNS.

  12. Vitamin D3 Adjuvant Treatment Stimulate Interleukin-10 Expression in Children with Nephrotic Syndrome Without Affecting to Clinical Outcome and Glucocorticoid Receptor Expression

    Directory of Open Access Journals (Sweden)

    Husnul Asariati

    2014-11-01

    Full Text Available Idiopathic nephrotic syndrome (INS is the most glomerular disease that occurred in childhood with high rate morbidity. Glucocorticoid is drug of choice for INS and responsiveness to this drug determined prognosis.Glucocorticoid upregulate transcription of anti-inflammatory cytokines such as IL-4 and IL-10. IL-10 is an anti-inflammatory cytokine and has multiple role in immune response include modulate Th1/Th2 response. Vitamin D3 interact with glucocorticoid signaling. Administered active form of vitamin D3 increase dexamethasone-induced IL-10 expression by regulatory T cells in steroid resistant asthmatic patient. Here we showed increase of CD4+ IL10+ expression after treatment both prednisone only and combination prednison with vitamin D3. Both in new-onset NS or rare relaps NS, combination treatment prednisone + vitamin D3 increase CD4+ IL10+ expression significantly compared to prednisone-only treated group (p= 0.003, which first group (new-onset nephrotic syndrome + prednisone and vitamin D3 treatment showed the most CD4+ IL10+ expression enhancement (9.53±3.89. However this study failed to show a correlation between CD4+ IL-10+ expression after prednisone and vitamin D3 treatment with clinical outcome (linear regression test, p= 0,125. This study also showed that there was a no correlation between CD4+ IL-10+ expression and CD3+ GR expression after prednison + vitamin D3 treatment (p= 0.088. CD4+ IL-10+ expression in new-onset and rarely relapsing nephrotic syndrome patients higher in prednisone + vitamin D3 treated group than prednisone-only treated group. There is no correlation between CD4+ IL-10+ expression and CD3+ GR expression nor CD4+ IL-10+ expression and clinical outcome.

  13. Abdominal complications in black and Indian children with nephrotic ...

    African Journals Online (AJOL)

    Abdominal complications were detected and investigated in 19 (10%) of 191 children with nephrotic syndrome who experienced 35 episodes of these complications. Fourteen children were Indian with steroid-responsive nephrotic syndrome, and 5 were black, of whom 4 had membranous nephropathy and 1 focal ...

  14. Urinary Tract Infections in Children with Primary Nephrotic ...

    African Journals Online (AJOL)

    Urinary Tract Infections in Children with Primary Nephrotic Syndrome and Acute Glomerulonephritis. ... West African Journal of Medicine ... Abstract. BACKGROUND: The occurrence of urinary tract infection (UTI) in children with Nephrotic syndrome (NS) has been widely reported by various workers, but not much has been ...

  15. EFFICACY OF THE IMMUNOSUPPRESSIVE THERAPY AGAINST THE NEPHROTIC SYNDROME AMONG CHILDREN

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    A.G. Timofeeva

    2007-01-01

    Full Text Available The researchers evaluated the efficacy of different immunosuppressive medications in case of steroid dependent and steroid resistant variants of the nephrtic syndrome among children: traditional alkylating agents (cyclophosphamide, chlorbutin, cyclosporine and mycophenolate mofetil. Cyclosporine proved to be most efficient in treatment against steroid dependent nephritic syndrome, as the researchers were more often able to cancel the steroids, while the recurrence of the nephritic syndrome developed less often. In the event of the steroid resistant nephritic syndrome, the full/partial remission was achieved among more than 80% patients also treated by cyclosporine. In case of the proliferating forms of the steroid resistant nephritic syndrome, the positive outcome was achieved among all the children under observation, if mycophenolate mofetil and steroids were further applied. Therapy by the conventional alkylating agents (cyclophosphamide, chlorbutin proved to be less efficient both for the relief from the steroid dependence and persistent remission of the steroid resistant nephritic syndrome.Key words: nephritic syndrome, immunosuppressive therapy, alkylating agents, cyclosporine, mycophenolate mofetil, children.

  16. Clinical value of NPHS2 analysis in early- and adult-onset steroid-resistant nephrotic syndrome.

    Science.gov (United States)

    Santín, Sheila; Tazón-Vega, Bárbara; Silva, Irene; Cobo, María Ángeles; Giménez, Isabel; Ruíz, Patricia; García-Maset, Rafael; Ballarín, José; Torra, Roser; Ars, Elisabet

    2011-02-01

    To date, very few cases with adult-onset focal segmental glomerulosclerosis (FSGS) carrying NPHS2 variants have been described, all of them being compound heterozygous for the p.R229Q variant and one pathogenic mutation. Mutation analysis was performed in 148 unrelated Spanish patients, of whom 50 presented with FSGS after 18 years of age. Pathogenicity of amino acid substitutions was evaluated through an in silico scoring system. Haplotype analysis was carried out using NPHS2 single nucleotide polymorphism and microsatellite markers. Compound heterozygous or homozygous NPHS2 pathogenic mutations were identified in seven childhood-onset steroid-resistant nephrotic syndrome (SRNS) cases. Six additional cases with late childhood- and adult-onset SRNS were compound heterozygotes for p.R229Q and one pathogenic mutation, mostly p.A284V. p.R229Q was more frequent among SRNS cases relative to controls (odds ratio=2.65; P=0.02). Significantly higher age at onset of the disease and slower progression to ESRD were found in patients with one pathogenic mutation plus the p.R229Q variant in respect to patients with two NPHS2 pathogenic mutations. NPHS2 analysis has a clinical value in both childhood- and adult-onset SRNS patients. For adult-onset patients, the first step should be screening for p.R229Q and, if positive, for p.A284V. These alleles are present in conserved haplotypes, suggesting a common origin for these substitutions. Patients carrying this specific NPHS2 allele combination did not respond to corticoids or immunosuppressors and showed FSGS, average 8-year progression to ESRD, and low risk for recurrence of FSGS after kidney transplant.

  17. Angiotensin-converting enzyme genotype is not a significant genetic risk factor for idiopathic nephrotic syndrome in Croatian children.

    Science.gov (United States)

    Batinić, Danko; Sertić, Jadranka; Ćorić, Marijana; Konjevoda, Paško; Batinić, Danica; Milošević, Danko

    2015-01-01

    The association of the angiotensin-converting enzyme (ACE) gene insertion/deletion (I/D) polymorphism with idiopathic nephrotic syndrome (INS) is controversial. Only scarce information on European populations is available. The aim of the study was to investigate the distribution of the ACE gene I/D polymorphism and its impact on INS in children from Croatia. Ninety-five children with INS were investigated: 30 with minimal change disease (MCD), 35 with mesangial proliferative glomerulonephritis (MesPGN) and 30 with focal segmental glomerulosclerosis (FSGS). The control group consisted of 73 healthy adults. ACE gene was analyzed using the PCR method. The results were correlated with clinical features, renal morphology and response to immunosuppresive therapy. There was no correlation of ACE genotype with gender, age of the disease onset, level of proteinuria, presence of hematuria or hypertension, and GFR at onset of the disease. No statistically significant differences in ACE genotype or allele frequencies between the controls and whole group of patients, MCD group, MesPGN group, FSGS group, steroid sensitive (SS) patients, steroid resistant (SR) patients, as well as each other, were found, although DD genotype tended to be more frequent in FSGS patients, SR patients, and frequent relapsers. Among 11 children treated with cyclophosphamide the D allele was significantly higher among non-responders (p = 0.003). DD genotype is not a genetic risk factor for acquiring INS nor significant phenotype modifier regarding to clinical and pathohistological picture and response to steroids in Croatian children. The potential application of ACE genotyping in predicting cyclophosphamide response deserves further investigation. © 2015 S. Karger AG, Basel.

  18. Efficacy and safety of cyclosporine versus tacrolimus in steroid and cyclophosphamide resistant nephrotic syndrome: A prospective study

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    N Prasad

    2018-01-01

    Full Text Available Calcineurin inhibitors (CNIs are the preferred drugs for treatment of childhood steroid-resistant nephrotic syndrome (SRNS who are also resistant to cyclophosphamide (CYC. Although few studies have shown a benefit of one over the other, efficacy and safety of either CNIs (tacrolimus [TAC] or cyclosporine [CSA] in this special population remained to be assessed in long-term studies. Forty-five children with SRNS who were also resistant to CYC (CYC-SRNS from January 2006 to June 2011, were included in the study. Patients were treated with CNI either TAC or CSA based on 1:1 allocations and were prospectively observed. Patients who were nonresponsive to CNIs had been treated with mycophenolate mofetil. Outcomes were measured in terms of remission of NS, adverse effects of drugs, and progression of disease. After 6 months of treatment, 16/23 (69.5% patients on CSA achieved remission and 18/22 (81.8% on TAC achieved remission (P = 0.3. The side effects hypertrichosis, and gum hyperplasia were significantly less in TAC group as compared to CSA group (P < 0.001. The 1-, 2-, 3-, 4-, and 5-year estimated renal survival (doubling of serum creatinine as event in CSA group was 96%, 91%, 85%, 54%, and 33% and in TAC group was 96%, 95%, 90%, 89%, and 79%, respectively (P = 0.02. Although TAC and CSA are equally efficacious, TAC has significantly less side effects. The long-term outcome of renal function was significantly better in patients who were treated with TAC as compared to CSA.

  19. A single-gene cause in 29.5% of cases of steroid-resistant nephrotic syndrome.

    Science.gov (United States)

    Sadowski, Carolin E; Lovric, Svjetlana; Ashraf, Shazia; Pabst, Werner L; Gee, Heon Yung; Kohl, Stefan; Engelmann, Susanne; Vega-Warner, Virginia; Fang, Humphrey; Halbritter, Jan; Somers, Michael J; Tan, Weizhen; Shril, Shirlee; Fessi, Inès; Lifton, Richard P; Bockenhauer, Detlef; El-Desoky, Sherif; Kari, Jameela A; Zenker, Martin; Kemper, Markus J; Mueller, Dominik; Fathy, Hanan M; Soliman, Neveen A; Hildebrandt, Friedhelm

    2015-06-01

    Steroid-resistant nephrotic syndrome (SRNS) is the second most frequent cause of ESRD in the first two decades of life. Effective treatment is lacking. First insights into disease mechanisms came from identification of single-gene causes of SRNS. However, the frequency of single-gene causation and its age distribution in large cohorts are unknown. We performed exon sequencing of NPHS2 and WT1 for 1783 unrelated, international families with SRNS. We then examined all patients by microfluidic multiplex PCR and next-generation sequencing for all 27 genes known to cause SRNS if mutated. We detected a single-gene cause in 29.5% (526 of 1783) of families with SRNS that manifested before 25 years of age. The fraction of families in whom a single-gene cause was identified inversely correlated with age of onset. Within clinically relevant age groups, the fraction of families with detection of the single-gene cause was as follows: onset in the first 3 months of life (69.4%), between 4 and 12 months old (49.7%), between 1 and 6 years old (25.3%), between 7 and 12 years old (17.8%), and between 13 and 18 years old (10.8%). For PLCE1, specific mutations correlated with age of onset. Notably, 1% of individuals carried mutations in genes that function within the coenzyme Q10 biosynthesis pathway, suggesting that SRNS may be treatable in these individuals. Our study results should facilitate molecular genetic diagnostics of SRNS, etiologic classification for therapeutic studies, generation of genotype-phenotype correlations, and the identification of individuals in whom a targeted treatment for SRNS may be available. Copyright © 2015 by the American Society of Nephrology.

  20. [Pefloxacin as a first-line treatment for nephrotic syndrome in minimal glomerular lesions in the adult. Multicenter study of 32 patients].

    Science.gov (United States)

    Pruna, A; Barka, A; Nochy, D; Hauet, T; Boulanger, H; Landais, P

    1997-01-01

    Minimal change nephrotic syndrome (MCNS) is the most frequent single cause of nephrotic syndrome occurring both in adults and children. Although it appears to be a self-limiting disorder (10% spontaneous remissions within the fortnight following the initial flare), MCNS displays a high rate of complications during the nephrotic period (10 to 15% cases) and prompts one to treat patients as early as possible. Corticosteroids are currently used as first-line treatment. A 16 weeks full-dose steroid course (1 mg/kg/day) usually induces remission in 75% MCNS in adults. Nevertheless, duration of treatment (9 months) and occurrence of relapses despite a slowly tapering dosage schedule, expose patients to steroids side-effects. Immunosuppressive drugs are recommended in case of steroid resistance and their side-effects are not harmless. Therefore, an alternative to steroids or immunosuppressives would lend a serious helping hand in MCNS management. The present work is dealing with pefloxacin efficacy in 40% MCNS in adults. Thirty-two MCNS adult patients were treated in a national multicenter study. A short-duration pefloxacin course (4 to 6 weeks) allowed partial or complete remission in 13 out of 32 cases. Thus far, this effect was undescribed for this class of drugs. Pefloxacin belongs to antibacterial agents of the fluoroquinolone family and is active against Gram negative Enterobacteria species. Fluoroquinolones also act on eukaryotic cells as lymphocytes and chondrocytes and alter IL2, gamma IFN and integrin expression. Although their precise mode of action is unknown in this kind of immunological disorder, fluoroquinolones might represent an alternative to steroids in some adult form of MCNS. However, predictive criteria for sensitivity to fluoroquinolones are currently not available and further controlled studies would be helpful using fluoroquinolones as first-line treatment in all the MCNS.

  1. Differential alteration in peripheral T-regulatory and T-effector cells with change in P-glycoprotein expression in Childhood Nephrotic Syndrome: A longitudinal study.

    Science.gov (United States)

    Prasad, Narayan; Jaiswal, Akhilesh K; Agarwal, Vikas; Yadav, Brijesh; Sharma, Raj Kumar; Rai, Mohit; Singh, Harshit; Chaturvedi, Saurabh; Singh, Ajay

    2015-04-01

    Childhood Idiopathic Nephrotic Syndrome (INS) responds to glucocorticoid therapy, however, 60-80% of patients relapse and some of them become steroid non responsive. INS may occur because of T cell dysfunction, abnormal cytokines and podocytopathies which reverse on steroid treatment. The reason of relapses could be imbalances in T cells phenotypes and respective cytokines. Herein, we hypothesize that relapses in INS may occur due to imbalance in T-regulatory and T-effector cell with their respective cytokines and overexpression of P-gp on lymphocytes. The frequency of peripheral blood CD4(+)CD25(+)FoxP3(+) Treg, CD4(+)IFN-γ(+) Th1 and CD4(+)IL-4(+) Th2 lymphocytes and their respective cytokines and P-gp expression on peripheral blood lymphocytes (PBLs) were analyzed in INS patients at baseline (n=26), during remission (n=24) and at relapse (n=15). Compared to baseline, the frequency of Tregs was significantly increased at remission and decreased during relapse. In contrast, the frequency of Th1 and Th2 lymphocytes was significantly decreased during remission and increased at the time of relapse. Similarly, expression of P-gp was significantly high at baseline and at the time of relapse as compared to remission. Levels of cytokines IL-10 and TGF-β in the supernatant of stimulated PBMCs was increased during remission and decreased during relapse. In contrast, levels of IFN-γ and IL-4 were decreased during remission and increased at the time of relapse. Steroid therapy in INS induces decreased P-gp expression on PBLs along with increased frequency and cytokine response of T-regulatory cells, and reduced frequency and respective cytokine response of Th1 and Th2 cells during remission. However, reversal in the frequency and respective cytokines of T-regs, Th1 and Th2, and P-gp expression on PBLs occurs during relapses on follow-up. Copyright © 2015 Elsevier Ltd. All rights reserved.

  2. Effects of probenecid on the pharmacokinetics of mizoribine and co-administration of the two drugs in patients with nephrotic syndrome.

    Science.gov (United States)

    Utsunomiya, Y; Hara, Y; Ito, H; Okonogi, H; Miyazaki, Y; Hashimoto, Y; Hosoya, T

    2010-11-01

    Probenecid (PRB) is an agent that reduces the systemic level of uric acid, and has the ability to inhibit the renal tubular secretion of agents that are co-administered with it. In this study, we evaluated the effects of PRB co-administered with mizoribine (MZR) on the pharmacokinetics (PK) of MZR in 12 patients with nephrotic syndrome. The elimination rate constant (kel) was used as an indicator of changes in the PK of MZR when the secretion of MZR was inhibited by co-administration of PRB, in order to determine the extent to which MZR was influenced by PRB. In 4 of the 12 patients studied, kel decreased and the biological half-life (t1/2) of MZR was prolonged when co-administered with PRB, in comparison with the values when MZR was used alone, thus revealing that the PK of MZR was influenced by PRB. Co-administration of PRB with MZR appears to be effective in prolonging the biological half-life of MZR and enhancing its effect in patients with nephrotic syndrome, although further studies will be required to determine the optimal dosage of PRB and renoprotective effects.

  3. CYSTATIN S AS A MARKER OF CYCLOSPORINE A RENAL TOXICITY IN CHILDREN WITH NEPHROTIC SYNDROME

    Directory of Open Access Journals (Sweden)

    O.V. Komarova

    2010-01-01

    Full Text Available The use of cystatin С as a probable marker of renal toxicity of cyclosporine A in children with nephritic syndrome, treated with this medication for a long time, is the subject of an interest. The present study included 29 children treated with cyclosporine A, divided into 3 groups depending on the duration of treatment (< 1 year, 1–2 years and > 2 years. The increase of cystatin С level in these groups compared with cystatin С level in control group (10 children without diseases of kidney was marked. The highest level of cystatin С was detected in children treated with therapeutic doses of cyclosporine A (3–5 mg/kg daily over 1 year. Authors showed positive correlation between cystatin С level in serum and medication’s dose.Key words: children, nephritic syndrome, cystatin С, cyclosporine A.(Voprosy sovremennoi pediatrii — Current Pediatrics. 2010;9(1:131-135

  4. Insulin-like growth factors (IGFs) and IGF binding proteins, serum acid-labile subunit and growth hormone binding protein in nephrotic children.

    Science.gov (United States)

    Haffner, D; Tönshoff, B; Blum, W F; Vickers, M; Siebler, T; Cronin, M J; Baxter, R C; Mehls, O

    1997-09-01

    We hypothesized that the increased glomerular permeability to serum proteins in the nephrotic syndrome might lead to alterations of the somatotropic hormone axis, thereby contributing to growth failure and catabolism in the nephrotic state. The insulin-like growth factors (IGF)-I and -II and the IGF binding proteins (IGFBP)-1, -2 and -3 were analyzed in serum and urine of 21 children with the nephrotic syndrome and normal glomerular filtration rate. Mean age-related serum IGF-I levels by RIA (-0.53 +/- 0.34 SD) were slightly, but significantly (P excretion rate of both peptides was enhanced fivefold. By RIA, mean age-related serum IGFBP-1 (2.05 +/- 0.19 SD) and, even more pronounced, IGFBP-2 (5.97 +/- 0.65 SD) were clearly elevated despite a 12-fold and 2-fold increase of the respective urinary excretion rate. There was a tight and specific correlation between age-related serum IGFBP-2 levels and the degree of the nephrotic syndrome, as estimated by serum albumin levels (r = -0.78, P 150 kDa IGFBP ternary complex in nephrotic serum, which in the presence of normal concentrations of the acid-labile subunit by RIA appears to be due to a reduction of intact IGFBP-3. Serum levels of the high-affinity GH binding protein that presumably reflects GH receptor status in tissues were normal. In summary, total serum IGFs in children with the nephrotic syndrome are normal, but the binding of IGFs to IGFBPs in the circulation is altered with a shift from the 150 kDa IGFBP complex to an excess of low molecular weight IGFBPs. Because increased unsaturated high-affinity IGFBPs in nephrotic serum have the ability to inhibit IGF action on target tissues by competing with the type 1 IGF receptor for IGF binding, this alteration is likely to contribute to growth failure and tissue catabolism in the nephrotic state.

  5. Cerebral sinovenous thrombosis in a nephrotic child

    Directory of Open Access Journals (Sweden)

    Rodrigues Marcelo Masruha

    2003-01-01

    Full Text Available Nephrotic syndrome in infancy and childhood is known to be associated with a hypercoagulable state and thromboembolic complications, but cerebral sinovenous thrombosis (CST is a very rare and serious one, with only a few isolated reports in the literature. A case is presented of a 9-year-old boy with nephrotic syndrome that acutely developed signs and symptoms of intracranial hypertension syndrome. CST was diagnosed on cranial CT and MRI and he gradually recovered after treatment with anticoagulants. The diagnosis of CST should be considered in any patient with nephrotic syndrome who develops neurologic symptoms. The discussion of this case, coupled with a review of the literature, emphasizes that early diagnosis is essential for institution of anticoagulation therapy and a successful outcome. This report also illustrates the difficulties that may be encountered in managing such a patient.

  6. Short courses of daily prednisolone during upper respiratory tract infections reduce relapse frequency in childhood nephrotic syndrome.

    Science.gov (United States)

    Abeyagunawardena, Asiri S; Thalgahagoda, R S; Dissanayake, Pathum V; Abeyagunawardena, Shamali; Illangasekera, Y A; Karunadasa, Umeshi I; Trompeter, Richard S

    2017-08-01

    Relapses of childhood nephrotic syndrome (NS) are frequently precipitated by viral upper respiratory tract infections (URTIs). A review of the literature reveals that in patients with steroid-dependent NS on alternate day corticosteroids, a short course of daily corticosteroid therapy during the course of an URTI may reduce relapse frequency. To assess the effect of a short course of low-dose corticosteroid therapy during the course of an URTI on relapse frequency in patients with steroid-sensitive NS who have not been taking any treatment for a minimum period of 3 months. A double-blind placebo-controlled crossover trial was conducted on 48 patients with idiopathic NS who had not been receiving corticosteroid therapy for a minimum of 3 months. Patients were randomized into two groups. Group A received 5 days of daily prednisolone at 0.5 mg/kg at the onset of an URTI while group B received 5 days of placebo. Both groups were followed up for 1 year and the URTI-induced relapse frequency was noted. A crossover was performed during the next year, with group A receiving placebo and group B receiving prednisolone. Thirty-three patients completed the study. In the treatment group, 115 episodes of URTI led to 11 relapses while in the control group 101 episodes of URTI led to 25 relapses. There was no significant difference between the mean number of URTIs between the treatment and control groups. The treatment group had significantly less relapses compared to the control group (p = 0.014). Within the treatment group, 65.6% did not relapse, while the remainder had a single relapse. In contrast, only 40.6% of the control group remained in remission while 40.6% suffered a single relapse and 18.8% had two or more relapses. Prescribing a short course of daily corticosteroids during an URTI significantly reduces the frequency of URTI-induced relapse in patients with steroid-responsive NS who are off corticosteroid therapy.

  7. Vitamin D receptor gene TaqI and Apal polymorphisms and steroid responsiveness in childhood idiopathic nephrotic syndrome.

    Science.gov (United States)

    Al-Eisa, Amal A; Haider, Mohammad Z

    2016-01-01

    Vitamin D activity is controlled by vitamin D receptors (VDRs), which are affected by different genetic polymorphisms, including TaqI and Apal restriction fragment length polymorphisms (RFLPs), which have been reported to be associated with several diseases. The aim of this study was to determine the frequency and the association of VDR gene polymorphisms with idiopathic nephrotic syndrome (INS) and steroid responsiveness in Kuwaiti children. Genotypes of the VDR TaqI gene polymorphism and the Apal gene polymorphism were analyzed using polymerase chain reaction-RFLP in 78 INS patients and 56 matched controls. A total of 78 INS (62 steroid sensitive [SS] and 16 steroid resistant [SR]) patients with a mean age of 6.5±3.1 years were studied. Male:female ratio was 2:1. The TT genotype of VDR-TaqI polymorphism was detected in 41% of the INS patients compared to 42% of the controls (P=0.816). The heterozygous TC genotype was detected in 33% of INS patients compared to 46% of the controls (P=0.462). The CC genotype was detected in 25.6% of INS patients and 21% of the controls (P=0.719). The C-allele frequency, in its homozygous and heterozygous forms, was 71% in INS patients compared to 63% in the controls (P=0.342). Similarly, no significant difference was detected in terms of VDR-Apal polymorphism in INS patients compared to the controls for all the three genotypes (P=0.76, P=0.207, and P=0.364, respectively, for GG, GT, and TT genotypes). The T-allele frequency, in its homozygous and heterozygous forms, was 89% in INS patients compared to 93% in the controls (P=0.076). No significant difference was found in any of the allele frequencies between SS and SR subgroups when compared with each other or when compared to the controls. Our data do not support the use of VDR-TaqI or -Apal gene polymorphisms as genetic markers of INS nor do they predict steroid responsiveness in children with the disease.

  8. Pseudotumour cerebri syndrome due to cryptococcal meningitis.

    Science.gov (United States)

    Cremer, P D; Johnston, I H; Halmagyi, G M

    1997-01-01

    Three cases are reported of the pseudotumour cerebri syndrome-that is, intracranial hypertension without mass lesion or enlarged ventricles, due to cryptococcal meningitis. In these patients the papilloedema was successfully treated with optic nerve sheath decompression, and the intracranial hypertension with lumboperitoneal CSF shunting. These cases support the concept that pseudotumour cerebri is a syndrome of intracranial hypertension that can be due to any disorder producing obstruction of the CSF pathways at the level of the arachnoid villi. This concept is important because it directs therapy to normalise the intracranial pressure and preserve vision. Images PMID:9010409

  9. Significance of electron dense deposits in patients with minimal change nephrotic syndrome.

    Science.gov (United States)

    Kim, Sae Yoon; Lee, Sang Su; Kim, Myoung Uk; Lee, Jae Min; Kang, Seok Jeong; Kim, Yong Jin; Park, Yong Hoon

    2012-04-01

    Minimal change nephritic syndrome (MCNS) is characterized by a lack of obvious abnormalities on light microscopy, but its electron microscopic findings include the negative immunofluorescence findings and the diffuse effacement of the epithelial cell foot processes. Rarely the presence of electron dense deposits (EDDs) has been reported, but its clinical significance remains obscure. Eleven patients with MCNS who had the EDD deposited were enrolled in the current study. We compared the clinical characteristics, laboratory results and response to steroid treatment between the two group: the EDD group (n=11; the male-to-female ratio, 8:3) and the non-EDD group (n=13, 8:5). There were no significant differences in most of the laboratory results or response to steroid treatment between the two groups. The frequency of relapses per year was significantly higher in the EDD group (1.1±0.7 times vs. 0.5±0.6 times; p=0.023). These EDDs were found in the mesangium or paramesangium. With no respect to the characteristics of EDDs, our results showed that they did not cause poor treatment outcomes except for the annual frequency of relapse. Further large-scale studies are warrented to determine the immunologic and prognostic significance of EDDs in patients with MCNS.

  10. Comparison of Efficacy of Tacrolimus Versus Cyclosporine in Childhood Steroid-Resistant Nephrotic Syndrome.

    Science.gov (United States)

    Shah, Syed Sajid Hussain; Hafeez, Farkhanda

    2016-07-01

    To compare the efficacy of tacrolimus versus cyclosporine (Calcineurin Inhibitors) in the management of childhood steroid-resistant nephritic syndrome (SRNS). Quasi-experimental study. Department of Paediatric Nephrology at The Children's Hospital and Institute of Child Health, Lahore, from August 2014 to September 2015. Patients of either gender aged 1 - 12 years, with the diagnosis of mesangioproliferative glomerulonephritis (MesangioPGN), focal segmental glomerulosclerosis (FSGS) or minimal-change disease (MCD) were included. Patients were assigned into two groups, one given tacrolimus in dose of 0.1 - 0.2 mg/kg/day in two divided doses, and other given cyclosporine in dose of 150 - 200 mg/m2/day in two divided doses along with oral steroids 30 mg/m2/day in divided doses, followed by alternate day with tapering dosage. Trough drug levels were done with dose adjustment accordingly. Patients were monitored and followed for the response to treatment and adverse effects of these two calcineurin inhibitors. Atotal of 84 patients, 58% males and 42% females, were included in the study. The age ranged from 1.25 to 12 years. The most common histopathological diagnosis was MesangioPGN (69.04%), FSGS (21.4%), and MCD (9.52%). Complete response was seen in 80.95% and 97.6% patients treated with cyclosporine and tacrolimus, respectively. Partial response was in 19.05% patients treated with cyclosporine and 2.4% in patients with tacrolimus. The most common adverse effect with cyclosporine and tacrolimus was hypertrichosis in 80.95% and 2.38%, hypertension 16.66% and 11.9% respectively while gum hypertrophy with cyclosporine was seen in 26.19% patients. Tacrolimus was more efficacious than cyclosporine in achieving remission in childhood SRNS with insignificant adverse effects.

  11. A case of rheumatic fever with acute post-streptococcal glomerulonephritis and nephrotic syndrome caused by a cutaneous infection with beta-hemolytic streptococci

    Directory of Open Access Journals (Sweden)

    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.

  12. A 17-Year-Old With Steroid-Resistant Nephrotic Syndrome.

    Science.gov (United States)

    Band, Molly E; Sheldon, Candice; Brancato, John; Parikh, Nehal S; D'Alessandri-Silva, Cynthia

    2016-05-01

    A 17-year-old girl presented with facial swelling and shortness of breath to an outside emergency department. She was treated for an allergic reaction with steroids and antihistamines, and discharged from the hospital. Subsequently, she was referred as an outpatient to pediatric nephrology for recurrent edema and proteinuria. Initial laboratory workup by nephrology was significant for a normal complete blood count and reassuring electrolyte panel. Pertinent laboratories were a creatinine of 0.5 mg/dL (0.4-1.1 mg/dL) and an albumin 2.3 g/dL (3.5-5.0 g/dL). The urine protein-to-creatinine ratio was >7 (<0.2). A renal ultrasound showed symmetrically sized kidneys with normal echotexture. The patient's renal biopsy results were consistent with minimal change disease. Based on the biopsy results, prednisone was started. Due to a poor response to prednisone, an alternate immunomodulator therapy was selected. Her subsequent complete blood counts showed a downward trend of all cell lines and an elevated serum uric acid. Concurrently, she reported worsening fatigue, low back pain, nausea, vomiting, night sweats, and pruritus. More details of her case and the outcome are presented. Copyright © 2016 by the American Academy of Pediatrics.

  13. Acute kidney injury in idiopathic nephrotic syndrome of childhood is a major risk factor for the development of chronic kidney disease.

    Science.gov (United States)

    Yaseen, Afshan; Tresa, Vina; Lanewala, Ali Asghar; Hashmi, Seema; Ali, Irshad; Khatri, Sabeeta; Mubarak, Muhammed

    2017-11-01

    Acute kidney injury (AKI) is an important complication of idiopathic nephrotic syndrome (INS) and is associated with adverse outcomes, especially the development of chronic kidney disease (CKD). We aimed to determine the clinical profile of children with INS who developed AKI and its short-term outcome. This prospective study was conducted from March 2014 to October 2015. A total of 119 children of INS (age: 2-18 years) fulfilling the pediatric RIFLE criteria for the diagnosis of AKI were enrolled and followed up for 3 months to determine the outcome. Factors predisposing to CKD were studied. The mean age at presentation was 8.8 ± 3.59 years and males were 74 (62.2%). At presentation, 61 (51.3%) children were in Risk category, 43 (36.1%) in Injury category, and 15 (12.6%) in Failure category. Most of them (41.2%) had steroid-resistant nephrotic syndrome (SRNS) and focal segmental glomerulosclerosis (FSGS) on histopathology (33.6%). Infections were the major predisposing factor for AKI in 67 (56.3%) cases. Drug toxicity was the next common, found in 52 (43.7%) children. A total of 65 (54.6%) children recovered from AKI, while 54 (45.4%) did not. CKD developed in 49 (41.2%) non-recovered cases and 5 (4.2%) children succumbed to acute illness. SRNS, cyclosporine use, FSGS on histology, and drug toxicity were significant factors associated with the development of CKD. AKI associated with INS is a reversible condition in most cases but it can progress to CKD, especially among those who have SRNS, FSGS, and drug toxicity.

  14. Nephrotic syndrome due to lupus-like glomerulonephritis in an HIV-positive patient

    NARCIS (Netherlands)

    Wiegersma, J. S.; Franssen, C. F. M.; Diepstra, A.

    2017-01-01

    Lupus nephritis, a well-known complication in systemic lupus erythematosus, is characterised by a proliferative glomerulonephritis or membranous nephropathy along with a full-house immunofluorescence pattern on renal biopsy. There are very few exceptions in which similar histopathological findings

  15. ¿Existe un síndrome nefrótico corticosensible con lesión mínima hereditario? Is there a corticosensitive nephrotic syndrome with a hereditary minimal lesion?

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    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

  16. Childhood Nephrotic Syndrome

    Science.gov (United States)

    ... in treating kidney disease . A child should see a pediatric nephrologist, who has special training to take care ... in the body to become inflamed and leak hepatitis , inflammation of the liver caused by a virus human immunodeficiency virus (HIV), a virus that ...

  17. Nephrotic Syndrome in Adults

    Science.gov (United States)

    ... Kidney Disease Weight Management Liver Disease Urologic Diseases Endocrine Diseases Diet & Nutrition Blood Diseases Diagnostic Tests La información ... Kidney Disease Weight Management Liver Disease Urologic Diseases Endocrine Diseases Diet & Nutrition Blood Diseases Diagnostic Tests La información ...

  18. [Level of Th17 cell and CD4(+);CD25(+); Foxp3(+); regulatory T cell in peripheral blood mononuclear cells of primary nephrotic syndrome in children].

    Science.gov (United States)

    Wang, Li; Li, Qiu; Wang, Li-jia; Li, Xin

    2010-08-01

    To investigate the levels and functions of Th17 cells and CD4(+); CD25(+); Foxp3(+); regulate cells(Treg) and explore their role in pathogenesis of primary nephrotic syndrome (PNS) in children. Children with PNS were divided into simple type nephritic syndrome group (SNS) (n = 20), and nephritic type nephritic syndrome group (NNS) (n = 15). 20 healthy subjects were selected as control group. The circulating frequencies of Th17 cells and Treg were measured by FCM. Real-time PCR were used to analyze the mRNA expressions of RORC, IL-23p19 and Foxp3 in peripheral blood mononuclear cells. The serum of IL-1ß, IL-6, TGF-ß1 were measured by ELISA. Circulating frequencies of Th17 cells, the mRNA levels of RORC, IL-23p19 and the serum of IL-1ß, IL-6 were higher in SNS and NNS groups than control group (P 0.05). Imbalance of Th17 and Treg cells might contribute to the pathogenesis of PNS in children and have associated with clinical presentation, pathological type, glucocorticoid sensitivity and prognosis of the disease.

  19. Síndrome nefrótica córtico-sensível e diabetes mellitus tipo 1 de início simultâneo Simultaneous onset of steroid-sensitive nephrotic syndrome and type 1 diabetes

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    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.

  20. The roles of urine interleukin-13, CD80, CD28, matrix metalloproteinase-2 and granzyme B in the pathogenesis of childhood minimal change nephrotic syndrome

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    Cengiz Zeybek

    2014-09-01

    Full Text Available Objective: Minimal change disease (MCD is the most common cause of nephrotic syndrome in childhood but its pathogenesis remains poorly understood. A T-cell-derived factor or factors were initially alleged as contributing to the disease pathogenesis. However, podocyte CD80 expression is now a commonly discussed theory. The aim of this study was to investigate the pathogenesis of MCD by determining urine interleukin-13, CD80, CD28, matrix metalloproteinase-2 (MMP-2, and granzyme B levels. Methods: Thirty patients and thirty healthy children were evaluated in this study. Six patients had biopsy proven MCD. The remaining patients were considered to have MCD because of their age at time of diagnosis; response to steroid treatment, absence of gross hematuria, hypocomplementemia or renal failure; and normal blood pressures in the active stage. The nephrotic-phase urine interleukin-13, CD80, CD28, MMP-2, and granzyme B levels of all patients were compared with the remission-stage urine levels of the same patients and control subjects. The urine samples were collected immediately before the application of immunosuppressive drugs or other treatment modalities. Results: Significantly higher interleukin-13, CD80, CD28, and MMP-2 levels were observed in the relapse period compared with both the remission period and control subjects. There was a significant positive correlation between the active-stage urine interleukin-13 and CD80 levels (r=0.619, p<0.001. Conclusion: Interleukin-13, CD80, CD28, and MMP-2 seem to have roles in the pathogenesis of MCD and using inhibitors of these molecules in treatment of steroid and immunosuppressive-resistant MCD cases can be thought. J Clin Exp Invest 2014; 5 (3: 354-361

  1. The Roche Total Mycophenolic Acid® assay: An application protocol for the ABX Pentra 400 analyzer and comparison with LC–MS in children with idiopathic nephrotic syndrome

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    François Parant

    2017-04-01

    Full Text Available Background: For TDM of mycophenolate acid (MPA, the Roche Total Mycophenolic Acid® assay based on the inhibition of recombinant inosine monophosphate dehydrogenase (IMPDH has been shown to be a simple and reliable alternative to chromatographic methods. We have adapted this assay on the ABX Pentra 400 analyzer (HORIBA. Objective: To investigate the analytical performances of the Roche Total Mycophenolic Acid® assay on the ABX Pentra 400 and to compare it to an LC-MS method using samples from children with nephrotic syndrome treated with mycophenolate mofetil (MMF. Material and methods: Configuration of the open-channel on the ABX Pentra 400 was based on the Roche MPA assay package insert. Precision was determined as described in the CLSI protocol EP5-A2. Comparison with the LC-MS method was performed using 356 plasma samples from 42 children with nephrotic syndrome (8 h pharmacokinetic profiles. Results: The enzymatic assay demonstrated high precision. The %CV for Within Run Imprecision ranged from 5.5% at 1.2 mg/L to 1.5% at 14.1 mg/L and Total Imprecision ranged from 9.3% to 2.5%. The method comparison with plasma samples from children yielded overall a good correlation and a good agreement between both methods. The Passing Bablok regression analysis showed the following results: [Roche MPA assay]=1.058 [MPA LC-MS] −0.06; rho=0.996. Conclusion: The Roche Total Mycophenolic Acid® assay is adaptable to the ABX Pentra 400 analyzer, and demonstrates accurate and precise measurement of MPA in plasma obtained from children with nephrotic syndrome. Keywords: Enzyme assay, Inosine monophosphate dehydrogenase, Mycophenolic acid, Nephrotic syndrome

  2. Síndrome nefrótico primario: análisis clínico patológico Primary nephrotic syndrome: pathological and clinical analysis

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    Ronald Armando Noguera Valverde

    2007-09-01

    Full Text Available El principal hallazgo histológico en las biopsias de los niños con síndrome nefrótico primario son lesiones mínimas que responden al tratamiento con esteroides. Se pueden presentar otras lesiones histológicas que no responden a los esteroides, algunas de evolución mortal. Otros pacientes muestran dependencia a esteroides y la consiguiente aparición de efectos indeseables. Con el objetivo de describir la correlación entre hallazgos clínicos, la respuesta inicial a esteroides y la histología en niños con síndrome nefrótico primario, se revisaron retrospectivamente los expedientes clínicos de 35 pacientes con síndrome nefrótico primario, con biopsias realizadas en el Hospital Nacional de Niños, en Costa Rica, entre enero de 1993 y diciembre de 1997. Se encontraron lesiones mínimas en el 45,7 % de las biopsias; glomerulonefritis mesangial difusa en el 28,6 %; glomeruloesclerosis focal y segmentaria en el 22,9 %; glomerulonefritis membranosa en el 2,9 %; y se encontró que la hematuria y la elevación de la creatinina están asociadas significativamente con la resistencia inicial a esteroides (p The main histological finding in biopsies from children with primary nephrotic syndrome are minimal lesions sensitive to steroid treatment. There may be other histological steroid-resistent lesions, some of them leading to fatal consequences. Other patients progress with steroid dependence and occurence of some adverse effects. With the objective of describing the corelation among clinical findings, initial response to steroids and the histology in children with primary nephrotic syndrome, the medical histories of 35 patients suffering primary nephrotic syndrome, whose biopsies had been performed at the National Child Hospital of Costa Rica from January, 1993 to December,1997, were prospectively reviewed. Sligt lesions were found in 45,7% of biopsies; diffuse mesangial glomerulonephritis in 28,6%; focal and segmentary glomerulosclerosis in

  3. Abdominal Compartment Syndrome due to OHSS

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    Firoozeh Veisi

    2012-03-01

    Full Text Available Abdominal compartment syndrome is a dangerous clinical situation, usually following abdominal injuries&operations. It is seldom observed in patients with gynecologic and obstetric problems. Abdominalcompartment syndrome may be consequence ovarian hyperstimulation syndrome. A 28-year-old womanpresented as a sever ovarian hyperstimulation.The increased IAP indicated that OHSS may beconsidered a compartment syndrome. Abdominal compartment syndrome needs laparotomy orparacentesis for reduction of pressure.

  4. [Multicenter survey of diagnostic and therapeutic status in Chinese childhood patients with steroid-sensitive, relaping/steroid-dependent nephrotic syndrome].

    Science.gov (United States)

    2014-03-01

    Nephrotic syndrome (NS) was recognized as a clinical syndrome caused by massive proteinuria, leading to hypoalbuminemia and edema. Primary nephrotic syndrome, accounted for about 90% of NS, seems to be one of common urologic-kidney diseases in childhood. The diagnosis and treatment of NS had so far failed to be unified nationwide. The purpose of this research was to survey the diagnosis and treatment status of steroid-sensitive, relaping/steroid-dependent nephrotic syndrome (SSNS, RNS/SDNS) in Chinese children and to evaluate the applicability and promotion of the evidence-based guideline on diagnosis and treatment of SSNS, RNS/SDNS (for trial implementation) conducted by The Subspecialty Group of Nephrology, the Society of Pediatrics, Chinese Medical Association in 2009. Questionnaires concerning children with SSNS, RNS/SDNS were designed by The Subspecialty Group of Nephrology, Society of Pediatrics, Chinese Medical Association and distributed to the doctors of 37 centers of 27 cities, 4 municipalities and 2 autonomous regions. A nationwide survey was conducted and data of hospitalized children (younger than 18 years of age) with SSNS, RNS/SDNS in 2008 and 2011 were analyzed retrospectively. Children diagnosed as primary nephrotic syndrome (PNS) accounted for 20.0% (7 444/37 134) of the hospitalized cases with urologic-kidney diseases in the corresponding time period; 3 725 cases were enrolled into this study. The male to female ratio was 3.2: 1 and the median age was 4.1 years (Q = 4.2 years); 3 186 (85.5%) cases were diagnosed as simple NS and 539 (14.5%) as nephritic NS; 2 825 (75.8%) PNS cases were the first episode and 1 050 (28.2%) cases were the relapse; 221 (5.9%) cases were diagnosed as SDNS and 749 (20.1%) as frequently relapse NS (FRNS) . At NS diagnosis, the proteinuria was 3+-4+ (3 260 cases, 87.5%) , median quantitive proteinuria for 24 hours was 112.0 mg/kg (Q = 109.3 mg/kg) , mean plasma albumin was (17.9 ± 6.4) g/L and mean cholesterol was (10

  5. [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.

  6. C5b-9-targeted molecular MR imaging in rats with Heymann nephritis: a new approach in the evaluation of nephrotic syndrome.

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    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.

  7. Spectrum of renal biopsy finding in idiopathic nephrotic syndrome in children: An 18-month retrospective analysis at a tertiary care pediatric nephrology center in North Karnataka, India

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    Prithi Rajendra Inamdar

    2017-01-01

    Full Text Available Context: Idiopathic nephrotic syndrome (NS is the most common glomerular nephropathy in pediatrics, with great variation in patient characteristics in different regions of the world. Difficult childhood NS poses a great diagnostic and management challenge for the treating nephrologists. The histopathological features of NS have been extensively studied by various authors from different regions of the world with a wide variation in the histopathological distribution. We aimed to describe the spectrum of histopathological diagnosis in childhood NS from North Karnataka, India, where there is a high incidence of consanguinity. Aims: The aim of this study is to study the spectrum of histopathology in children who underwent a renal biopsy in our tertiary care pediatric nephrology center. Settings and Design: Retrospective observational study at a tertiary care pediatric nephrology center over 1 year from January 2013 to June 2015. Subjects and Methods: The medical records of all children diagnosed as NS were retrospectively reviewed for demographic data, clinical course, and histopathological diagnosis. Statistical Analysis Used: Descriptive statistics. Results: Twenty-five children underwent biopsy in the study period. Seventeen (68% were male as compared to 8 (32% females. Thirteen (52% of biopsied patients showed minimal change NS, 6 (24% had focal segmental glomerulosclerosis, and 3 (14% patients showed IgA nephropathy while 1 patient had mesangioproliferative glomerulonephritis, IgM nephropathy, and Finnish type NS each. Conclusions: Minimal change nephritic syndrome is the major biopsy finding in our part of the country even in steroid-resistant NS. This finding needs to be confirmed with studies with bigger sample size.

  8. 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

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    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

  9. HLA-DQA1 and APOL1 as Risk Loci for Childhood-Onset Steroid-Sensitive and Steroid-Resistant Nephrotic Syndrome.

    Science.gov (United States)

    Adeyemo, Adebowale; Esezobor, Christopher; Solarin, Adaobi; Abeyagunawardena, Asiri; Kari, Jameela A; El Desoky, Sherif; Greenbaum, Larry A; Kamel, Margret; Kallash, Mahmoud; Silva, Cynthia; Young, Alex; Hunley, Tracey E; de Jesus-Gonzalez, Nilka; Srivastava, Tarak; Gbadegesin, Rasheed

    2018-03-01

    Few data exist for the genetic variants underlying the risk for steroid-sensitive nephrotic syndrome (SSNS) in children. The objectives of this study were to evaluate HLA-DQA1 and APOL1 variants as risk factors for SSNS in African American children and use classic HLA antigen types and amino acid inference to refine the HLA-DQA1 association. Case-control study. African American children with SSNS or steroid-resistant nephrotic syndrome (SRNS) were enrolled from Duke University and centers participating in the Midwest Pediatric Nephrology Consortium. Genetic variants in HLA-DQA1 (C34Y [rs1129740]; F41S [rs1071630]) and APOL1 high-risk alleles. SSNS and SRNS. Direct sequencing for the HLA-DQA1 and APOL1 variants in 115 African American children (65 with SSNS and 50 with SRNS). Imputation of classic HLA alleles and amino acids was done in 363 South Asian children. The 2 HLA-DQA1 variants were significantly associated with SSNS in African American children (C34Y: P=5.7 × 10 -11 ; OR, 3.53; 95% CI, 2.33-5.42; F41S: P=1.2 × 10 -13 ; OR, 4.08; 95% CI, 2.70-6.28), but not with SRNS (C34Y: P=0.6; F41S: P=0.2). APOL1 high-risk variants were not associated with SSNS (P=0.5) but showed significant associations with SRNS (P=1.04 × 10 -7 ; OR, 4.17; 95% CI, 2.23-7.64). HLA-DQA1*0201, HLA-DQB1*0201, and HLA-DRB1*0701 were the classic HLA alleles with the most significant associations with SSNS risk. The most significantly associated amino acid positions were HLA-DQα1 56 and 76 (both P=2.8 × 10 -7 ). Conditional analysis revealed that these variants most likely account for the observed association. Modest sample size and limited statistical power to detect small to moderate effect sizes. Children studied may not be representative of all African American children in the United States. HLA-DQA1 is a risk locus for SSNS, but not SRNS, in African American children, consistent with its role in SSNS risk in children of European, Asian, and African ancestries. There is little

  10. 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.

  11. 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.

  12. 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.

  13. Paraneoplastic cushing syndrome due to wilm's tumor

    International Nuclear Information System (INIS)

    Faizan, M.; Anwar, S.; Hameed, A.; Manzoor, J.; Saleem, M.; Mehmood, Q.; Ali, A. S.

    2017-01-01

    Paraneoplastic syndromes are rare disorders that are triggered by an altered immune system response to neoplasm. Paraneoplastic syndromes may be the first or the most prominent manifestations of cancer. Wilm's tumor is the most frequent pediatric renal malignancy and usually presents with abdominal mass. Unusual presentations like acquired von Willebrand disease, sudden death due to pulmonary embolism and Cushing syndrome have been described in the literature. Cushing syndrome, as the presenting symptom of a malignant renal tumor in children, is a very rare entity. Few case reports are available in the literature exploring the option of preoperative chemotherapy as well as upfront nephrectomy. We report a rare case of paraneoplastic Cushing syndrome due to a Wilm's tumor. Based on gradual decrease of postoperative weight, blood pressure, serum adrenocorticotropic hormone, and plasma cortisol levels, alongwith histological confirmation of Wilm's tumor, paraneoplastic Cushing syndrome due to Wilm's tumor was confirmed. (author)

  14. [A genetic childhood disease with consequences in adult life: the Denys-Drash syndrome

    NARCIS (Netherlands)

    Lowik, M.; Berk-Mortel, F. van den; Noordam, C.; Hamersvelt, H.W. van; Heuvel, L.P.W.J. van den; Levtchenko, E.N.

    2005-01-01

    In a 17-year-old woman with absent sexual development and a congenital nephrotic syndrome leading to renal failure, the Denys-Drash syndrome was diagnosed after development of an ovarian dysgerminoma. The Denys-Drash syndrome is characterised by the triad: progressive nephropathy due to diffuse

  15. Serum erythropoietin level in anemic and non-anemic nephrotic children with normal kidney functions

    International Nuclear Information System (INIS)

    Moustafa, A.M.E.; Moawad, A.T.; Gad, A.A.; Ahmed, S.M.

    2005-01-01

    Nephrotic syndrome (NS) is associated with a significant alteration in protein metabolism. While lowering the concentration of certain proteins, the disease often raises the level of certain other proteins. The current study aimed to investigate the serum erythropoietin (EPO) levels in children with NS either anemic or non-anemic and to compare them to children with iron deficiency anemia (IDA) and healthy controls with normal hemoglobin level (NHB). Sixteen nephrotic children with anemia (NS-A) and 15 nephrotic children with normal hemoglobin level (NS-NHB) were examined and compared with 10 children with iron deficiency anemia (IDA) and 10 healthy controls (NHB). Circulating serum EPO levels, blood indices and iron status were measured in nephrotic patients with anemia (NS-A) and compared to those nephrotic patients with normal HE (NS-NHB). Most NS-A children were steroid resistant. The NS-A children showed greater EPO levels than those without anemia (21.01 ±4.02 mlU/ml versus 9.18 ± 0.79 mlU/ml; P < 0.001) but their response to treatment of anemia was inappropriately low when compared to IDA (EPO 96.9 ±4.9 mlU/ml) despite similar HB concentration. A significant positive correlation was observed between serum EPO and serum albumin in NS-A (r = 0.84, P < 0.001) and in NS-NHB group (r = 0.89, P < 0.001). Moreover, a significant positive correlation was observed between serum EPO and HB in the nephrotic groups indicating a blunted EPO response to anemia in NS-A (r 0.63, P < 0.05) and in NS-NHB group (r = 0.80, P < 0.001). In conclusion, anemia is a common feature of NS and is present even before the worsening of kidney function. Depletion of the iron stores due to loss of iron and transferrin in urine due to massive proteinurea may contribute to the development of anemia, but it was found that iron replacement was ineffective alone

  16. Nicolau syndrome due to hyaluronic acid injections.

    Science.gov (United States)

    Andre, Pierre; Haneke, Eckart

    2016-08-01

    Six cases of vascular compromise after hyaluronic injection are reported. Clinical symptoms realized a Nicolau syndrome, which is characterized by immediate pain, livedoid pattern and a few days later by the appearance of scabs and skin necrosis. This type of complication is rare, but may be dramatic and injectors must be aware of that. A thorough knowledge of facial anatomy is mandatory to avoid the risky facial areas. The use of a flexible cannula instead of a sharp needle has much less risk of hurting vessels and must be preferred. The support of the patient is discussed and a treatment protocol is proposed.

  17. Short course daily prednisolone therapy during an upper respiratory tract infection in children with relapsing steroid-sensitive nephrotic syndrome (PREDNOS 2): protocol for a randomised controlled trial

    Science.gov (United States)

    2014-01-01

    Background Relapses of childhood steroid-sensitive nephrotic syndrome (SSNS) are treated with a 4- to 8-week course of high-dose oral prednisolone, which may be associated with significant adverse effects. There is a clear association between upper respiratory tract infection (URTI) and relapse development. Previous studies in developing nations have suggested that introducing a 5- to 7-day course of daily prednisolone during an URTI may prevent a relapse developing and the need for a treatment course of high-dose prednisolone. The aim of PREDNOS 2 is to evaluate the effectiveness of a 6-day course of daily prednisolone therapy during an URTI in reducing the development of a subsequent relapse in a developed nation. Methods/design The subjects will be 300 children with relapsing SSNS (≥2 relapses in preceding year), who will be randomised to receive either a 6-day course of daily prednisolone or no change to their current therapy (with the use of placebo to double blind) each time they develop an URTI over 12 months. A strict definition for URTI will be used. Subjects will be reviewed at 3, 6, 9 and 12 months to capture data regarding relapse history, ongoing therapy and adverse effect profile, including behavioural problems and quality of life. A formal health economic analysis will also be performed. The primary end point of the study will be the incidence of URTI-related relapse (3 days of Albustix +++) following the first infection during the 12-month follow-up period. DNA and RNA samples will be collected to identify a potential genetic cause for the disease. Subjects will be recruited from over 100 UK centres with the assistance of the Medicines for Children Research Network. PREDNOS 2 is funded by the National Institute for Health Research Health Technology Assessment Programme (11/129/261). Discussion We propose that PREDNOS 2 will be a pivotal study that will inform the future standard of care for children with SSNS. If it is possible to reduce the

  18. Short course daily prednisolone therapy during an upper respiratory tract infection in children with relapsing steroid-sensitive nephrotic syndrome (PREDNOS 2): protocol for a randomised controlled trial.

    Science.gov (United States)

    Webb, Nicholas J A; Frew, Emma; Brettell, Elizabeth A; Milford, David V; Bockenhauer, Detlef; Saleem, Moin A; Christian, Martin; Hall, Angela S; Koziell, Ania; Maxwell, Heather; Hegde, Shivram; Finlay, Eric R; Gilbert, Rodney D; Booth, Jenny; Jones, Caroline; McKeever, Karl; Cook, Wendy; Ives, Natalie J

    2014-04-27

    Relapses of childhood steroid-sensitive nephrotic syndrome (SSNS) are treated with a 4- to 8-week course of high-dose oral prednisolone, which may be associated with significant adverse effects. There is a clear association between upper respiratory tract infection (URTI) and relapse development. Previous studies in developing nations have suggested that introducing a 5- to 7-day course of daily prednisolone during an URTI may prevent a relapse developing and the need for a treatment course of high-dose prednisolone. The aim of PREDNOS 2 is to evaluate the effectiveness of a 6-day course of daily prednisolone therapy during an URTI in reducing the development of a subsequent relapse in a developed nation. The subjects will be 300 children with relapsing SSNS (≥2 relapses in preceding year), who will be randomised to receive either a 6-day course of daily prednisolone or no change to their current therapy (with the use of placebo to double blind) each time they develop an URTI over 12 months. A strict definition for URTI will be used. Subjects will be reviewed at 3, 6, 9 and 12 months to capture data regarding relapse history, ongoing therapy and adverse effect profile, including behavioural problems and quality of life. A formal health economic analysis will also be performed. The primary end point of the study will be the incidence of URTI-related relapse (3 days of Albustix +++) following the first infection during the 12-month follow-up period. DNA and RNA samples will be collected to identify a potential genetic cause for the disease. Subjects will be recruited from over 100 UK centres with the assistance of the Medicines for Children Research Network.PREDNOS 2 is funded by the National Institute for Health Research Health Technology Assessment Programme (11/129/261). We propose that PREDNOS 2 will be a pivotal study that will inform the future standard of care for children with SSNS. If it is possible to reduce the disease relapse rate effectively and

  19. Steven-Johnson syndrome due to unknown drugs [corrected].

    Science.gov (United States)

    Shivamurthy, Raghu Prasada M; Kallappa, Ravindra; Reddy, Shashikala G H; Rangappa, Druva Kumar B

    2012-01-01

    Steven-Johnson syndrome may be considered as a cytotoxic immune reaction to drugs, infections etc. This is a case report of Steven-Johnson syndrome due to an ayurvedic preparation which was used in the treatment of mental retardation in a young girl.

  20. Steven-Johnson syndrome due to ayurvedic drugs

    OpenAIRE

    Shivamurthy, Raghu Prasada M.; Kallappa, Ravindra; Reddy, Shashikala G. H.; Rangappa, Druva Kumar B.

    2012-01-01

    Steven?Johnson syndrome may be considered as a cytotoxic immune reaction to drugs, infections etc. This is a case report of Steven?Johnson syndrome due to an ayurvedic preparation which was used in the treatment of mental retardation in a young girl.

  1. Clinical predictors of lacunar syndrome not due to lacunar infarction

    Directory of Open Access Journals (Sweden)

    Comes Emili

    2010-05-01

    Full Text Available Background Lacunar syndrome not due to lacunar infarct is poorly characterised. This single centre, retrospective study was conducted to describe the clinical characteristics of patients with lacunar syndrome not due to lacunar infarct and to identify clinical predictors of this variant of lacunar stroke. Methods A total of 146 patients with lacunar syndrome not due to lacunar infarction were included in the "Sagrat Cor Hospital of Barcelona Stroke Registry" during a period of 19 years (1986-2004. Data from stroke patients are entered in the stroke registry following a standardized protocol with 161 items regarding demographics, risk factors, clinical features, laboratory and neuroimaging data, complications and outcome. The characteristics of these 146 patients with lacunar syndrome not due to lacunar infarct were compared with those of the 733 patients with lacunar infarction. Results Lacunar syndrome not due to lacunar infarct accounted for 16.6% (146/879 of all cases of lacunar stroke. Subtypes of lacunar syndromes included pure motor stroke in 63 patients, sensorimotor stroke in 51, pure sensory stroke in 14, atypical lacunar syndrome in 9, ataxic hemiparesis in 5 and dysarthria-clumsy hand in 4. Valvular heart disease, atrial fibrillation, sudden onset, limb weakness and sensory symptoms were significantly more frequent among patients with lacunar syndrome not due to lacunar infarct than in those with lacunar infarction, whereas diabetes was less frequent. In the multivariate analysis, atrial fibrillation (OR = 4.62, sensorimotor stroke (OR = 4.05, limb weakness (OR = 2.09, sudden onset (OR = 2.06 and age (OR = 0.96 were independent predictors of lacunar syndrome not due to lacunar infarct. Conclusions Although lacunar syndromes are highly suggestive of small deep cerebral infarctions, lacunar syndromes not due to lacunar infarcts are found in 16.6% of cases. The presence of sensorimotor stroke, limb weakness and sudden onset in a patient

  2. Steroids combined with levothyroxine to treat children with idiopathic nephrotic syndrome: a retrospective single-center study.

    Science.gov (United States)

    Guo, Qing-Yin; Zhu, Qing-Jun; Liu, Yu-Feng; Zhang, Hui-Juan; Ding, Ying; Zhai, Wen-Sheng; Ren, Xian-Qing; Zhang, Jian; Zhang, Xia; Yang, Meng

    2014-06-01

    The clinical efficacy and safety of low-dose levothyroxine in the treatment of idiopathic nephritic syndrome accompanied by thyroid dysfunction have not been established. One hundred and sixty-four patients were divided into three groups according to the levels of thyroid hormone and treatment. The thyroid status, efficacy and adverse reactions of thyroid treatment were observed in each group. Thyroid dysfunction was found in 73 patients. 40 cases were treated with steroids combined with levothyroxine. Proteinuria, cholesterol and thyroid-stimulating hormone (TSH) levels were significantly higher in patients with thyroid dysfunction, whereas serum albumin and free and total T3 and T4 levels were lower than those of euthyroid patients. The time for proteinuria remission in patients receiving levothyroxine therapy was shorter and their serum albumin higher than for patients without levothyroxine treatment. Thyroid hormonal changes are related to the degree of both proteinuria and serum albumin in patients with INS. Combined treatment with low-dose levothyroxine supplementation and steroids in children with INS and thyroid dysfunction is associated with reduced proteinuria and increased plasma albumin compared with patients treated with steroids only.

  3. 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. Copyright © 2015 Elsevier B.V. All rights reserved.

  4. Urinary Tract Infections in Children with Primary Nephrotic

    African Journals Online (AJOL)

    user1

    ABSTRACT. BACKGROUND: The occurrence of urinary tract infection. (UTI) in children with Nephrotic syndrome (NS) has been widely reported by various workers, but not much has been documented about its occurrence among children with acute glomerulonephritis (AGN). Hence, the level of susceptibility to UTI by both ...

  5. APOL1-associated glomerular disease among African-American children: a collaboration of the Chronic Kidney Disease in Children (CKiD) and Nephrotic Syndrome Study Network (NEPTUNE) cohorts.

    Science.gov (United States)

    Ng, Derek K; Robertson, Catherine C; Woroniecki, Robert P; Limou, Sophie; Gillies, Christopher E; Reidy, Kimberly J; Winkler, Cheryl A; Hingorani, Sangeeta; Gibson, Keisha L; Hjorten, Rebecca; Sethna, Christine B; Kopp, Jeffrey B; Moxey-Mims, Marva; Furth, Susan L; Warady, Bradley A; Kretzler, Matthias; Sedor, John R; Kaskel, Frederick J; Sampson, Matthew G

    2017-06-01

    Individuals of African ancestry harboring two variant alleles within apolipoprotein L1 ( APOL1 ) are classified with a high-risk (HR) genotype. Adults with an HR genotype have increased risk of focal segmental glomerulosclerosis and chronic kidney disease compared with those with a low-risk (LR) genotype (0 or 1 variants). The role of APOL1 risk genotypes in children with glomerular disease is less well known. This study characterized 104 African-American children with a glomerular disease by APOL1 genotype in two cohorts: the Chronic Kidney Disease in Children (CKiD) and Nephrotic Syndrome Study Network (NEPTUNE). Among these subjects, 46% had an HR genotype with a similar age at cohort enrollment. For APOL1 HR children, the median age of disease onset was older (CKiD: 4.5 versus 11.5 years for LR versus HR; NEPTUNE: 11 versus 14 years for LR versus HR, respectively) and preterm birth was more common [CKiD: 27 versus 4%; NEPTUNE: 26 versus 12%; combined odds ratio 4.6 (95% confidence interval: 1.4, 15.5)]. Within studies, HR children had lower initial estimated glomerular filtration rate (eGFR) (CKiD: 53 versus 69 mL/min/1.73 m 2 ; NEPTUNE: 74 versus 94 mL/min/1.73 m 2 ). Longitudinal eGFR decline was faster among HR children versus LR (CKiD: -18 versus -8% per year; NEPTUNE: -13 versus -3% per year). Children with an HR genotype in CKiD and NEPTUNE seem to have a more aggressive form of glomerular disease, in part due to a higher prevalence of focal segmental glomerulosclerosis. These consistent findings across independent cohorts suggest a common natural history for children with APOL1 -associated glomerular disease. Further study is needed to determine the generalizability of these findings. © The Author 2016. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved.

  6. Mugwort-Mustard Allergy Syndrome due to Broccoli Consumption

    Directory of Open Access Journals (Sweden)

    Yuri Sugita

    2016-01-01

    Full Text Available Pollen-food allergy syndrome (PFAS is a relatively rare form of food allergy which develops in individuals who are sensitized to pollen. Tree pollens, especially birch pollen, frequently induce PFAS; however, the incidence of PFAS due to grass or weed pollens such as ragweed or mugwort is relatively rare. Mugwort-mustard allergy syndrome (MMAS is an example of a PFAS in which individuals sensitized to mugwort may develop an allergy to mustard and experience severe reactions. We herein describe a case of MMAS due to broccoli consumption.

  7. nephrotic syndrome in namibian children

    African Journals Online (AJOL)

    standard, including oedema, serum albumin below 30 g/l. (levels were mostly below 25 g/l,) hypercholesterolaemia and heavy proteinuria (> than 1000 mg/m'). .... be related to inland or coastal factors, with the coastal areas having higher rates.' However, the inland Namibian (Ovambo) group, with a high incidence, seems ...

  8. Genetic Aspects of Nephrotic Syndrome

    DEFF Research Database (Denmark)

    Joshi, Shivani

    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...... with SSNS are rare, but do indicate a genetic predisposition. In study III we investigated a potential genetic linkage in 9 European SSNS families with at least two affected siblings. Using SNP6 whole genome linkage analysis we could establish linkage of the disease phenotype to chromosome 15 with a maximum...... immunosuppressive treatment in future patients with the same genotype. Our findings also demonstrate that inheritance of different alleles at independent genetic loci in NS may contribute to the disease phenotype. SSNS is clinically distinct from SRNS and its pathogenesis is still unclear. Our study showed linkage...

  9. LYMPHOMA AND THE NEPHROTIC SYNDROME*

    African Journals Online (AJOL)

    B., Department of Medicine, Universiry of Cape Town and Groote Schuur Hospital, Cape Town ... II lesion is produced by the antigen-antibody complex ... lymphocyte. A gradation could be traced from this cell to a more primitive frankly nucleolated type. This blast cell was not fully characteristic of a lymphoblast and the.

  10. Acute glomerulonephritis mimicking nephrotic syndrome

    African Journals Online (AJOL)

    2016-01-04

    Jan 4, 2016 ... Abstract: Background: Acute post infectious glomerulonephritis. (APIGN) describes a wide range of glomerulonephritis character- ized by an immunologic response of the kidney to varieties of infec- tious agent commonly bacteria. It is characterized by an abrupt on- set of haematuria, moderate oedema ...

  11. Heterochromia iridis and Horner's syndrome due to paravertebral neurilemmoma.

    Science.gov (United States)

    Sayed, A K; Miller, B A; Lack, E E; Sallan, S E; Levey, R H

    1983-01-01

    A case of heterochromia iridis and Horner's syndrome is reported in a 7-year old girl with paravertebral neurilemmoma. These clinical findings can be useful in the early diagnosis of mediastinal tumors in the paravertebral axis. While typically associated with neuroblastoma, these findings can be due to tumors which are inately benign--in this case neurilemmoma. The mechanism for heterochromia is briefly discussed.

  12. Neuroblastoma Presenting with Acute Kidney Injury, Hyponatremic-Hypertensive-Like Syndrome and Nephrotic Proteinuria in a 10-Month-Old Child

    Directory of Open Access Journals (Sweden)

    Giovanni Maria Poggi

    2011-08-01

    Full Text Available Neuroblastoma is the most common extracranial solid tumor in childhood. Its presenting signs and symptoms may be highly variable, depending on the location of the primary tumor and its local or metastatic diffusion and, rarely, with paraneoplastic syndrome such as opsoclonus-myoclonus-ataxia syndrome and gastrointestinal disturbances, due to autoantibodies or to aberrant secretion of vasoactive intestinal peptide. Herein we describe a 10-month-old child with neuroblastoma presenting with a complex clinical picture characterized by acute kidney injury manifested by renal insufficiency and signs and symptoms of tubulointerstitial damage, with polyuria, polydipsia, glucosuria, aminoaciduria and hypochloremic metabolic alkalosis, and of glomerular damage with heavy proteinuria. Imaging study documented a suprarenal mass enveloping the aorta and its abdominal and renal ramifications and bilaterally renal veins. This clinical picture shows some analogies with the hyponatremic-hypertensive syndrome concerning the renovascular disease; however, in absence of systemic arterial hypertension, the heavy proteinuria and the polyuria could be explained by sectional increased intraglomerular pressure, due to local renal blood vessels constriction. Hypochloremic metabolic alkalosis probably developed because of local production of renin, responsible of renin-angiotensin-aldosterone system activation, but above all because of chloride loss through sweating. The long lasting dehydration, due to vomiting, sweating and polyuria, caused prolonged prerenal failure evolving in proximal tubular damage manifestations.

  13. Hybrid management of Paget-Schroetter syndrome due to thoracic outlet syndrome.

    Science.gov (United States)

    De Caridi, Giovanni; Massara, Mafalda; Greco, Michele; Villari, Simona; Squillaci, Domenico; Spinelli, Francesco

    2016-02-01

    Paget-Schroetter syndrome due to thoracic outlet syndrome is a rare but potentially disabling condition that generally affects young patients otherwise healthy. The prompt diagnosis and treatment of Paget-Schroetter syndrome is necessary to avoid major morbidity and long-term disability. The more modern treatment paradigm reported in the current literature consists of hybrid procedures with surgical decompression of the thoracic outlet and endovascular techniques to potentially improve long-term vein patency. However, there seems to be no consensus in the literature with regard to the timing and precise nature of active management, and there is presently no agreed protocol for the optimum management of Paget-Schroetter syndrome. Controversy exists partly because no randomised controlled studies are present in literature. We present a case of Paget-Schroetter syndrome due to thoracic outlet syndrome in a young male patient submitted to a multimodal procedure.

  14. A Case with Repeated Recurrent Acute Coronary Syndrome due to Pseudoephedrine Use: Kounis Syndrome

    Directory of Open Access Journals (Sweden)

    Metin Çeliker

    2014-01-01

    Full Text Available Allergic reaction-associated acute coronary syndrome picture is defined as Kounis syndrome. Although drug use is the most common cause of allergic reaction, foods and environmental factors may also play a role in the etiology. Herein, a case with acute coronary syndrome that developed two times at 8-month interval due to pseudoephedrine use for upper respiratory tract infection is presented.

  15. Anton's syndrome due to cerebrovascular disease: a case report

    Directory of Open Access Journals (Sweden)

    Maddula Mohana

    2009-09-01

    Full Text Available Abstract Introduction Anton's syndrome describes the condition in which patients deny their blindness despite objective evidence of visual loss, and moreover confabulate to support their stance. It is a rare extension of cortical blindness in which, in addition to the injury to the occipital cortex, other cortical centres are also affected, with patients typically behaving as if they were sighted. Case presentation We present a case report of an 83-year-old white woman with cortical blindness as a result of bilateral occipital lobe infarcts. Despite her obvious blindness, illustrated by her walking into objects, the patient expressed denial of visual loss and demonstrated confabulation in her accounts of her surroundings, consistent with a diagnosis of Anton's syndrome. Conclusions A suspicion of cortical blindness and Anton's syndrome should be considered in patients with atypical visual loss and evidence of occipital lobe injury. Cerebrovascular disease is the most common cause of Anton's syndrome, as in our patient. However, any condition that may result in cortical blindness can potentially lead to Anton's syndrome. Recovery of visual function will depend on the underlying aetiology, with cases due to occipital lobe infarction after cerebrovascular events being less likely to result in complete recovery. Management in these circumstances should accordingly focus on secondary prevention and rehabilitation.

  16. Differentiation of reversible ischemia from end-stage renal failure in nephrotic children with 131I-hippurate dynamic scintigraphy

    International Nuclear Information System (INIS)

    Hattner, R.S.; Maltz, H.E.; Holliday, M.A.

    1977-01-01

    In renal failure associated with the nephrotic syndrome, therapeutic strategy is highly dependent upon the cause of the renal failure. Dynamic hippurate scintigraphy was studied in five pediatric patients. Four had nephrotic syndrome, and of these, three had acute renal failure. The fifth patient had end-stage renal failure. Specific alteration in renal hippurate kinetics offers a noninvasive assessment of renal failure in this clinical setting

  17. A CLINICORADIOLOGICAL STUDY OF MIDDLE LOBE SYNDROME DUE TO TUBERCULOSIS

    Directory of Open Access Journals (Sweden)

    Saurabh Karmakar

    2016-09-01

    Full Text Available BACKGROUND Although pulmonary tuberculosis is a common disease in India, tuberculosis of right middle lobe is infrequent. Tuberculosis of the right middle lobe leading to chronic collapse is a cause of Right Middle Lobe syndrome. METHODS The patients attended Pulmonary Medicine Outdoor at Era’s Lucknow Medical College, Lucknow from April 2015 to March 2016. The purpose of this study is to describe the clinicoradiological features of patients of middle lobe syndrome due to tuberculosis. All patients presented with cough with or without expectoration, fever, chest pain, haemoptysis and constitutional symptoms like loss of appetite and weight. Chest X-ray PA view revealed ill-defined opacity abutting the right cardiac border. HRCT thorax was done in each case. The diagnosis of tuberculous aetiology was based on (1 History of chronic cough and fever, not responding to antibiotic therapy and constitutional symptoms, (2 A positive tuberculin test using 2 TU of PPD RT 23 and (3 Detection of acid fast bacilli by direct smear or Mycobacterium tuberculosis by polymerase chain reaction in bronchoalveolar lavage. RESULTS Out of 10 patients, 4 (40% were males and 6 (60% were females. The mean ages of the males were 55.8 years and females were 60.8 years and overall mean age was 59 years. Most of the patients were females and belonged to the middle age and old age group. ATT was started in all the patients. CONCLUSIONS Right middle lobe syndrome predominantly affects the older population and the female gender. Although tuberculosis is a common disease in India, Middle Lobe Syndrome is a very rare presentation of the disease. Due to non-specific symptoms and usually normal chest X-ray PA view in Right Middle Lobe Syndrome, we should keep a high index of suspicion to diagnose the condition.

  18. Fractures and Fanconi syndrome due to prolonged sodium valproate use.

    Science.gov (United States)

    Dhillon, N; Högler, W

    2011-06-01

    Sodium valproate (VPA) is commonly used to treat epilepsy in children. Renal dysfunction is a rare side eff ect but can present as tubulopathy such as Fanconi syndrome. We report on an 8-year-old disabled girl with myoclonic epilepsy who was referred for investigation of recurrent low impact fractures of the distal femur which were initially thought to be caused by her severe immobility. However, she was subsequently found to have hypophosphataemia secondary to Fanconi syndrome due to prolonged VPA use. After VPA withdrawal renal function and serum phosphate levels normalised and X-rays improved dramatically. The possibility of drug-induced osteoporosis and fractures should always be considered in disabled children, even in the presence of severe immobility.

  19. Cushing's syndrome due to interaction between inhaled corticosteroids and itraconazole.

    Science.gov (United States)

    Bolland, Mark J; Bagg, Warwick; Thomas, Mark G; Lucas, Jennifer A; Ticehurst, Rob; Black, Peter N

    2004-01-01

    To report a case of an interaction between inhaled corticosteroids and itraconazole causing iatrogenic Cushing's syndrome and provide a review of the relevant literature. A 70-year-old white woman on long-term treatment with high-dose inhaled corticosteroids for asthma was diagnosed as having Scedosporium apiospermum infection of the skin and subcutaneous tissues. As a result, she was treated with itraconazole for 2 months. She subsequently developed Cushing's syndrome due to a probable cytochrome P450-mediated interaction between itraconazole and budesonide. She also had secondary adrenal insufficiency requiring prolonged treatment with replacement hydrocortisone. Budesonide is a potent glucocorticoid that is metabolized in the liver by the CYP3A4 isoenzyme to inactive metabolites. Itraconazole is a potent cytochrome P450 inhibitor. It can inhibit the metabolism of oral or inhaled corticosteroids, producing cortisol excess leading to Cushing's syndrome and adrenal insufficiency. An assessment of causality indicated a possible adverse interaction between itraconazole and budesonide. The combination of itraconazole and inhaled corticosteroids is increasingly being used to treat conditions such as allergic bronchopulmonary aspergillosis. Clinicians need to be aware of the potential for an interaction between such a combination.

  20. Cavernous sinus syndrome due to osteochondromatosis in a cat.

    Science.gov (United States)

    Perazzi, Anna; Bernardini, Marco; Mandara, Maria T; De Benedictis, Giulia M; De Strobel, Francesca; Zotti, Alessandro

    2013-12-01

    A 1-year-old sexually intact male Korat cat was referred for ophthalmological consultation due to anisocoria. Mydriasis with external ophthalmoplegia and absence of pupillary light responses in the right eye and nasofacial hypalgesia were seen. Cavernous sinus syndrome (CSS) was suspected. Bilateral deformities of the jaw and phalangeal bones, severe spinal pain and abnormal conformation of the lumbar spine were also present. Radiographic examination revealed several mineralised masses in the appendicular and axial skeleton, indicative of multiple cartilaginous exostoses. For further investigation of the CSS-related neurological deficits, the cat underwent computed tomography (CT) examination of the skull. CT images revealed a non-vascularised, calcified, amorphous mass originating from the right lateral skull base and superimposing on the sella turcica. Based on the severity of diffuse lesions and owing to the clinical signs of extreme pain, the cat was euthanased. A diffuse skeletal and intracranial osteochondromatosis was diagnosed histologically.

  1. Nephrotic hyperlipidemia and atherothrombotic events in Nigerian ...

    African Journals Online (AJOL)

    Background Nephrotic hyperlipidaemia is a risk factor for atherothrombotic cardiovascular events. Directional changes in fractional lipidaemia appear to be more relevant. This study is aimed at determining the nature of association of nephrotic hyperlipidemia and atherothrobiotic event among. Nigerians. Method 51 adult ...

  2. Glucocorticoid-induced osteoporosis in the lumbar spine, forearm, and mandible of nephrotic patients

    DEFF Research Database (Denmark)

    Olgaard, K; Storm, Tina; van Wowern, N

    1992-01-01

    The long-term effects of high dose steroid treatment with either prednisone (PDN) or deflazacort (DFZ) were examined on various parts of the skeleton in 29 patients with nephrotic syndrome. All had normal skeleton at the start of the steroid treatment. At the beginning, PDN was given as 80 mg/day...

  3. Reflex sympathetic dystrophy syndrome due to arteriovenous fistula.

    Science.gov (United States)

    Unek, Ilkay Tugba; Birlik, Merih; Cavdar, Caner; Ersoy, Rifki; Onen, Fatos; Celik, Ali; Camsari, Taner

    2005-10-01

    A patient with end-stage renal disease presented with reflex sympathetic dystrophy syndrome (RSDS) on her left hand 1 month after arteriovenous fistula (AVF) surgery. Magnetic resonance angiography revealed steal syndrome at the AVF level. Bone scintigraphy revealed early-stage RSDS. We considered that arterial insufficiency because of steal phenomenon following AVF surgery and underlying occlusive arterial disease triggered RSDS development.

  4. Subcutaneous Implantable Cardioverter Defibrillator Lead Failure due to Twiddler Syndrome

    NARCIS (Netherlands)

    Kooiman, Kirsten M.; Brouwer, Tom F.; van Halm, Vokko P.; Knops, Reinoud E.

    2015-01-01

    We present a case of Twiddler syndrome in a patient with a subcutaneous implantable cardioverter defibrillator (S-ICD). The patient presented herself to the outpatient clinic with pain in the left chest. Chest x-ray confirmed Twiddler syndrome and ICD read-out revealed lead failure resulting in

  5. Angelman Syndrome Due to UBE3A Gene Mutation.

    Science.gov (United States)

    Goswami, Jyotindra Narayan; Sahu, Jitendra Kumar; Singhi, Pratibha

    2017-12-18

    A 12-y-old boy presented with developmental delay, autism, epilepsy, limb tremors and behavioral problems which posed a diagnostic challenge. Though his clinical profile and electroencephalogram were suggestive of Angelman syndrome, initial genetic tests were unyielding. Exome sequencing revealed a previously unreported mutation of Ubiquitin Protein Ligase E3A (UBE3A) gene, confirming the diagnosis of Angelman syndrome. The case is aimed to sensitize pediatricians about Angelman syndrome and to highlight the role of sequential investigations in establishing the diagnosis.

  6. Iatrogenic Cushing's Syndrome Due to Topical Ocular Glucocorticoid Treatment.

    Science.gov (United States)

    Fukuhara, Daisuke; Takiura, Toshihiko; Keino, Hiroshi; Okada, Annabelle A; Yan, Kunimasa

    2017-02-01

    Iatrogenic Cushing's syndrome (CS) is a severe adverse effect of systemic glucocorticoid (GC) therapy in children, but is extremely rare in the setting of topical ocular GC therapy. In this article, we report the case of a 9-year-old girl suffering from idiopathic uveitis who developed CS due to topical ocular GC treatment. She was referred to the ophthalmology department with a complaint of painful eyes, at which time she was diagnosed with bilateral iridocyclitis and started on a treatment of betamethasone sodium phosphate eye drops. Six months after the initiation of topical ocular GC treatment, she was referred to our pediatric department with stunted growth, truncal obesity, purple skin striate, buffalo hump, and moon face. Because her serum cortisol and plasma adrenocorticotropic hormone levels were undetectable, she was diagnosed with iatrogenic CS. After the doses of topical ocular GC were reduced, the clinical symptoms of CS were improved. The fact that the amount of topical ocular GC with our patient was apparently less than that of similar previous cases tempted us to perform genetic analysis of her NR3C1 gene. We found that our patient had a single heterozygous nucleotide substitution in the 3' untranslated region of the NR3C1 gene, which may explain why she developed CS. However, additional investigations are required to determine if our findings can be extrapolated to other patients. In conclusion, clinicians should be aware that even extremely low doses of topical ocular steroid therapy can cause iatrogenic CS. Copyright © 2017 by the American Academy of Pediatrics.

  7. Serotonin Syndrome Due to Overdose Intake of SSRI

    OpenAIRE

    Yavuz Orak; Sevgi Akbulut

    2015-01-01

    Serotonin syndrome is a drug side effect resulting from serotonergic hyperactivity. The severity of its symptoms can be mild and overlooked and sometimes it may cause life-threatening serious consequences. This syndrome is caused by the administration of one or more drugs having serotonergic activity. This case is a 25-year-old female patient who attempted suicide by ingesting an overdose of her prescription medications: 60 units of 100-mg Faver (Fluvoxamine), 20 units of 50-mg Setral (Setral...

  8. RRH: envenoming syndrome due to 200 stings from Africanized honeybees

    Directory of Open Access Journals (Sweden)

    Guilherme Almeida Rosa da Silva

    2013-02-01

    Full Text Available Envenoming syndrome from Africanized bee stings is a toxic syndrome caused by the inoculation of large amounts of venom from multiple bee stings, generally more than five hundred. The incidence of severe toxicity from Africanized bee stings is rare but deadly. This report reveals that because of the small volume of distribution, having fewer stings does not exempt a patient from experiencing an unfavorable outcome, particularly in children, elderly people or underweight people.

  9. Placenta accreta: adherent placenta due to Asherman syndrome

    DEFF Research Database (Denmark)

    Engelbrechtsen, Line; Langhoff-Roos, Jens; Kjer, Jens Jørgen

    2015-01-01

    It is important to be aware of the risk of abnormally invasive placenta in patients with a history of Asherman syndrome and uterine scarring. A prenatal diagnosis by ultrasonography is useful when planning of mode of delivery.......It is important to be aware of the risk of abnormally invasive placenta in patients with a history of Asherman syndrome and uterine scarring. A prenatal diagnosis by ultrasonography is useful when planning of mode of delivery....

  10. Horner Syndrome Due to Intrathoracic Multinodular Goiter (Case Report

    Directory of Open Access Journals (Sweden)

    Selda Korkmaz

    2013-09-01

    Full Text Available Horner syndrome is characterized by miosis, ptosis, facial anhydrosis and enophtalmus and is caused by a lesion along the oculosymphathetic pathway from the hypothalamus to the eye. The long course of the oculosympathetic pathway predisposes it to a wide variety of pathologic processes, ranging from harmless vascular headaches to life-threatening conditions such as carotid artery dissection or malignancy. Thyroid neoplasms, benign or malign, are among unusual causes of Horner syndrome and represent 1.3 - 7.8% of cases. Especially, early diagnosis of benign thyroid neoplasms such as multinodular goiter has particular importance because of its reversiblity with appropriate treatment. We report a case of Horner syndrome which has been developed as a complication of intrathoracic multinodular goiter

  11. [Endoscopic treatment of bleeding due to Mallory-Weiss syndrome].

    Science.gov (United States)

    Pantsyrev, Iu M; Fedorov, E D; Timofeev, M E; Mikhalev, A I

    2003-01-01

    Experience in diagnosis and treatment of 276 patients with Mallory--Weiss syndrome (MWS) is presented. Definition of the syndrome and brief historical review (from first description of disruption of esophageal-gastric connection to first report of successful hemostasis through endoscope) are given. Particular attention is devoted to etiology and pathogenesis of this syndrome. Clinical material and picture of disease, results of endoscopic diagnosis are analyzed, efficacy of endoscopic hemostasis and prophylaxis of hemorrhage is demonstrated. Indications to various methods of endoscopic hemostasis are regarded, results of treatment and lethal outcomes are analyzed. It is demonstrated that endoscopic procedures are the method of choice in the treatment of this disease reducing emergency surgery rate to 0.4%.

  12. 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

  13. Serotonin Syndrome Due to Overdose Intake of SSRI

    Directory of Open Access Journals (Sweden)

    Yavuz Orak

    2015-08-01

    Full Text Available Serotonin syndrome is a drug side effect resulting from serotonergic hyperactivity. The severity of its symptoms can be mild and overlooked and sometimes it may cause life-threatening serious consequences. This syndrome is caused by the administration of one or more drugs having serotonergic activity. This case is a 25-year-old female patient who attempted suicide by ingesting an overdose of her prescription medications: 60 units of 100-mg Faver (Fluvoxamine, 20 units of 50-mg Setral (Setraline, and 10 units of 20-mg Paxil (Paroxetine.

  14. latrogenic chronic compartment syndrome of leg due to ruptured ...

    African Journals Online (AJOL)

    A 45 year old male complaining of chronic pain and swelling of his left calf with features suggestive of chronic compartment syndrome following therapeutic aspiration of a popliteal cyst has been presented. It is a rare type of presentation which has not been reported to the knowledge of the authors. Review of literature and

  15. Junctional chiasmatic syndrome due to large anterior communicating artery aneurysm

    OpenAIRE

    Maruthi Kesani; Pavan Kumar Pelluru; Suchanda Bhattacharjee; Rajesh Alugolu; A K Purohit

    2017-01-01

    Even though aneurysm involving the anterior communicating artery (A Com A) was common in clinical practice, producing compressive symptoms such as visual loss was rare. We report a case, in which patient had gradually progressive visual loss with features of the junctional chiasmatic syndrome, imaging revealed partially thrombosed large A Com A aneurysm. Intraoperatively, aneurysm was found compressing the optic chiasm and right optic nerve, following clipping and decompression of the optic n...

  16. Outbreak of exogenous Cushing's syndrome due to unlicensed medications.

    Science.gov (United States)

    Azizi, Fereidoun; Jahed, Adel; Hedayati, Mehdi; Lankarani, Mahnaz; Bejestani, Hadi Shahrad; Esfahanian, Fatemeh; Beyraghi, Nargues; Noroozi, Alireza; Kobarfard, Farzad

    2008-12-01

    Despite the widespread medical use of glucocorticoids, reports of factitious administration of these hormones have been uncommon. We herein report an outbreak of Cushing's syndrome in Tehran among the addicts using Tamgesic (a brand of Buprenorphine) to help them through the narcotic withdrawal stage, without knowledge of the glucocorticoid content of the black-market drug. Case histories of 19 patients with a final diagnosis of iatrogenic Cushing's syndrome were reviewed. Liquid chromatography/mass spectrometry (LC-Mass) method was used to evaluate glucocorticoid existence in the brand. High performance liquid chromatography was used to determine plasma dexamethasone level. No buprenorphine was present in the vials. Each Tamgesic vial contained 0.4 mg of Dexamethasone disodium phosphate; Heroin was also found in them. The duration of injection abuse and the total dexamethasone intake was 4.5 (1-18) months and 2.6 (0.8-8) mg/day, respectively. Median plasma dexamethasone concentration was 5.8 nmol/l, with a range of 5-8.7. Physical findings of the cases were not different from those of the classic endogenous Cushing's syndrome but their serum cortisol and urinary free cortisol were suppressed. Severe life-threatening complications were demonstrated in five cases. Surreptitious use of steroids resulting in Cushing's syndrome may be more common in opium addicts; a high degree of suspicion is needed to uncover this disorder. Whenever facing a cushingoid appearance in addicts, the possibility of using black market drugs with corticosteroid contents should be kept in mind.

  17. West syndrome due to vitamin B12 deficiency.

    Science.gov (United States)

    Serin, Hepsen Mine; Kara, Aslıhan Oruçoğlu; Oğuz, Baran

    2015-12-01

    Vitamin B12 is one of the essential vitamins affecting various systems of the body. Vitamin B12 deficiency in infants often produces haematological and neurological deficits including macrocyticanaemia, neurodevelopmental delay or regression, irritability, weakness, hypotonia, ataxia, apathy, tremor andseizures. In this article, we report the case of a six-month-old male patient diagnosed with West syndrome associated with vitamin B12 deficiency. Although the patient had no evidence of macrocytic anemia in complete blood count, we measured the level of vitamin B12 because the patient had hypotonicity and found it to be low. No other problem was found in the other investigations directed to the etiology of West syndrome. He was being exclusively breast-fed and vitamin B12 deficiency was related with nutritional inadequacy of his mother. Vitamin B12 deficiency should be considered in the differential diagnosis of patients presenting with different neurological findings. In addition, vitamin B12 deficiency should be considered as a rare cause in West syndrome which has a heterogeneous etiology.

  18. Hypercalcemic encephalopathy due to milk alkali syndrome and injection teriparatide

    Directory of Open Access Journals (Sweden)

    Sandeep Kharb

    2012-01-01

    Full Text Available An 82-year-old male, a known case of severe osteoporosis with vertebral fracture and prostatic carcinoma, was treated with gonadotropin releasing hormone analogue, calcium carbonate, cholecalciferol sachet and injection teriparatide. His diet consisted of milk and curd. He developed altered behavior and generalized weakness, and on investigation, hypercalcemia, hypokalemia, and metabolic alkalosis with low parathyroid hormone levels were detected. Injection teriparatide was stopped and he was managed with forced saline diuresis and injection zoledronic acid. He was diagnosed as a case of milk alkali syndrome in whom teriparatide and prolonged immobilization played a permissive role in the development of hypercalcemic encephalopathy.

  19. Facial paralysis due to Ramsay Hunt syndrome - A rare condition

    Directory of Open Access Journals (Sweden)

    Aline Lariessy Campos Paiva

    Full Text Available Summary Ramsay Hunt syndrome (or herpes zoster oticus is a rare complication of herpes zoster in which reactivation of latent varicella zoster virus infection in the geniculate ganglion occurs. Usually, there are auricular vesicles and symptoms and signs such otalgia and peripheral facial paralysis. In addition, rarely, a rash around the mouth can be seen. Immunodeficient patients are more susceptible to this condition. Diagnosis is essentially based on symptoms. We report the case of a diabetic female patient who sought the emergency department with a complaint of this rare entity.

  20. Junctional chiasmatic syndrome due to large anterior communicating artery aneurysm

    Directory of Open Access Journals (Sweden)

    Maruthi Kesani

    2017-01-01

    Full Text Available Even though aneurysm involving the anterior communicating artery (A Com A was common in clinical practice, producing compressive symptoms such as visual loss was rare. We report a case, in which patient had gradually progressive visual loss with features of the junctional chiasmatic syndrome, imaging revealed partially thrombosed large A Com A aneurysm. Intraoperatively, aneurysm was found compressing the optic chiasm and right optic nerve, following clipping and decompression of the optic nerve and chiasm there was gradually improvement in the vision over 2 weeks postoperatively.

  1. A case of orbital apex syndrome due to Pseudomonas aeruginosa infection

    OpenAIRE

    Takeshi Kusunoki; Kaori Kase; Katsuhisa Ikeda

    2011-01-01

    Orbital apex syndrome is commonly been thought to have a poor prognosis. Many cases of this syndrome have been reported to be caused by paranasal sinus mycosis. We encountered a very rare case (60-year-old woman) of sinusitis with orbital apex syndrome due to Pseudomonas aeruginosa infection. She had received insulin and dialysis for diabtes and diabetic nephropathy, moreover anticoagulants after heart by-pass surgery. She underwent endoscopic sinus operation and was treated with antibiotics,...

  2. Further patient with Angelman syndrome due to paternal disomy of chromosome 15 and a milder phenotype

    Energy Technology Data Exchange (ETDEWEB)

    Gillessen-Kaesbach, G.; Passarge, E.; Horsthemke, B. [Institut fuer Humangenetik, Essen (Germany)

    1995-04-10

    This {open_quotes}Letter to the Editor{close_quotes} decribes a patient with Angelman syndrome due to paternal uniparental disomy of chromosome 15 and a milder phenotype compared to Angelman syndrome patients with a 15q deletion. 10 refs., 1 fig.

  3. Expanded motor and psychiatric phenotype in autosomal dominant Segawa syndrome due to GTP cyclohydrolase deficiency.

    NARCIS (Netherlands)

    Hove, J.L. van; Steyaert, J.; Matthijs, G.; Legius, E.; Theys, P.; Wevers, R.A.; Romstad, A.; Moller, L.B.; Hedrich, K.; Goriounov, D.; Blau, N.; Klein, C.; Casaer, P.

    2006-01-01

    BACKGROUND: Segawa syndrome due to GTP cyclohydrolase deficiency is an autosomal dominant disorder with variable expression, that is clinically characterised by l-dopa responsive, diurnally fluctuating dystonia and parkinsonian symptoms. OBJECTIVE: To delineate the neurological and psychiatric

  4. Pulmonary hypertension due to acute respiratory distress syndrome

    Directory of Open Access Journals (Sweden)

    S.A. Ñamendys-Silva

    2014-10-01

    Full Text Available Our aims were to describe the prevalence of pulmonary hypertension in patients with acute respiratory distress syndrome (ARDS, to characterize their hemodynamic cardiopulmonary profiles, and to correlate these parameters with outcome. All consecutive patients over 16 years of age who were in the intensive care unit with a diagnosis of ARDS and an in situ pulmonary artery catheter for hemodynamic monitoring were studied. Pulmonary hypertension was diagnosed when the mean pulmonary artery pressure was >25 mmHg at rest with a pulmonary artery occlusion pressure or left atrial pressure <15 mmHg. During the study period, 30 of 402 critically ill patients (7.46% who were admitted to the ICU fulfilled the criteria for ARDS. Of the 30 patients with ARDS, 14 met the criteria for pulmonary hypertension, a prevalence of 46.6% (95% CI; 28-66%. The most common cause of ARDS was pneumonia (56.3%. The overall mortality was 36.6% and was similar in patients with and without pulmonary hypertension. Differences in patients' hemodynamic profiles were influenced by the presence of pulmonary hypertension. The levels of positive end-expiratory pressure and peak pressure were higher in patients with pulmonary hypertension, and the PaCO2 was higher in those who died. The level of airway pressure seemed to influence the onset of pulmonary hypertension. Survival was determined by the severity of organ failure at admission to the intensive care unit.

  5. Hypocalcemia due to 22q11.2 deletion syndrome diagnosed in adulthood

    Directory of Open Access Journals (Sweden)

    Maria Cabrer

    2018-01-01

    Full Text Available Chromosome 22q11.2 deletion syndrome (22q11.2DS is a genetic syndrome that may present with hypocalcemia due to primary hypoparathyroidism (PH at any age. We report a new diagnosis of 22q11.2DS in a 57-year-old man who presented with symptomatic hypocalcemia. It is important to consider genetic causes of hypocalcemia due to PH regardless of age.

  6. Genetics Home Reference: Denys-Drash syndrome

    Science.gov (United States)

    ... Conditions Diagnosis & Management Resources Genetic Testing (1 link) Genetic Testing Registry: Drash syndrome Other Diagnosis and Management Resources (2 links) GeneReview: Wilms Tumor Predisposition MedlinePlus Encyclopedia: Nephrotic Syndrome General Information from MedlinePlus ( ...

  7. Acute glomerulonephritis mimicking nephrotic syndrome | Adedoyin ...

    African Journals Online (AJOL)

    There was no antecedent sorethroat or skin rash. At presentation, both patients had mild dyspnea, anasarca, massive ascitis, and hypertension (130/80mmHg and 150/100mmHg respectively) both systolic and diastolic blood pressure were greater than 99 percentile. Laboratory investigations revealed massive proteinuria ...

  8. Congenital nephrotic syndrome: A diagnostic and management ...

    African Journals Online (AJOL)

    kidney showed dilated Bowman's capsules with florid focal segmental glomerular sclerosis, global glomerulosclerosis and interstitial fibrosis. Although the child remained mildly oedematous, her albumin and diuretic infusions decreased to not more than once weekly. Her kidney function was normal and she remained ...

  9. Cyclosporin in steroid- resistant nephrotic syndrome

    African Journals Online (AJOL)

    1994-11-11

    Nov 11, 1994 ... infections, impaired hepatic, renal or neurological function, concomitant therapy with nephrotoxic agents, cytostatics, ketoconazole, phenytoin, barbiturates, rifampicin, isoniazid or steroids, malabsorption, cancer, end-stage irreversible renal disease, diabetes mellitus, uncontrolled hypertension and drug or ...

  10. Genetics Home Reference: congenital nephrotic syndrome

    Science.gov (United States)

    ... Ashraf S, Vega-Warner V, Bockenhauer D, Elshakhs NA, Joseph M, Hildebrandt F; Gesellschaft für Pädiatrische Nephrologie (GPN) ... National Institutes of Health National Library of Medicine Lister Hill National Center for Biomedical Communications 8600 Rockville ...

  11. Childhood Nephrotic Syndrome in Ilorin | Adedoyin | Nigerian ...

    African Journals Online (AJOL)

    Nigerian Journal of Paediatrics. Journal Home · ABOUT THIS JOURNAL · Advanced Search · Current Issue · Archives · Journal Home > Vol 28, No 3 (2001) >. Log in or Register to get access to full text downloads. Username, Password, Remember me, or Register · Download this PDF file. The PDF file you selected should ...

  12. Conversion to sirolimus from cyclosporine may induce nephrotic proteinuria and progressive deterioration of renal function in chronic allograft nephropathy patients.

    Science.gov (United States)

    Boratyńska, M; Banasik, M; Watorek, E; Falkiewicz, K; Patrzałek, D; Szyber, P; Klinger, M

    2006-01-01

    Antiproliferative and non-nephrotoxic properties of sirolimus have been exploited for treatment of patients with chronic graft dysfunction. In this paper we point to the possible association of nephrotic syndrome and renal impairment with rapid conversion from cyclosporine (CsA) to sirolimus in patients with chronic nephropathy. Five male patients, ages 34 to 56 years, with chronic renal failure in the course of glomerulonephritis, were transplanted between 1997 and 1999. For the first 49 to 65 months, the immunosuppressive regimen consisted of CsA, azathioprine (AZA), and prednisone. Thereafter, due to chronic nephropathy evidenced by biopsy, conversion to sirolimus was performed with sharp withdrawal of CsA. The serum creatinine level prior to conversion was 1.9 +/- 0.3 mg/dL. Trace to 86 mg/dL proteinuria was found in 3 patients, while 2 patients had about 200 mg/dL. After 2 to 4 months of sirolimus treatment the proteinuria progressed (558 +/- 183 mg/dL); edema, hypoproteinemia, hypoalbuminemia, and hyperlipidemia developed; and the serum creatinine increased to 3.5 +/- 0.8 mg/dL. Biopsies performed in three patients revealed new pathologic changes. After 4 to 5 months, we performed reconversion to calcineurin inhibitor. Proteinuria decreased to 0 to 150 mg/dL; nevertheless the serum creatinine was continuously rising. Six to 15 months after the conversion, 3 patients returned to dialysis. The fourth patient, who was earlier reconverted, has a serum creatinine level of 2.0 mg/dL after 15 months. In conclusion, conversion from CsA to sirolimus may induce nephrotic syndrome with progressive deterioration of renal function. Converted patients require careful monitoring of proteinuria and renal function. Early reconversion to calcineurin inhibitor may prevent progressive deterioration of graft function.

  13. ACUTE CORONARY SYNDROME DUE TO CORONARY VASOSPASM – CASE REPORT

    Directory of Open Access Journals (Sweden)

    S. D. Klimovskiy

    2017-01-01

    Full Text Available Spasm of the coronary arteries is a dynamic narrowing of one or more coronary arteries, leading to significant restriction of the lumen. The leading role in coronary  artery spasm diagnostic belongs to coronary  angiography and its accompanying provocative tests. Mechanisms of coronary  artery spasm development remain incompletely clarified and it's considered as a multifactorial disease with its own specific risk factors.  Though pathophysiology is no longer a matter of dispute, further  efforts should be aimed at the practical application of the recommendations. Diagnosis of the coronary  artery spasm  has important practical significance, due to the prevailing benefit of calcium antagonists in the treatment and absence of benefit from percutaneous coronary  interventions in most cases. A clinical case of the patient with multivessel, multifocal coronary  artery spasm is presented.

  14. Reduced activity of 11beta-hydroxysteroid dehydrogenase type 2 is not responsible for sodium retention in nephrotic rats

    DEFF Research Database (Denmark)

    Bistrup, C; Thiesson, H C; Jensen, B L

    2005-01-01

    has been suggested to allow glucocorticoids to stimulate MR, thereby contributing to sodium retention. We tested this hypothesis in the puromycin aminonucleoside model of nephrotic syndrome in rats. METHODS: Complete sodium and potassium intakes and excretions (faeces and urine) were measured in rats......)] to suppress endogenous glucocorticoids in the proteinuric stage during active sodium retention. RESULTS: Nephrotic rats developed proteinuria, positive sodium balance, decreased plasma aldosterone concentration, and decreased urinary Na(+)/K(+) ratio. 11betaHSD2 mRNA expression was down-regulated but protein...... expression was unchanged. SGK mRNA and phosphorylated SGK protein were up-regulated while total SGK protein expression was unchanged. Dexamethasone treatment, which suppressed plasma corticosterone concentration, did not correct sodium balance or fluid retention in nephrotic rats. CONCLUSION: Our results do...

  15. Gluteal compartment syndrome due to prolonged immobilization after alcohol intoxication: a case report.

    Science.gov (United States)

    Iizuka, Shinichi; Miura, Naoyuki; Fukushima, Tomokazu; Seki, Tomoko; Sugimoto, Katuhiko; Inokuchi, Sadaki

    2011-07-20

    Gluteal compartment syndrome is a relatively rare condition that mostly result from atraumatic causes such as prolonged immobilization due to drug abuse or alcoholic intoxication and incorrect positioning during surgical procedures rather than traumatic causes. Early diagnosis is difficult and sometimes delayed or overlooked because of poor physical signs resulting from altered mental status and inappropriate diagnosis by clinicians. It has been reported that more than half of the cases of gluteal compartment syndrome are associated with crush syndrome and sciatic nerve palsy. Early diagnosis and immediate fasciotomy are necessary to improve the functional prognosis. Here, we report the case of a patient with gluteal compartment syndrome caused by prolonged immobilization after acute alcoholic intoxication. After disease onset, the patient developed complications of crush syndrome and sciatic nerve palsy, but immediate fasciotomy improved his condition.

  16. Superior Vena Cava Syndrome due to Thrombosis: A Rare Paraneoplastic Presentation of Bronchogenic Carcinoma

    Directory of Open Access Journals (Sweden)

    Avradip Santra

    2016-07-01

    Full Text Available Superior vena cava (SVC syndrome is not an uncommon occurrence in patients with malignancy and it is often described as a medical emergency. In majority of the cases, SVC syndrome occurs due to mechanical obstruction of the SVC by extraluminal compression with primary intrathoracic malignancies. However, intraluminal obstruction due to thrombosis can also produce symptoms and signs of SVC syndrome. Clot-related SVC obstruction is mostly associated with indwelling central venous catheter and pacemaker leads, although such thrombosis can occur spontaneously in a background of a hypercoagulable state, e.g., malignancy. Here, an unusual case of sudden onset SVC syndrome has been reported, which on initial radiologic evaluation was found to have a lung nodule without any significant mediastinal mass or adenopathy compressing SVC. Subsequent investigation with Doppler ultrasonography of the neck showed thrombosis in the right internal jugular, right subclavian and right brachiocephalic vein, which was responsible for SVC syndrome. Histopathological evaluation of lung nodule confirmed presence of an adenocarcinoma. Therefore, venous thromboembolism as a paraneoplastic syndrome should be kept in mind while evaluating a case of SVC obstruction in a cancer patient. Management of the underlying disease is of prime importance in such cases and anticoagulation is the mainstay of therapy. Ability to identify paraneoplastic syndrome may have a significant effect on clinical outcome, ranging from early diagnosis to improved quality of life of the patient.

  17. Steven Johnson syndrome due to I.V Ceftriaxone--a case report.

    OpenAIRE

    Narayanan Veena; Mamatha G; Ashok L; Rajashekar N

    2003-01-01

    Steven-Johnson syndrome (SJS) is a rare vesiculobullous disease characterized by an acute cutaneous eruption that ivolves the skin and mucous membranes including those of the oral cavity. A rare case of Steven-Johnson syndrome, an unexpected treatment response, in a 25-year-old female patient due to administration of intravenous Cefriaxone (1 gm), a third generation cephalosporin has been reported and literature reviewed.

  18. Two cases of Cushing's syndrome due to overuse of topical steroid in the diaper area.

    Science.gov (United States)

    Semiz, Serap; Balci, Yasemin Işik; Ergin, Seniz; Candemir, Maşallah; Polat, Aziz

    2008-01-01

    Topical use of corticosteroids may cause immunosuppression and iatrogenic Cushing's syndrome via hypothalamic-pituitary-adrenal axis. We report two cases with iatrogenic Cushing's syndrome with different clinical outcomes due to abuse of same potent topical steroid clobetasol propionate. One of them died because of fatal disseminated cytomegalovirus infection. The other patient recovered completely. Physicians and parents should be informed about the adverse effects of such potent topical corticosteroids and physicians should prescribe less potent agents, especially during infancy.

  19. Concomitant Cushing's syndrome due to adrenal adenoma in a patient with systemic lupus erythematosus.

    Science.gov (United States)

    Shimizu, Masatoshi; Kawata, Masahito; Okada, Toshio; Yuu, Housai; Kurahashi, Toshifumi; Yamanaka, Kunito; Umezu, Keiichi

    2002-11-01

    A 51-year-old woman had been admin